This is cryptorchidism. It has to be treated before the age of 2 years, and hormonal treatment is currently in disuse. The treatment of first choice is orchidopexy.

This is cryptorchidism. It has to be treated before the age of 2 years, and hormonal treatment is currently in disuse. The treatment of first choice is [HIDDEN].

12-month-old boy, who in the health examinations practiced since birth presents right testicle in inguinal canal that is not possible to descend to the scrotum. Mark the CORRECT answer:

The most likely diagnosis is retractile testis.

Wait until two years of age for spontaneous decrease of the synovial fluid.

Human chorionic gonadotropin is the treatment of first choice.

The indication for orchidopexy should not be deferred.

The efficacy of human chorionic gonadotropin in retractile testis.

To evaluate the efficacy of hCG therapy on retractile testis in various testicular locations and age groups. This study comprised 123 boys whose diagnosis were retractile testis with scrotal base, high scrotal and superficial inguinal localizations with an average age of 4,2 (1-8) years. 123 boys with retractile testes were given a total dose of 9000 hCG and its effect on scrotal base, high scrotal and superficial inguinal located testes were evaluated after 2 weeks and 6 months of completing hCG course. The patients were evaluated in four age groups such as less than 2 years of age, between 2-4, 4-6 years and more than 6 years of age and the response rates were also noted in these age groups. In unilateral cases, the response in base, high scrotal and superficial inguinal levels were found as 100%, 83,3%, 72,7% respectively where as these rates were found as 100%, 92,6%, 76,3% respectively in bilateral cases after 2 weeks of hCG therapy. The response rates in less than 2 year old group, 2-4, 4-6 and more than 6 years group were found as 0%, 80%, 78,7%, 100% respectively in unilateral cases and 33,3%, 90%, 86,9% and 83,3% respectively in bilateral cases. After 6 months, reascend was observed in 12,4% in unilateral group and 6,7% in bilateral group. 72,7-100% of retractile testes respond to hCG administration with the highest response in the age of more than six year group. High response rates observed in retractile testes after hCG therapy in more than 2 years old age group indicate that hCG must be given as a primary treatment in retractile testes and orchiopexy for the failures. Also patients must be followed up closely for reascend cases.

Acquired undescended (ascended) testis: effects of human chorionic gonadotropin.

A total of 7 boys 4 to 10 years old was evaluated for undescended testes. All patients had been seen previously by a pediatric urologist and diagnosed as having a retractile testis. In fact 4 boys had undergone surgical correction of a contralateral undescended testis at an earlier date at which time the testis in question could be manipulated into the scrotum. Another boy was examined under anesthesia and the operation was canceled because the testis could be brought into the scrotum. Subsequently, on followup evaluation 2 to 8 years later the ipsilateral testis could not be manipulated into the scrotum. Of the boys 6 then were treated with a short course of human chorionic gonadotropin. Four patients had a positive response but in 3 the testis was undescended again at examination 6 months later. Of the boys 6 ultimately underwent orchiopexy. Boys with highly retractile testes require periodic examination until puberty to ensure that those testes do not ascend secondarily.

[HCG in the treatment of cryptorchidism. The effect of age and position of the testis].

In a recent study, the effect of human chorionic gonadotropin (HCG) in the treatment of children with cryptorchidism aged less than four years was questioned. The purpose of the present study was to determine, on the basis of a retrospective examination of patients' records, the effect of HCG treatment, whether outcomes are age-dependent and whether the effects of treatment are related to the position of the testis. Patients diagnosed with undescended testis who had been treated at the Department of Urology at Gentofte Hospital (Copenhagen) in the period from 1 November 1998 to 31 May 2003 were identified. Patients who had been treated with HCG were included in the sample. Boys who at an earlier stage had been operated on in the inguinal or scrotal region, or who had suffered from inguinal hernia, were excluded, and clinical retractile testes were separated from true undescended testes. The criterion of success for efficient treatment was complete descent to the bottom of the scrotum. In the 306 records examined, 121 patients met the inclusion criteria. In total, there were 170 undescended testes. The patients' median age at time of treatment was 3.6 years. The overall success rate was 34.7%. The success rates in the individual age categories were: 1-2 years, 36.7%; 3-4 years, 33.4%; and 5-13 years, 35%. The position of the testis at the beginning of treatment showed that the lower the pretreatment position, the better the success rate. The overall success of HCG treatment of cryptorchidism was 34.7%. No age dependency of HCG effects was found, but the position of the testis before treatment influenced the success rate.

Ascending testis following inguinal hernia repair in children.

Failure to replace the testes in the scrotum during hernia repair leads to iatrogenic undescended testes. At other times, the testes may spontaneously move back to the inguinal area after being placed in the scrotum, thus resulting in ascending testes. The cases in this study were assessed. Records of 910 boys operated due to inguinal hernia were assessed retrospectively. Following hernia repair, the testes were placed in the scrotum. After the operation, all the testes were checked for being in the scrotum. They were called for follow-up after the operation. Their testes were checked for remaining in the scrotum. Ascending testes were detected in 4 (0.43%) of the patients. These patients had scrotal hypoplasia and/or retractile testes. Their age ranged between 1-3 years. Ascending testes were bilateral in 2 patients, and on the right side in 2. Human chorionic gonodotropin (hCG) was initiated in 3 patients. Two of them improved. Two underwent scrotal orchiopexy. These patients may benefit from hCG in the early postoperative period. Later, scrotal orchiopexy may be needed. Patients who have retractile testes or scrotal hypoplasia in addition to inguinal hernia need orchiopexy together with herniorrhaphy.

[Non-scrotal testes; first line of management].

For non-scrotal testes a distinction can be made between retractile testes (completely descended and normally developed but sometimes situated subcutaneously in the groin area), retained testes (testes cannot be brought into the scrotum or this can only be achieved using light manual pressure) and ectopic testes (lying outside of the descent trajectory). It is estimated that 0.7-0.8% of all boys have as yet undescended testes. The first few days after the birth are the most suitable for testing and registration, as then the cremaster reflex is absent. Registration should take place in both the youth healthcare file and in the 'growth book' for the parents. Retractile testes do not require treatment. There is no consensus concerning the treatment of (possible) acquired nonscrotal testes. For undescended testes the management depends on previous testes localisations. For ectopic testes and testes that have never been scrotal, a referral for surgical treatment should be made prior to the second birthday. Orchidopexy (a better description is orchidofuniculolysis followed by orchidopexy) is only justified in the case of testes which have never descended. In the case of a clear indication, the general practitioner should make a prompt referral (before the second birthday) and in other cases assurance should be provided and an expectant policy adopted until puberty.

Prospective evaluation of human chorionic gonadotropin in the differentiation of undescended testes from retractile testes.

We prospectively evaluated the efficacy of human chorionic gonadotropin (HCG) in the treatment of undescended testis and sought to determine whether HCG assists in the differentiation of undescended testis from retractile testis. Patients with undescended testes were offered HCG. Testis position, laterality and the presence or absence of a hypoplastic scrotum were noted. The same physician (G. W. K.) recorded physical findings prospectively and stated clinical impression of descent. A total of 67 patients with 90 undescended or retractile testes were treated and evaluated with HCG. Of the 64 undescended testes 13 (20%) descended with HCG therapy, with none requiring subsequent surgery. Of the 26 retractile testes 15 (58%) descended with HCG (p <0.001). Based on physical examination, 100% of retractile testes descended if the testis was in the high scrotal position but only 40% descended if the testis was in the superficial pouch or inguinal area. In the undescended testes group no ectopic or nonpalpable testis descended with HCG. Evaluation of HCG with age demonstrated minimal response (15%) to HCG at less than 24 months, and a peak response between ages 2 and 6 years (75%) with response decreasing thereafter. HCG may have a limited role in the evaluation of undescended testis in patients younger than 2 years. HCG can serve as an adjunct in the clinical diagnosis of retractile testis in older children.

Effect of a combined GnRH/hCG therapy in boys with undescended testicles: evaluated in relation to testicular localization within the first week after birth.

Among 509 boys referred with undescended testicles, 112 had true undescended testicles unilaterally and 62 bilaterally. Patients with true undescended testicles were offered hormonal treatment unless the condition was associated with hernia or previous operations. Boys less than 5 years old were primarily treated with gonadotrophin releasing hormone (GnRH), while boys more than 5 years old were primarily treated with human chorionic gonadotrophin (hCG). If the effect of the primary treatment was insufficient the other hormone was given. Testicular descent was obtained for 64% (23/36) of the intraabdominally located testicles in boys with bilaterally undescended testicles versus only 14% (3/21) in boys with unilaterally undescended testicles (p < .001). Treating 1 to 4 years old boys with GnRH resulted in descent in 16 of 95 testicles and secondary treatment with hCG yielded an additional 34, whereas secondary treatment of 5 to 13 years old boys with GnRH added only 10 descended testicles to 51 of 101 testicles. Reading the maternity records of 272 of the boys support other studies showing that testicles may reascend. Boys with endocrinological or "surgical" causes of incomplete testicular descent were relatively more likely to have had one or two undescended testicles during the first postnatal week compared with boys found to have only retractile testicles (p < .001). Treatment with hormones resulted in descent in 56% of boys whose testicles were both descended within one week after birth. Conversely, only 1 of 20 boys with unilateral testicular undescent postnatally was sufficiently treated with hormones (p < .001). The hormonal effect in boys with bilaterally undescended testicles at delivery did not differ significantly from boys with either one or none undescended testicle postnatally. In 35 of 51 boys (69%) in whom the hormonal effect was insufficient, operation revealed a "surgical cause" of the incomplete testicular descent.

[The undescended testis: arguments in favour of early treatment, provided retractile testis and acquired non-scrotal testis have been excluded].

--Guidelines for the treatment ofundescended testis (UDT) are sparse. Often an operation in the second year of life is advised. --Recent data indicate that the normal maturation process, which will ultimately lead to a normal quantity and quality of germ cells, is impaired as early as in the first half year of a newborn's life. None of the guidelines take this into account. Spontaneous descent after the fourth month following birth, of testes that have previously not descended, hardly ever occurs. --No differences have been shown in complication numbers between surgery before and after the first birthday. Orchidopexy prior to the 13th birthday reduces the risk of testicular cancer. --Therefore, based on these data, it is advised to perform orchidopexy in the second half of the first year of a newborn's life. In older boys a UDT must be treated before the 13th birthday. --In the Netherlands a lot more orchidopexies are done despite what may be expected based on prevalence numbers of UDT: testes retaining a normal volume that would most probably have descended spontaneously come puberty. --It remains important to carry out a careful physical examination and document the position of the testes soon after birth, and later on if UDT is suspected, to avoid unnecessary operations on retractile testes and acquired UDT.

Cryptorchidism

Diagnosis The most common diagnostic dilemma in otherwise normal boys is distinguishing a retractile testis from a testis that will not descend spontaneously into the scrotum. Retractile testes are more common than truly undescended testes and do not need to be operated on. In normal males, as the cremaster muscle relaxes or contracts, the testis moves lower or higher ("retracts") in the scrotum. This cremasteric reflex is much more active in infant boys than older men. A retractile testis high in the scrotum can be difficult to distinguish from a position in the lower inguinal canal. Though various maneuvers are used to do so, such as using a cross-legged position, soaping the examiner's fingers, or examining in a warm bath, the benefit of surgery in these cases can be a matter of clinical judgment.

Reappraisal of the role of human chorionic gonadotropin in the diagnosis and treatment of the nonpalpable testis: a 10-year experience.

We retrospectively evaluated the ability of human chorionic gonadotropin (HCG) to make the nonpalpable cryptorchid testis become palpable and promote testicular descent. Through surgical bookings we identified 94 patients younger than 11 years who received HCG between 1984 and 1994 for the diagnosis or treatment of a nonpalpable undescended testis. The dose of HCG was 1,500 IU/m.2 intramuscularly 2 times weekly for 4 weeks. Testis location was determined by physical examination before and after hormone administration, and confirmed at surgical exploration. Of the 99 nonpalpable testes identified in 94 patients 39 (39%) became palpable following HCG administration and only 2 (2%) completely descended. A total of 60 testes remained nonpalpable with the most common reason being an absent or severely atrophic testis (40, 67%). Of the testes remaining nonpalpable after hormonal stimulation 73% were surgically located at or distal to the internal ring. HCG is preoperatively efficacious in causing the nonpalpable undescended testis to become palpable. For patients failing to respond to hormonal stimulation we recommend preliminary inguinal exploration, since most testes or testicular remnants are located within the inguinal canal or immediately below the internal ring.

Pediatrics_Nelson

The undescended testis is usually histologically normal at birth. Atrophy and dysplasia are found after the first year of life. Some boys have congenital dysplasia in the contralateral descended testis. Surgical correction at an early age results in greater chance of adult fertility. Administration of human chorionic gonadotropin causes testosterone release from functioning testes and may result in descent of retractile testes. Orchidopexy is usually done in the second year of life. Most extra-abdominal testes can be brought into the scrotum with correction of the associated hernia. If the testis is not palpable, ultrasound or magnetic resonance imaging may determine its location. The closer the testis is to the internal inguinal ring, the better the chance of successful orchidopexy.

[German guideline on undescended testis-what is relevant in daily routine?]

With an incidence of 0.7-3% in male infants, undescended testicles is one of the most common congenital anomalies. In the first 6 months of life, the testicles may spontaneously descend in up to 70% of individuals. If the testicle is not in a scrotal position afterwards, fertility can gradually be reduced and the risk of a testicular tumor increases. Therefore, the current German guideline for undescended testis recommends that therapy should be take place between 6 and 12 months of life. After extensive information on the advantages and disadvantages, hormone therapy with the aim of a descensus or in those with bilateral anomaly with the aim of improving the germ cell pool can be offered. After the first year of life, hormone therapy is obsolete. Otherwise, surgical intervention is the treatment of choice. In the case of gliding or deep inguinal testis via scrotal or inguinal access, in the case of nonpalpable and sonographically undetectable testis, laparoscopy is carried out for diagnosis and simultaneous therapy. In the first postoperative year, adequate follow-up should be done to detect a re-ascensus and/or insufficient growth. Regular self-examinations from the age of 15 serve for the early detection of a testicular tumor that occurs only very rarely (approximately 0.003%).

Comparison of diagnostic and treatment guidelines for undescended testis.

Cryptorchidism or undescended testis is the single most common genitourinary disease in male neonates. In most cases, the testes will descend spontaneously by 3 months of age. If the testes do not descend by 6 months of age, the probability of spontaneous descent thereafter is low. About 1%-2% of boys older than 6 months have undescended testes after their early postnatal descent. In some cases, a testis vanishes in the abdomen or reascends after birth which was present in the scrotum at birth. An inguinal undescended testis is sometimes mistaken for an inguinal hernia. A surgical specialist referral is recommended if descent does not occur by 6 months, undescended testis is newly diagnosed after 6 months of age, or testicular torsion is suspected. International guidelines do not recommend ultrasonography or other diagnostic imaging because they cannot add diagnostic accuracy or change treatment. Routine hormonal therapy is not recommended for undescended testis due to a lack of evidence. Orchiopexy is recommended between 6 and 18 months at the latest to protect the fertility potential and decrease the risk of malignant changes. Patients with unilateral undescended testis have an infertility rate of up to 10%. This rate is even higher in patients with bilateral undescended testes, with intra-abdominal undescended testis, or who underwent delayed orchiopexy. Patients with undescended testis have a threefold increased risk of testicular cancer later in life compared to the general population. Self-examination after puberty is recommended to facilitate early cancer detection. A timely referral to a surgical specialist and timely surgical correction are the most important factors for decreasing infertility and testicular cancer rates.

The undescended testicle.

Cryptorchidism results from a complex hereditary series of incompletely understood events involving the HPG axis. The incidence is indirectly related to birth weight and dramatically decreases during the first 3 months after birth. Many nonscrotal testes are retractile and require no therapy whatsoever. True cryptorchid testes develop identifiable histologic alterations within 2 years of parturition, and endocrine abnormalities are often detectable during infancy. Hormonal therapy with hCG is effective in causing descent in only a small percent of children with cryptorchidism. GnRH nasal spray is no different from placebo in double-blind studies in which retractile testes have been excluded. The results are best in low-lying testes and in older children, but a recognized late-failure rate requires continued surveillance. HCG therapy appears to be of little use in nonpalpable cryptorchid testes. The risk of testicular cancer is increased in men with a history of cryptorchidism and even includes the contralateral descended testes. This risk may be reduced by early orchidopexy. Fertility is impaired in men with cryptorchidism and is reported to be no better than 75% and 50%, respectively, in men who have undergone successful unilateral or bilateral orchidopexy. There is unconfirmed evidence that orchidopexy carried out before the age of 2 years may improve these fertility rates. It is recommended that all children with cryptorchid testes undergo treatment by the age of 1 or 2 years. The parents of children with a nonpalpable testis should be informed of the high rate of testicular absence. If hormonal therapy is to be used, it must be initiated at 10 months of age. Treatment failures must be identified quickly to allow prompt referral of these children to a pediatric urologist or surgeon for orchidopexy.

Management of undescended testis.

The term cryptorchidism indicates a testis, which has failed to descend to the scrotum and is located at any point along the normal path of descent or at an ectopic site. Hormones play a pivotal role in testicular descent except during the migration to the level of internal inguinal ring. Cryptorchidism is present in about 4.5% of newborns with a higher incidence in preterms. The incidence decreases to 1.2% by the first year. It is classified as palpable and impalpable. The most common site of an ectopic testis is superficial inguinal pouch. Retractile testis is often bilateral and most common in boys between 5 and 6 years of age. Hypospadias and inguinal hernias are the most common associated anomalies seen with undescended testis. A thorough clinical examination helps in arriving at the etiology. A short hCG stimulation test helps to exclude anorchia. Different imaging techniques are of little help in diagnosis and require the help of an experienced radiologist. Laparoscopy has an important role in the diagnosis and management of undescended testis. The common complications include torsion and atrophy of testis. Infertility is seen in about 40% of unilateral and 70% of bilateral cryptorchidism. Undescended testis is 20 to 40 times more likely to undergo malignant transformation than normal testis. Both hCG and GnRH have been used with limited success in these children. All boys with cryptorchidism should be referred to a pediatric surgeon before 2 years of age. These children should be followed up every year after surgery to identify testicular tumors.

Practical approach to evaluating testicular status in infants and children.

To review the differences between normal, retractile, ectopic, ascended, and undescended testes and to describe the optimal way to perform a testicular examination to distinguish one from the other, as well as to demonstrate that ultrasound imaging is not necessary and to clarify when to consider specialist referral. This paper is based on selected findings from a MEDLINE search on undescended testes and orchiopexy referrals, and on our experience at the Urology Clinic at the Children's Hospital of Eastern Ontario in Ottawa, including review of referrals to our clinic for undescended testes and the resultant findings of normal variants versus surgical cases. The MeSH headings used in our MEDLINE search included <iundescended testicle, retractile testicle, ectopic testicle, ascended testicle, referral and consultation,</i and <iorchiopexy</i. An <iundescended testis</i is defined as the true absence of one testis (or both testes) from normal scrotal position. Ectopic and ascended testes will likewise be absent from the scrotum, the latter having been present at one point in development. Differentiating among testicular examination findings is important, as descended and retractile testes are managed conservatively, while prompt surgical intervention should be offered for ascended, ectopic, and undescended testes. Uncertainty surrounding the diagnosis of an undescended testis causes anxiety, might lead to unwarranted imaging, and might increase the wait list for specialty assessment. For this reason, avoidance of ultrasound in the evaluation of undescended testes was included in the recent Choosing Wisely Canada campaign. We seek to clarify the physical examination findings in the evaluation of possible undescended testes, the suggested referral parameters, and the subsequent management. Undescended testes and their variants are common. As decision for referral is based on the primary care physician's physical examination findings, we clarify distinguishing between normal and abnormal findings on testicular examination to aid in appropriate referral for subspecialist evaluation. Consultation, if needed, should be sought at 6 months' corrected gestational age, or at detection if later than 6 months, without delay for ultrasound imaging, as surgical management is recommended for those patients with undescended, ectopic, or ascended testes.

[Treatment of an undescended testis with human chorionic gonadotropin (HCG)].

Seventy one patients with ectopic testis of age between 2 and 11.5 years were treated with human chorionic gonadotropin (HCG) at doses recommended by the International Health Foundation. The descent of testis to the scrotum was achieved in almost half of the treated boys (49.3%). The descent was successful mainly in cases of lower inguinal position of the undescended testicle, and only rarely when the testicle was situated higher. The descent was never successful in cases when the scrotum was small and underdeveloped.

Effect of human chorionic gonadotrophin (hCG)/follicle-stimulating hormone treatment versus hCG treatment alone on testicular descent: a double-blind placebo-controlled study.

The medical treatment of retentio testis remains controversial because of ineffectiveness and/or adverse events. Follicle-stimulating hormone (FSH) seems to influence the spontaneous descent of the testis; furthermore, it induces luteinizing hormone (LH) receptors. Therefore, we performed a double-blind placebo-controlled study to investigate the effect of FSH with human chorionic gonadotrophin (hCG) versus hCG alone in retentio testis patients. Twenty-two boys with retentio testis were investigated, excluding retractile testis. Group A (N = 14: four with bilateral and 10 with unilateral retentio testis; mean age 3.15 years) was treated with 150 IU of FSH twice a week for 2 weeks followed by 150 IU of FSH and 250 IU of hCG (half the recommended World Health Organization dose) twice a week for another 4 weeks. Group B (N = 8: two with bilateral and six with unilateral retentito testis; mean age 3.3 years) was treated with 250 IU of hCG twice a week for 6 weeks. Testicular position, volume and consistency as well as the appearance of the scrotum and the penile length were determined at the start of the treatment as well as at weeks 2, 4, 6 and 12 by two independent investigators. Blood investigation consisted of measurements of LH, FSH, testosterone and sex hormone-binding globulin. Successful descent was considered when the testis reached a mid- or low scrotal position. In group A, 6/18 testes descended successfully. In group B, 6/10 testes descended. Of the unsuccessfully treated patients, six of group A and three of group B underwent surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

The volume of unilaterally undescended testis after hCG therapy compared to orchidopexy and combined methods.

The aim of the study was to compare the effects of human chorionic gonadotropin (hCG) therapy with those of surgical or combined therapy on testicular volume (TV) in boys at different ages with unilateral canalicular undescended testis (UDT). In total, 155 boys aged 1 to 12 years were treated: either surgically (ST), or by 50 IU/kg body weight hCG administration every three days for five weeks (HT), or by a combination of the two. The patients underwent ultrasound examination of TV before the treatment, 9-12 (median 10) and 24-39 months (median 32) after therapy. The testicular atrophy index (TAI) of the affected testicle was calculated. The success rate was 94.7% for ST, 39.2% for HT and 98% for HST patients. The atrophy rate was 5.3% for ST, 0% for HT and 2% for HST. Neither treatment type nor patient age significantly influenced gonadal atrophy. No significant differences in TV of the affected testis were observed after treatment between the groups. The TAI values were significantly the lowest in HT group (p = 0.0006). Both TV and TAI changes from the baseline values did not differ between the treatment groups. At the 24- to 39-month follow-up, no significant differences were observed in the change in baseline TV and baseline TAI between age groups. TV of the affected testis increased significantly (p = 0.0000), and TAI decreased significantly over time (p = 0.01), with no significant differences depending on the age group, treatment type or the interaction of the two factors. The hCG therapy did not impair the development of affected and healthy testes, neither as single nor as neoadjuvant therapy, both during early assessment and after 2-3 years. Patients' age at the initiation of treatment seems irrelevant.

Testicle

Testes follow the "path of descent" from high in the posterior fetal abdomen to the inguinal ring and beyond to the inguinal canal and into the scrotum. In most cases (97% full-term, 70% preterm), both testes have descended by birth. In most other cases, only one testis fails to descend (cryptorchidism) and that will probably express itself within a year. Pubertal The testes grow in response to the start of spermatogenesis. Size depends on lytic function, sperm production (amount of spermatogenesis present in testis), interstitial fluid, and Sertoli cell fluid production. After puberty, the volume of the testes can be increased by over 500% as compared to the pre-pubertal size. Testicles are fully descended before one reaches puberty. Clinical significance Protection and injury

Cryptorchidism: incidence and sperm quality in infertile men.

In a population of 8500 men attending the andrology outpatient clinic, 200 men (2.35%) were recorded as having some disturbances with the descent of the testes into the scrotum. Medical history of the patients revealed that 51 underwent unilateral orchidopexy; 40 bilateral orchidopexy; and 24 were treated with human chorionic gonadotropin in order to induce descent of their testes. In addition, 6 patients reported spontaneous descent of the testes, and 13 others were found to be unilaterally cryptorchid upon physical examination. Results of semen analysis, hormonal profile, testes position, and testicular volume were compared to those of 105 proven fertile men. The major finding of this study shows that post-partum undescended testes suffer from primary Sertoli cell malfunction as reflected by elevated serum follicle stimulating hormone levels. Serum luteinizing hormone and testosterone levels were within the normal range. Surgical descent of the testes did not improve sperm production, proved by low sperm quality of all the study groups, compared to the cryptorchid group. Among the patients who were operated on, no correlation was found between age at operation and semen variables. All groups showed poor sperm quality which can be defined as oligoteratoasthenozoospermia. The degree of spermatogenic damage was in the following order of diagnosis or treatment: bilateral orchidopexy greater than cryptorchid testes greater than hormonal treatment greater than unilateral orchidopexy greater than late spontaneous descent of the testes. Thus, it is advisable to postpone surgical treatment of cryptorchidism and apply this only after a waiting period, and if the hormonal approach has failed to descend the testis.

Pitfalls of conventional human chorionic gonadotropin stimulation test to detect hormonally functional cryptorchid testes in midchildhood.

To report two cases misdiagnosed as bilateral anorchism in midchildhood on the basis of multiple conventional human chorionic gonadotropin (HCG) stimulation tests and sonograms of the abdomen and pelvis. In two young male patients with cryptorchidism who were considered to have anorchism, we describe the findings on clinical examination, the testosterone levels before and after standard HCG stimulation testing, and sonographic findings during the midchildhood period. In both cases, as the children approached puberty the diagnosis was found to be incorrect. Two boys, 8 and 91/2 years old, were seen in consultation in our Pediatric Endocrine Clinic with a presumed diagnosis of anorchism. In the first case, multiple conventional HCG stimulation tests were done. In the second case, a single stimulation test was performed during routine follow-up assessments. In both cases, testosterone levels before and after HCG stimulation were consistent with the diagnosis of absent functional testicular tissue. Sonograms of the abdomen and pelvis also failed to detect the testicles. Both patients were ultimately noted to be pubertal (at 14 1/2 and >13 1/2 years, respectively) and to have early pubertal testosterone levels. A testicle was detected in one patient by abdominal computed tomographic scan and in the other by palpation of the inguinal canal. Conventional dosing and duration of the HCG stimulation test, as widely recommended in standard textbooks and in articles in the medical literature, may not elicit positive HCG-induced testosterone responses during midchildhood for detection of functional testicular tissue. During the midchildhood period, which is characterized by low gonadotropin, low sex steroid production, and a highly sensitive hypothalamic-pituitary-gonadal axis to feedback inhibition, a prolonged HCG stimulation test-perhaps of 4 to 6 weeks' duration-may be necessary. In addition, other investigational modalities may need to be used to detect the presence of functional testicular tissue during this developmental period.

Surgery_Schwartz

the incidence of infertility is approx-imately two times higher in men with unilateral orchidopexy compared to men with normal testicular descent.The use of chorionic gonadotropin occasionally may be effective in patients with bilateral undescended testes, suggest-ing that these patients are more apt to have a hormone insuf-ficiency than children with unilateral undescended testicle. The combination of micro-penis and bilateral undescended testes is an indication for hormonal evaluation and testoster-one replacement if indicated. If there is no testicular descent after a month of endocrine therapy, operative correction should be undertaken. A child with unilateral cryptorchidism should have surgical correction of the problem. The operation is typi-cally performed through a combined groin and scrotal incision. The cord vessels are fully mobilized, and the testicle is placed in a dartos pouch within the scrotum. An inguinal hernia often accompanies a cryptorchid testis. This should be

13 Years' experience with the combined hormonal therapy of cryptorchidism.

We analyze the results of the combined treatment with luteinizing hormone releasing hormone (LH-RH) and human chorionic gonadotropin (HCG) of a large series of patients with cryptorchidism. Between 1987 and 1999 and after strict differentiation between cryptorchid, retractile and gliding testes, 2,467 boys with 2,962 cryptorchid-gliding testes were treated with the combined hormonal therapy. LH-RH was administrated as a nasal spray at a dosage of 1.2 microg daily for a period of 4 weeks. HCG was injected intramuscularly, 5 times at 2-day intervals at a dosage adjusted according to the age. In the prospective study 2,476 boys with 2,962 cryptorchid testes were hormonally treated. Of the 2,962 evaluated cases 1,200 (40.52%) have been treated surgically after the hormone therapy. In 1,762 cases, 59.48% of cryptorchid testes were in the scrotum after combined hormone treatment. Treatment with LH-RH and HCG induced the descent of the testes to a normal scrotal position of boys with cryptorchidism in 59.48% of the evaluated cases. The combined treatment was effective for inducing descent of cryptorchid and gliding testes. According to the evaluated intraoperative findings, the failure of the combined therapy in 40.52% of the cases is due to the fact that the free descent is limited by local factors such as anatomical alterations of the inguinal canal, epididymal abnormalities or ectopic distal attachment of the lig. testis.

Cryptorchidism

Undescended testes are associated with reduced fertility, increased risk of testicular germ-cell tumors, and psychological problems when fully-grown. Undescended testes are also more susceptible to testicular torsion (and subsequent infarction) and inguinal hernias. Without intervention, an undescended testicle will usually descend during the first year of life, but to reduce these risks, undescended testes can be brought into the scrotum in infancy by a surgical procedure called an orchiopexy. Although cryptorchidism nearly always refers to congenital absence or maldescent, a testis observed in the scrotum in early infancy can occasionally "reascend" (move back up) into the inguinal canal. A testis that can readily move or be moved between the scrotum and canal is referred to as retractile. Cryptorchidism, hypospadias, testicular cancer, and poor semen quality make up the syndrome known as testicular dysgenesis syndrome. Signs and symptoms

Pediatrics_Nelson

Retractile testes are normal testes that retract into the inguinal canal from an exaggerated cremasteric reflex. The diagnosis of retractile testes is likely if testes are palpable inthe newborn period but not at later examination. Frequentlyparents describe seeing their son’s testes in his scrotum whenhe is in the bath and seeing one or both “disappear” when hegets cold. Available @ StudentConsult.com Torsion of the testis is an emergency requiring prompt diagnosis and treatment to save the affected testis. Torsion accounts for 40% of cases of acute scrotal pain and swelling and is the major cause of the acute scrotum in boys less than 6 years of age. It is thought to arise from abnormal fixation of the testis to the scrotum. On examination the testicle is swollen and tender, and the cremasteric reflex is absent. The absence of blood flow on nuclear scan or Doppler ultrasound is consistent with torsion.

[Cryptorchism. Outcome of treatment and referral patterns in an unselected group of patients in a 3-year period].

The age of diagnosis and referral together with the efficacy of HCG treatment were studied retrospectively in 196 unselected cryptorchid patients seen over a period of three years. The median age of diagnosis was 2 years and 7 months, whereas the median age of referral was three years later. During the period of study, guidelines for referral and therapy were published in a nationwide journal and in the local region, and a slight but significant fall in age of referral was seen thereafter. The median age of treatment with HCG, 92 patients, was 6 years and 11 months, and median age of surgery, was 7 years and 7 months. The rate of success with HCG was for bilateral testes 41% and for unilateral testes 21%, giving an overall success rate of 30%. This result is lower than previously reported, which is most likely explained by a higher suprascrotal position of testes before treatment. Guidelines and recommendations for referral and therapy seem to influence the time of referral, which is, however, in this study not in accordance with the consensus of definitive treatment before the age of two years. Early diagnosis is recommended and should be followed by referral to a paediatric department with particular interest and knowledge about cryptorchidism.

Orchiopexy-laparoscopy or traditional surgical technique in patients with an undescended palpable testicle.

To compare orchiopexy by laparoscopy versus traditional surgical technique in patients with an undescended palpable testicle in the inguinal canal. A prospective, comparative, observational, longitudinal, and double-blind research was done between August 2006 and March 2009 in the Centro de Especialidades Médicas del Estado de Veracruz, "Dr. Rafael Lucio"; 63 patients underwent surgery, age 1-10 years, all with the diagnosis of palpable undescended testicle in the inguinal canal; in 33 patients, the traditional surgical technique and in 30 patients laparoscopy were done. A visual analogue scale (VAS) was used to evaluate post-surgery pain. A testicle ultrasound was practiced before surgery and at 6 months after it. The majority of patients were 1-4 years old with a median age of 2.3 years; 51 cases were unilateral and 12 cases were bilateral; 37 testicles were descended with the open traditional surgical technique and 38 through laparoscopy (75 testicles); 44 on the right side and 31 on the left side; there was a hernia associated with 37 undescended testicles, 23 with open surgical technique, and 14 by laparoscopy, without relapsing in any patient. The median surgery time with the open surgical technique was 38 minutes and by laparoscopy, it was 45 minutes. The gobernaculum testis was sectioned by laparoscopy in 23 descended testicles to facilitate the procedure, in the remaining 11 it was not necessary; whereas in the open technique, all the gobernaculum testis were sectioned. In 80% of cases, the laparoscopy caused less pain when compared with the other technique. All patients regardless of the technique used left hospital during the first 24 hours. All have had follow-up for more than 6 months with a median of 18 months, with satisfactory results in relation to size and location of the testicle, with a good ultrasound correlation, and not finding any statistical differences between surgical techniques. There were no accidents with any of the techniques, and 1 patient with the open technique had an important hematoma; hemophilia was later diagnosed in the patient. The esthetical aspect was better with laparoscopy, but the cost was 15% more expensive with the open technique. Both techniques had satisfactory results without any significant differences to make us choose one over the other. It is the surgeons' decision based on experience and training on laparoscopy to choose any of the techniques.

[Surgical indications in testicular ectopias].

The ideal age for treatment is still controversial. However, all recent histological studies show that the number of spermatogonia and the diameter of the tubules remain virtually normal until the beginning of the third year of life. Treatment therefore should take place in the second year, after all chances of spontaneous migration have vanished and before lesions due to cryptorchidism develop. Chorionic gonadotropic hormones should be used systematically; they avoid surgery in only one out of five cases, but they make it possible to test the possibilities of testicular descent and testosterone secretion. Surgical treatment should follow precise rules. The spermatic vessels might, if necessary, be severed, but care must be taken to preserve the gubernaculum. The alternative is, microsurgical autotransplantation. Only time will show whether the poor fertility of these patients is bettered by early treatment.

Cryptorchidism

When the undescended testis is in the inguinal canal, hormonal therapy is sometimes attempted and very occasionally successful. The most commonly used hormone therapy is human chorionic gonadotropin (hCG). A series of hCG injections (10 injections over five weeks is common) is given and the status of the testis/testes is reassessed at the end. Although many trials have been published, the reported success rates range widely, from roughly 5% to 50%, probably reflecting the varying criteria for distinguishing retractile testes from low inguinal testes. Hormone treatment does have the occasional incidental benefits of allowing confirmation of Leydig cell responsiveness (proven by a rise of the testosterone by the end of the injections) or inducing additional growth of a small penis (via the testosterone rise). Some surgeons have reported facilitation of surgery, perhaps by enhancing the size, vascularity, or healing of the tissue. A newer hormonal intervention used in Europe is the use

The fate of undescended testes in patients with gastroschisis.

Cryptorchidism is frequently associated with gastroschisis, yet little is published on its management in such circumstances. In a review of 10 consecutive boys with gastroschisis since 1980, 4 had undescended testes. Gestational age and birth weight did not differ from the 6 boys with normally descended testes. The first two patients had associated arthrogryposis multiplex congenita. The first underwent bilateral orchidopexy at 9 years of age for inguinal testes. In the second patient, the left testis was intraabdominal at the level of the sigmoid colon at birth; at 3 months of age, when a left inguinal hernia repair was required, left groin exploration revealed the testis at the internal ring and orchidopexy was performed successfully. In the third patient the left spermatic vessels were divided at the time of gastroschisis repair and the testis anchored in the prebubic area. The second-stage orchidopexy was performed at 16 months. In the last patient the intraabdominal testis could be placed in a scrotal pouch without mobilisation or division of the vessels. From our experience and a review of the literature we conclude that: 1) undescended testes are frequently associated with gastroschisis; 2) mechanical factors rather than prematurity are likely responsible for this association; 3) if the testis easily reaches the scrotum, orchidopexy can be done safely at the time of gastroschisis repair; 4) if the testis does not reach easily and appears to have a gubernaculum, it may be preferable to leave it in place since spontaneous descent can occur.

[Results of treating cryptorchism with HCG in boys previously qualified for surgical treatment].

Hundred one boys, aged between 1.5 and 13 years, previously classified for surgery for uni- or bilateral cryptorchidism were examined. In 78 patients (77.2%) cryptorchidism was diagnosed, including 51 cases (50.5%) of unilateral undescended testicle. In the remaining 23 boys (22.9%) migrating testes were found: unilateral in 6 cases (5.9%) and bilateral in 17 cases (16.8%). An improvement was achieved after HCG therapy in 21 cases of migrating testes (91.3%), partial improvement in 1 case (4.35%), and no effect in 1 case (4.35%). The treatment with HCG produced recovery in 20 cases (25.6%) of cryptorchidism, partial improvement in cases (23.2%), and no effect in 40 cases (51.2%). A complete recovery was achieved in 41 out of 101 patients, i.e. in 40.6%, partial improvement in 19 cases (18.6%), and no effect in 41 cases (40.6%). The treatment with HCG enabled an avoidance of unnecessary surgery in 40.6% of all examined boys.


None of the other three options give that clinical picture (DPV is usually asymptomatic, CRVO produces abrupt and total or near total loss of vision with the typical "cherry red spot" in the fundus, and both an arteritic and non-arteritic NOIA would produce papillary edema usually with peripapillary but not macular hemorrhages, and none of these 3 options have any association with drusen in the contralateral eye). AMD in its exudative form presents with both this clinical presentation (loss of vision and metamorphopsia) and the characteristic fundus described in the question, in addition to the fact that drusen are usually observed in the contralateral eye.

An 84-year-old woman presents with loss of vision in the left eye of 4 days of evolution accompanied by metamorphopsia. The macula shows abundant hard exudates, two small deep hemorrhages and a localized neurosensory retinal detachment. In the contralateral eye there are abundant soft drusen. Which of the following diagnoses do you think is the most likely?

Exudative age-related macular degeneration (AMD).

Non-arteritic anterior ischemic optic neuropathy.

[Preventive treatment using laser of age-related macular degeneration of the contralateral eye after age-related macular degeneration of the first eye].

Since 1982, and with informed patient consent, we have photocoagulated confluent drusen and limited serous pigment epithelium detachment (SPED) in the fellow eye of ten patients suffering from advanced, disciform type, age-related macula degeneration (ARMD). This treatment was only carried out on appearance of metamorphopsia. Photocoagulation was performed with either the green ray of the argon laser, or the yellow ray of a dye laser. Spots of about 200 microns were placed in a grid-like fashion among the drusen. No complications were observed due to the treatment. The follow-up period on these ten patients, eight women and two men, mean age 77 years, was two to eight years, and the three patients have died. The drusen disappeared completely in three patients and partially in one. The functional results seemed favorable in three cases. In one case of confluent drusen associated with SPED and serous retinal detachment, vision improved remarkably from 0.3 to 0.5 with a Parinaud 2, with a follow-up of five years. In another case, the improvement was from 0.4 to 0.7 but the patient died after only a few months. In another case, vision has been stable for five years. The vision of the seven remaining patients deteriorated; three cases showed central areolar sclerosis, and one case a localised new vessel with vision less than 0.1. In three cases vision dropped to 0.2 and Parinaud 6, but they have been stable for at least four years (eight years for one patient).(ABSTRACT TRUNCATED AT 250 WORDS)

The causes of hyperreflective dots in optical coherence tomography excluding diabetic macular edema and retinal venous occlusion§.

To investigate the causes of hyperreflective dots (HRDs) in spectral domain optical coherence tomography (OCT) excluding diabetic macular edema (DME) and RVO (retinal vein occlusion). The medical records of 56 patients with HRDs documented by OCT were reviewed retrospectively. The patients with DME and RVO were excluded from the study in order to prevent misdiagnosing hard exudates or HRDs. The causes, unilaterality or bilaterality of HRD and demographic properties of the patients with HRD were evaluated. Sixty four eyes of 56 patients having HRDs were included in this study. Of the patients with HRD, 17 (30.36%) were women and 39 (69.64%) were men. The ages of patients were between 13 to 84 years (median 60.18 years). The causes of HRD were as follows: papilledema in 4 eyes (6.25%), active neovascular age related macular degeneration (AMD) in 33 eyes (51.56%), familial dominant drusen in 2 eyes (3.13%), central serous chorioretinopathy in 19 eyes (29.69%) and commotio retina in 2 eyes (3.13%), choroidal folds in one eye (1.56%), branch retinal artery occlusion in one eye (1.56%), central retinal artery occlusion in one patient (1.56%) and Best vitelliform macular dystrophy in one eye (1.56%). The most common cause of HRD was AMD. The causes of HRDs in both eyes were AMD and papilledema. The most common causes of HRDs excluding DME and RVO seem as active exudative AMD. The presence of HRDs in retinal diseases might affect the decisions and the results of the treatment and the prognosis of diseases.

Small retinal haemorrhages accompanied by macular soft drusen: prevalence, and funduscopic and angiographic characteristics.

To investigate the prevalence and clinical significance of small retinal haemorrhages accompanied by macular soft drusen in exudative age-related macular degeneration (AMD). This observational case series included patients who had first been diagnosed with exudative AMD. Small retinal haemorrhages were defined as preretinal or intraretinal haemorrhages, no larger than half the disc diameter in size and located within 3000 μm of the fovea centre. If there was more than one haemorrhage, the entire affected area was less than two-thirds of the disc diameter. Macular soft drusen was defined as the presence of soft drusen (≥125 μm in diameter) within the macular area. The presence of retinal angiomatous proliferation (RAP) was estimated based on the results of indocyanine green angiography (ICGA). The prevalence of reticular pseudodrusen was also estimated. Among the 1921 eyes from 1604 patients who were newly diagnosed with exudative AMD during the 40 months prior to the study, 101 eyes (5.3%) from 79 patients presented with the fundus characteristics described above. ICGA images were available for 69 eyes. Among these eyes, 28 eyes (43.1%) and 25 eyes (38.5%) were found to have type 1 and 2 RAP, respectively. A chorioretinal anastomosis (type 3 RAP) was identified in 12 (18.5%) eyes. Reticular pseudodrusen were noted in 78 eyes (77.2%). The presence of small retinal haemorrhages accompanied by macular soft drusen was highly predictive of RAP. The high prevalence of both soft drusen and reticular pseudodrusen in these eyes may suggest a profound decrease in choroidal perfusion in these eyes.

Drusen

Drusen, from the German word for node or geode (singular, "Druse"), are tiny yellow or white accumulations of extracellular material that build up between Bruch's membrane and the retinal pigment epithelium of the eye. The presence of a few small ("hard") drusen is normal with advancing age, and most people over 40 have some hard drusen. However, the presence of larger and more numerous drusen in the macula is a common early sign of age-related macular degeneration (AMD). Classification Drusen are associated with aging and macular degeneration are distinct from another clinical entity, optic disc drusen, which is present on the optic nerve head. Both age-related drusen and optic disc drusen can be observed by ophthalmoscopy. Optical coherence tomography scans of the orbits or head, calcification at the head of the optic nerve without change in size of globe strongly suggests drusen in a middle-age or elderly patient.

Wet Age-Related Macular Degeneration (Wet AMD) -- History and Physical

On examination, patients frequently have decreased best-corrected visual acuity (BCVA), and Amsler grid evaluation may reveal areas of central or paracentral scotoma or visual distortion. Ophthalmic examination of the anterior segment of the eye is usually normal. ARMD-related CNV has several different appearances on the dilated funduscopic exam. [17] These include: A gray-green membrane deep into the retina is usually associated with an overlying neurosensory retinal detachment. There may be the presence of blood, lipid, or subretinal fluid. RPE detachments appear clinically as dome-shaped, sharply demarcated elevations of the RPE; these may also be serous, fibrovascular, drusenoid, or hemorrhagic. [18] There may be massive subretinal hemorrhage with central vision loss or, less commonly, breakthrough vitreous hemorrhage with peripheral vision loss. RPE tears. Disciform scars may be present, which may appear as white or yellow subretinal membranes with or without RPE hyperplasia.

Fifteen-year cumulative incidence of age-related macular degeneration: the Beaver Dam Eye Study.

To describe the 15-year cumulative incidence of signs of early and late age-related macular degeneration (AMD). Population-based cohort study. We included 3917 persons, 43 to 86 years of age at the time of a baseline examination in 1988 through 1990 and with information collected in follow-up in 1993 through 1995, and/or 1998 through 2000, and/or 2003 through 2005. Grading of stereoscopic fundus photographs using the Wisconsin Age-Related Maculopathy Grading System. Cumulative incidence of drusen type and size, pigmentary abnormalities, geographic atrophy, and exudative AMD accounting for competing risk of death. The 15-year cumulative incidence was 14.3% for early AMD (the presence of either soft indistinct drusen or the presence of pigmentary abnormalities together with any type of drusen) and 3.1% for late AMD (presence of exudative AMD or geographic atrophy). There was an increased incidence of AMD lesions with age (P<0.05). Individuals > or = 75 years of age at baseline had significantly (P<0.01) higher 15-year incidences of the following characteristics than people 43 to 54 years of age: larger drusen (125 mum in diameter, 24.1% vs 10.6%), soft indistinct drusen (18.7% vs 6.5%), retinal pigmentary abnormalities (20.2% vs 3.7%), exudative macular degeneration (4.4% vs 0.4%), and pure geographic atrophy (3.2% vs 0%). Controlling for age, compared with those with small numbers of only small hard drusen (1-2), those with large numbers of only hard drusen (> or =8) had an increased 15-year age-adjusted incidence of both soft indistinct drusen (16.3% vs 4.7%) and pigmentary abnormalities (10.6% vs 2.7%). Eyes with soft indistinct drusen or pigmentary abnormalities at baseline were more likely to develop late AMD at follow-up than eyes without these lesions (17.8% vs 1.2% and 12.9% vs 1.7%, respectively). We document the long-term incidence of signs of AMD and a continuum from small hard drusen to late AMD in older persons in the population. The 15-year cumulative incidence of late AMD in people > or = 75 years of age (8%) indicates a public health problem of significant proportions because the United States population this age is expected to increase by 54% between 2005 and 2025.

Macular degeneration

AMD-like pathology begins with small yellow deposits (drusen) in the macula, between the retinal pigment epithelium and the underlying choroid. Most people with these early changes (referred to as age-related maculopathy) still have good vision. People with drusen may or may not develop AMD. In fact, the majority of people over age 60 have drusen with no adverse effects. The risk of developing symptoms is higher when the drusen are large and numerous, and associated with the disturbance in the pigmented cell layer under the macula. Large and soft drusen are thought to be related to elevated cholesterol deposits. Early AMD Early AMD is diagnosed based on the presence of medium-sized drusen, about the width of an average human hair. Early AMD is usually asymptomatic. Intermediate AMD Intermediate AMD is diagnosed by large drusen and/or any retinal pigment abnormalities. Intermediate AMD may cause some vision loss, but, like early AMD, it is usually asymptomatic.

Maculopathy

A maculopathy is any pathological condition of the macula, an area at the centre of the retina that is associated with highly sensitive, accurate vision. Forms of maculopathies Age-Related Macular Degeneration is a degenerative maculopathy associated with progressive sight loss. It is characterised by changes in pigmentation in the Retinal Pigment Epithelium, the appearance of drusen on the retina of the eye and choroidal neovascularization. AMD has two forms; 'dry' or atrophic/non-exudative AMD, and 'wet' or exudative/neovascular AMD. Malattia Leventinese (or Doyne’s honeycomb retinal dystrophy) is another maculopathy with a similar pathology to wet AMD. Cellophane Maculopathy A fine glistening membrane forms over the macula, obscuring the vision.

The time pattern of bilateral exudative age-related macular degeneration.

To study time patterns in bilateral exudative age-related macular degeneration (AMD) and the pattern of drusen before and after the onset of exudative AMD. Out of 2220 individuals in the Icelandic genetic study of AMD, 151 had bilateral exudative AMD. We searched for previous records in the Icelandic University Retina Unit. For the 65 patients with a fluorescein angiography record of both eyes, we established the time between the onset of disease in each eye. For the 53 patients with colour fundus photographs of the latter eye taken prior to the occurrence of exudative disease, we graded the drusen before and after the onset of exudative AMD in the second eye. The time interval between the onset of exudative AMD in the first and second eyes was 2.5 years (95% CI: 1.8-3.2; n = 65) and the median was 1.8 years. In 82% of cases the second eye was affected within 4 years. Soft drusen in the macula were found in 95% of eyes that later developed exudative disease (n = 53). Soft and hard drusen decreased in number in the central macula following the development of exudative disease. Bilateral exudative AMD develops within a few years in both eyes. Drusen are less visible following the onset of exudative AMD in the second eye.

Wet Age-Related Macular Degeneration (Wet AMD) -- Differential Diagnosis

Breakthrough vitreous hemorrhage can also occur in wet ARMD, and diagnosis may be challenging if there is a poor view on dilated funduscopic examination. Diagnosis may be established by evaluating the patient’s fellow eye or obtaining a thorough history. Other potential causes of vitreous hemorrhage include: Proliferative diabetic retinopathy Retinal tear or retinal detachment Hemorrhagic posterior vitreous detachment Neovascularization from other causes, including vein occlusions, radiation retinopathy, or sickle cell retinopathy

Age-related macular degeneration.

Age-related macular degeneration (AMD) is the leading cause of irreversible severe visual loss in the United States in people over 50 years of age. The nonexudative stage includes hard drusen (associated with localized dysfunction of the retinal pigment epithelium [RPE]), soft drusen (associated with diffuse dysfunction of the RPE), and geographic (areolar) atrophy. These fundus changes may predispose the eye to develop the neovascular/exudative stages of AMD. Most patients who develop severe visual loss from AMD have this exudative stage. Treatment for AMD has been shown to be effective for only a small proportion of patients who have a well-defined choroidal neovascular membrane (CNVM) more than 200 microns from the foveal center. Even in successfully treated cases, severe visual loss is postponed only for about 18 months because of the high rate of recurrent CNVMs that extend into the fovea. Thus, despite recent breakthroughs in laser treatment for AMD, most patients who develop the exudative form of AMD will develop central visual impairment. At the present time, the only available treatments for the majority of patients who develop the exudative form of AMD are low vision aids. Investigators are currently evaluating whether treatment is effective for membranes within 200 microns of the foveal center. Future studies need to be directed toward further understanding of the pathogenesis, treatment and prevention of the blinding complications of AMD.

[Macular diseases--application of automated static perimetry and optical coherence tomography].

The usefulness of automated static perimetry and optical coherence tomography in the management of macular diseases has been described. Scotomata in eyes with central serous chorioretinopathy could be evaluated with central 10-degree automated static perimetry. The degree of visual field defects in eyes with the disease varied greatly with mean deviation of -10 dB or less in as many as 10% of the subjects. Although retinitis pigmentosa is a diffuse retinal dystrophy, eyes with a moderately advanced stage of retinitis pigmentosa should be managed as a macular disease, because the functioning retina is confined within the vascular arcade. The progressive nature in this stage of the disease could be demonstrated with a central 10-degree automated static perimetry measured once or twice a year and the use of univariate linear regression of mean deviation, in half of the patients with a mean follow-up period of 5 years. Functional recovery in eyes with exudative age-related macular degeneration after laser surgery or submacular surgery could be evaluated with central 10-degree automated static perimetry. Eyes with increased mean deviation in spite of reduced visual acuity after therapeutic intervention should also be evaluated. Macular function could also be evaluated using a color test. A newly developed color saturation discrimination test was applied to patients with age-related macular degeneration, retinitis pigmentosa, and cone dystrophy. The degree of dyschromatopsia was less in eyes with age-related macular degeneration than in those with retinitis pigmentosa or cone dystrophy with the same level of acuity loss. The highly protrusive nature of the orange-red nodule in eyes with idiopathic polypoidal choroidal vasculopathy was demonstrated with dimensional measurement with OCT. The degree of protrusion was greater than in eyes with serous pigment epithelial detachment, which suggests that the polypoidal lesion is covered with rigid tissues including Bruch's membrane. Parafoveal retinal sensitivity obtained with automated static perimetry was studied in correlation with retinal thickness measured using OCT in eyes with branch retinal vein occlusion showing macular edema without macular non-perfusion or massive retinal hemorrhages. The increased retinal thickness due to macular edema is closely correlated with retinal sensitivity both at the fovea and in the parafoveal area. Eighty-nine phakic eyes of 46 patients with retinitis pigmentosa patients were studied to detect cystoid macular edema using OCT. Cystoid lesions were observed in the macula in 12 eyes in 6 (13%) of 46 patients. Some eyes with OCT-proven cystoid macular edema did not show dye pooling in the fluorescein angiogram. The width of the total area of cystoid lesions was positively correlated with best-corrected visual acuity but the thickness of the neurosensory retina at the center of the fovea was not. OCT findings of successfully repaired macular holes were categorized into 3 groups. Eyes with U-type showed a normal foveal contour and a dark layer corresponding to the outer segment of photoreceptors. Eyes with V-type showed a notch in the surface of repaired neurosensory retina without a dark layer on the retinal pigment epithelium. Those with W-type showed a defect of the neurosensory retina, where the retinal pigment epithelium was exposed. The visual results were excellent in eyes with U-type, but poor in those with W-type.

Acute visual loss

Acute visual loss is a rapid loss of the ability to see. It is caused by many ocular conditions like retinal detachment, glaucoma, macular degeneration, and giant cell arteritis, etc. Main causes Retinal detachment Retinal detachment should be considered if there were preceding flashes or floaters, or if there is a new visual field defect in one eye. If treated early enough, retinal tear and detachment can have a good outcome. Glaucoma Angle-closure glaucoma should be considered if there is painful loss of vision with a red eye, nausea or vomiting. The eye pressure will be very high typically greater than 40 mmHg. Emergent laser treatment to the iris may prevent blindness. Macular degeneration Wet macular degeneration should be considered in older people with new distortion of their vision with bleeding in the macula. Vision can often be regained with prompt eye injections with anti-VEGF agents. Giant cell arteritis

[Age-related Macular Degeneration in the Japanese].

Age-related macular degeneration (AMD) in the Japanese often shows different clinical features from those described in Caucasians. For example, we often observe choroidal neovascularization (CNV) in elderly patients without drusen in the fundus. The high incidence of polypoidal choroidal vasculopathy (PCV) in AMD among Japanese is well-known. The reason why such differences occur in clinical manifestations of AMD has been one of my main interests. In this review article, I will discuss the characteristics of AMD in the Japanese population, as found in our recent study. I. Prevalence and clinical characteristics of AMD in the Japanese population. Cohort studies are important to determine the prevalence and incidence of diseases. In Japan, cohort studies began to be carried out rather late compared with Western countries. Although good cohort studies from Japan are reported in the literature, the size of the cohorts was not sufficiently large to determine the prevalence of AMD. However, a recent meta-analysis of Asian cohorts has shown that the prevalence of late AMD in Asians is not different from that reported in Caucasians. On the other hand, the prevalence of early AMD appears lower in the Japanese than in Caucasians. Recently, we have published the results of the Nagahama Cohort study. In this cohort study, we found a high prevalence of drusen. It seems that the incidence of dry AMD is likely to increase among Japanese. In Japan, most retina specialists classify AMD into three categories : typical AMD, PCV, and retinal angiomatous proliferation (RAP). However, there are no definite diagnostic criteria to distinguish between the three conditions. To compare the clinical features of Japanese and Western cases of AMD, and to determine the incidence of the three types of AMD, we exchanged data about 100 consecutive cases between Kyoto University and Centre d'Ophtalmologie de Paris, France. Interestingly, the diagnoses made by the two institutes were not always in agreement. We also found more cases of PCV among the Japanese than among the French. II. PCV. About 50% of exudative AMD cases in the Japanese population are PCV. Because of its peculiar angiographic findings, PCV has long been considered to be a distinct clinical entity different from the usual exudative AMD. Also, there have been serious discussions on the nature of PCV. In our analyses, about 20% of PCV cases show rather large lesion sizes that exceed the vascular arcade. Scar formation in the macula and compromised vision are frequent findings in such cases. The occurrence of PCV in the inferior staphyloma or in angioid streaks shows heterogeneity in PCV. These findings suggest that PCV may be a finding on indocyanine green angiography rather than a distinct clinical entity. Spectral domain OCT examination shows that the branching vascular network of PCV is located between the retinal pigment epithelium and Bruch's membrane. In cases with retinal pigment epithelial detachment, CNV from the branching vascular network was found to extend along the roof of the detached retinal pigment epithelium. Such findings show that the branching vascular network of PCV is type 1 CNV. Complement factor H (CFH) and age-related maculopathy 2 (ARMS2)/High temperature requirement 1 (HTRA1) located on chromosome 10 (10q26) are well-established disease susceptible genes of AMD. In the Japanese, the prevalence of CFH Y402H gene polymorphism is low and ARMS2/HITRA1 plays a more important role in the development of AMD. In ARMS2 A69S polymorphism, a large deletion/insertion (443de1/54ins) that is reported in Caucasians was also found in Japanese. Thus, the genetic background of Caucasian and Japanese AMD is quite similar, as is also the case with exudative AMD and PCV. Our findings show that PCV is not a distinct clinical entity but is a subtype of exudative AMD. III. Exudative AMD with choroidal vascular hyperpermeability. Choroidal vascular hyperpermeability observed in central serous chorioretinopathy can be found in about 20% to 30% of exudative AMD cases in Japanese. Such cases often show a thick choroid, lack of drusen, and rather good visual prognosis with slow progression of the disease. Recently, "pachychoroid neovasculopathy" has been described by a group from New York. Such cases of AMD with choroidal vascular hyperpermeability, a thick choroid, and lack of drusen appears to belong to pachychoroid neovasculopathy. We studied the risk allele frequencies of CFH I62V and ARMS2 A69S gene polymorphisms in three groups : usual exudative AMD, pachychoroid neovasculopathy, and normal controls. Interestingly, cases of pachychoroid neovasculopathy show different gene polymorphisms of CFH I62V and ARMS2 A69S from the usual cases of exudative AMD and a more similar pattern to normal controls. Therefore, the possible mechanisms of the CNV development in such cases may differ from the classic well-documented drusen-dependent pathways. IV. Atrophic AMD in Japanese. Data from the Nagahama Cohort study show an increasing prevalence of drusen in Japanese. Recently, more extensive information on drusen has become available and the redefinition of drusen is currently in progress. In particular, the importance of reticular pseudodrusen (RPD) is more widely appreciated. This type of drusen is often found in Japanese AMD. Although the nature and location of RPD are still debatable, many investigators believe that this type of drusen is located under the sensory retina rather than under Bruch's membrane. In our analyses, RPD was found in 18.4% of late AMD cases in Japanese. It was more common in eyes with RAP or atrophic AMD and was seldom found in PCV. ARMS2 A69S gene polymorphism was found more frequently in cases of exudative AMD with RPD, than in cases of exudative AMD without RPD. Eyes with RPD show a thin choroid and diminished vascular densities of choroidal vessels.

[Humphrey Perimetry and Retinal Diseases].

Since, in most eyes with retinal diseases quality of vision is greatly affected by visual field defects including paracentral scotoma and inferior field defects, visual function should be assessed by central 30- or 10-degrree automated static perimetry as well as visual acuity testing. The reduction of light sensitivity, demonstrated in the results of Humphrey central 10-2 perimetry, is more apparent than visual acuity loss in eyes with central serous chorioretinopathy (CSC), in which patients complain of dimness in the visual field of the affected eye. While reduced light sensitivity in eyes with acute CSC is well correlated with the height of subretinal fluid, marked and irreversible light sensitivity loss is demonstrated in the absence of subretinal fluid in eyes with chronic CSC due to structural damage in the photoreceptors. Various degrees of light sensitivity loss are seen in eyes wih age-related macular degeneration corresponding to intraretinal or subretinal pathology including intra- or subretinal fluid, fibrous scarring containing choroidal neovascularization and atrophic changes. The mean deviation (MD) of Humphrey central 10-2 perimetry is useful in predicting the visual outcome in eyes with exudative AMD after photodynamic therapy or intravitreal injection of anti-vascular endothelial growth factor. The progression of retinitis pigmentosa is well assssed with MD of Humphrey central 10-2 perimetry, which decreases linearly in the stage of residual visual field of 10 degrees or less. The age of patients with visual loss below 0.5 is delayed in eyes showing pencil-like configuration of "Traquair's island of visual field", in which a small area of normal light sensitivity around the fixation point is surrounded by absolute scotoma. With less visual acuity loss compared with that seen in eyes with central retinal artery occlusion; eyes with branch retinal artery occlusion show marked visual field defects, which are permanent and profound simulating the nasal-step pattern seen in eyes with glaucoma. Non-perfusion areas in fluorescein angiograms of eyes with branch retinal vein occlusion generally demonstrate reduced light sensitivity in the results of Humphrey central 30-2 perimetry, the degree of which tends to correlate with severity of non-perfusion. The light sensitivity in the area of detached retina in eyes with rhegmatogenous retinal detachment is generally reduced to show absolute scotoma, yet these eyes recover greatly after successful surgical repair.

InternalMed_Harrison

• Next, examine the macula. The macula is the area between the superior and inferior temporal vascular arcades, and its center is the fovea. You can examine the macula by pointing your ophthalmoscope about 15° temporal to the optic disc. Alternatively, ask the patient to look into the center of the light. Note the foveal reflex and the presence of any hemorrhage, exudate, abnormal blood vessels, scars, deposits, or other abnormalities. • Examine the retinal blood vessels by re-identifying the optic disc and following each of the four main branches away from the disc. The veins are dark red and relatively large. The arteries are narrower and bright red. • Ask the patient to look in the eight cardinal directions to allow you to view the peripheral fundus. In a patient with a well-dilated pupil, it is possible to visualize as far as the equator.

A Case of Idiopathic Dense Vitreous Hemorrhage: Suspected Rupture of a Large Retinal Arterial Macroaneurysm on the Optic Disc.

We report a novel case of vitreous hemorrhage associated with suspected rupture of 2-disc-diameter retinal arterial macroaneurysm on the optic disc. A 90-year-old woman presented with blurred vision (sudden onset) in her left eye. Examination of the fundus revealed acute onset vitreous hemorrhage of unknown origin without retinal detachment. She underwent vitrectomy, but after excision of the dense vitreous hemorrhage, a 2-disc-diameter hematoma appeared on the optic disc and was removed promptly. Because the bleeding at the base of the hematoma was of arterial origin and pulsating, the first vitrectomy could not achieve hemostasis. Five days after the first surgery, we performed a second vitrectomy. This revealed a subretinal hemorrhage along the superior and inferior arcade vessels and a macular hole, which was almost completely closed with an inverted internal limiting membrane flap. Unfortunately, the macular hole reopened 41 days after the second surgery. In patients presenting with only a large hematoma on the optic disc, it might be prudent to leave the hematoma. However, this large retinal arterial macroaneurysm was on a rare location on the optic disc, making it doubly difficult for the surgeons to diagnose and choose the best option intraoperatively. The differential diagnosis for dense vitreous hemorrhage of unknown origin should include a large retinal arterial macroaneurysm on the optic disc.

InternalMed_Harrison

Optic Disc Drusen These are refractile deposits within the substance of the optic nerve head (Fig. 39-13). They are unrelated to drusen of the retina, which occur in age-related macular degeneration. Optic disc drusen are most common in people of northern European descent. Their diagnosis is obvious when they are visible as glittering particles on the surface of the optic disc. However, in many patients they are hidden beneath the surface, producing pseudopapilledema. It is important to recognize optic disc drusen to avoid an unnecessary evaluation for papilledema. Ultrasound or computed tomography (CT) scanning is sensitive for detection of buried optic disc drusen because they contain calcium. In most patients, optic disc drusen are an incidental, innocuous finding, but they can produce visual obscurations. On perimetry they give rise to enlarged blind spots and arcuate CHAPTER 39 Disorders of the Eye

Long-term follow-up of a case of Coats disease in a 10-year-old boy with spontaneous peeling of preretinal macular fibrosis: a case report.

Coats disease is a retinal vascular disorder characterized by aneurysms and telangiectasias. Macular fibrosis is a complication of Coats disease that results in vision loss. Macular fibrosis rarely develops in the natural course and often occurs after treatment with intravitreal bevacizumab, photocoagulation, or cryotherapy. Here, we have described an unusual case of spontaneous peeling of preretinal macular fibrosis in a patient with untreated Coats disease. A 10-year-old Japanese boy presented with vision loss in his left eye. The patient's left visual acuity was 20/28. Fundus examination of his left eye revealed thick preretinal macular fibrosis around the optic disc and macula. In addition, retinal telangiectasis, microaneurysms, hard exudates, and retinal hemorrhages were observed in the left peripheral temporal retina. We diagnosed his condition as Coats disease with preretinal macular fibrosis. Two months later, optical coherence tomography revealed preretinal macular fibrosis detachment at the foveal lesion without any treatment. During follow-up, preretinal macular fibrosis at the macular lesion was completely detached. Further, posterior vitreous detachment was observed and the shape of the macula and the patient's left visual acuity had improved. In our case, both formation and spontaneous peeling of preretinal macular fibrosis occurred without any treatment for Coats disease, which is an unusual finding. Vitreous changes might have occurred during the natural clinical course, causing subsequent posterior vitreous detachment and resulting in spontaneous peeling of fibrosis.

Acute Unilateral Anterior Ischemic Optic Neuropathy Secondary to Optic Nerve Head Drusen: Report of a Rare Coexistence.

A 45-year-old white male noticed on awakening the painless loss of inferior vision in the left eye 2 days ago. He was otherwise well and his medical history was unremarkable. Visual acuity was 20/20 in OD and 20/32 in OS with a left inferior altitudinal defect and right blind spot enlargement demonstrable on visual field test. On fundus examination, both disc margins were blurred and the left disc was diffusely oedematous, with linear haemorrhages in the adjacent nerve fibre layer. Radiologic imaging and laboratory tests were unremarkable. Bilateral optic nerve head drusen (ONHD) was demonstrated by optical coherence tomography and fundus autofluorescence imaging. Unilateral acute non-arteritic anterior ischemic optic neuropathy (NAION) and concomitant bilateral ONHD were diagnosed. NAION may develop secondary to ONHD. Therefore, clinicians should be aware of this rare association and inform the patients about this risk. Patients with ONHD should be followed-up periodically in terms of possible ischemic complications.

Clinical Features and Course of Patients with Peripheral Exudative Hemorrhagic Chorioretinopathy.

To evaluate the clinical characteristics of patients who were followed in our clinic with the diagnosis of peripheral exudative hemorrhagic chorioretinopathy (PEHC). Medical records of 12 patients who were diagnosed with PEHC in İstanbul University İstanbul Faculty of Medicine, Department of Ophthalmology between July 2006 and June 2014 were reviewed retrospectively. This study included 21 eyes of 12 patients. Four (33.3%) of the patients were male and 8 (66.7%) were female and ages ranged between 73 and 89 years. Eight (66.7%) of the patients were referred to us with the diagnosis of choroidal mass. Unilateral involvement was found in 3 and bilateral involvement in 9 patients. Temporal quadrants were involved in all eyes. Fifteen eyes (71.4%) had subretinal hemorrhage and hemorrhagic/serous retinal pigment epithelial detachment, 11 (52.4%) had lipid exudation, 5 (23.8%) had chronic retinal pigment epithelium alterations, 2 (9.5%) had subretinal fibrosis and 1 (4.8%) had vitreous hemorrhage. PEHC lesions were accompanied by drusen in 11 eyes (52.4%), geographic atrophy in 2 eyes (9.5%), and choroidal neovascularization scar in 2 eyes (9.5%). Treatment was done in both eyes of a patient for lesions which threatened the macula, in a patient with bilateral macular edema and in a patient with vitreous hemorrhage. The remaining eyes were followed-up without any treatment because the lesions did not threaten the macula and they showed no progression during follow-up. PEHC is a degenerative disease of peripheral retina that is seen in older patients, and signs of age-related macular degeneration (AMD) may accompany this pathology. Especially in patients with AMD findings, the peripheral retina must be evaluated carefully for existing PEHC lesions.

Metamorphopsia

Pathology The mechanisms that result in the development of metamorphopsia involve structural changes in the retina of the eye (retinal mechanism) as well as processing changes in the cerebral cortex of the brain (cortical mechanism). The retinal mechanism involves the displacement of retinal layers which results in the mislocation of light on the retina. The cortical mechanism, which was discovered after the retinal mechanism, is affected by perceptual “filling-in” and visual crowding effects. The cortical mechanism was found to work in combination with the retinal mechanism to contribute to metamorphopsia in long-standing maculopathy or after the treatment of macular disorders. Causes of Metamorphopsia Metamorphopsia can be a symptom of a number of eye disorders involving the retina or macula. Some of these conditions include the following: Age-related macular degeneration Epiretinal membrane and vitreomacular traction Posterior vitreous detachment Macular hole

Peripheral field loss: something old, something new.

A 37-year-old patient presented with acute visual field loss diagnosed as non-arteritic anterior ischemic optic neuropathy in the setting of optic nerve drusen. Her visual field loss had progressed when compared to the visual field done 2 years previously. Fundus examination showed bilateral optic nerve head drusen and left retinal nerve fiber layer edema consistent with non-arteritic anterior ischemic optic neuropathy.

Severe vision loss secondary to retinal arteriolar occlusions after multiple intravitreal brolucizumab administrations.

To describe a case of unilateral retinal arteriolar occlusion following multiple intravitreal brolucizumab injections for neovascular age-related macular degeneration (nAMD). A 92-year-old Caucasian woman presented with blurry vision in her left eye (OS) after receiving the third dose of intravitreal brolucizumab. At the time of presentation, visual acuity (VA) was 20/40 in her right eye (OD) and had decreased from 20/150 to count finger (CF) at 1-foot OS. On examination, there was no evidence of active inflammation in the anterior chamber OU. Dilated fundus examination showed no vitritis in OD and 1+ vitreous cells OS, flame-shaped hemorrhage at the superior optic disc margin, and retinal whitening surrounding the proximal portion of the supero-temporal branch of the central retinal artery. There were drusen in OS and retinal pigment epithelial (RPE) changes in the maculae of OU. Intra-arteriolar greyish deposits were seen OS. Fluorescein angiography (FA) showed hyper-fluorescence in the maculae corresponding to fibrovascular pigment epithelial detachments (PED) OU. No peri-vascular leakage was noted OU. Delayed filling of multiple arterioles in early and late phases OS was observed on FA. The patient was diagnosed with retinal arteriolar occlusion associated with repeated intravitreal brolucizumab administrations. Retinal arteriolar occlusion with severe vision loss, possibly secondary to inflammatory responses, can occur after subsequent intravitreal brolucizumab injections, even if no inflammation occurred after initial administrations. Vaso-occlusive disease should be considered as a potential ocular complication, with acute as well as delayed onset, following intravitreal brolucizumab therapy.

Acute visual loss and optic disc edema followed by optic atrophy in two cases with deeply buried optic disc drusen: a mimicker of atypical optic neuritis.

Sudden visual loss and optic disc edema caused by optic neuritis (ON) is usually followed by significant visual recovery. However, little or no recovery occurs when the loss is caused by atypical ON, especially in patients with neuromyelitis optica (NMO). Optic disc drusen (ODD) is a cause of pseudo optic disc edema and may be a predisposing factor for non-arteritic anterior ischemic optic neuropathy (NAION), thereby mimicking atypical ON. In such cases, if globular concretions are seen protruding from the disc substance, ODD may be suspected. The purpose of this paper is to describe two patients with acute visual loss followed by optic disc atrophy initially labeled as atypical ON. Though not suspected on clinical examination, optical coherence tomography (OCT) revealed deeply buried ODD as a predisposing factor for NAION. Case 1: A 48-year-old woman had bilateral sequential visual loss associated with optic disc edema. Despite treatment, vision did not improve and severe disc pallor ensued. Atypical ON was suspected. Eventually, she was started on immunosuppressant therapy based on a tentative diagnosis of NMO-spectrum disorder. On examination 5 years later, only severe optic disc pallor was observed, but OCT radial B-scans showed ovoid hyporeflective areas in the retrolaminar region of both eyes, compatible with ODD; this led to a diagnosis of NAION and deeply buried ODD. Case 2. A 35-year-old woman with suspicion of ON in the left eye and a history of previous atypical ON in the right eye was referred for neuro-ophthalmic examination which revealed diffuse optic disc pallor and a dense arcuate visual field defect in the right eye. OCT B-scans passing through the disc showed large ovoid areas of reduced reflectivity in the retrolaminar region of the optic disc in the right eye. These findings helped confirm the diagnosis of NAION in one eye, with deeply buried ODD as predisposing factor. Deeply buried ODD may be associated with NAION causing irreversible visual loss and optic disc pallor, a condition easily mistaken for atypical ON. Awareness of such occurrence is important to avoid unnecessary testing and minimize the risk of mismanagement.

[Characteristics of optical coherence tomography for exudative age-related macular degeneration].

To observe the characteristics of optical coherence tomography (OCT) for exudative age-related macular degeneration (AMD). Thirty-eight patients (42 eyes) diagnosed as exudative AMD by color fundus photography, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were examined by OCT. The results of OCT were analyzed and compared with that of FFA and ICGA. The characteristics of OCT for exudative AMD: fibrotic choroidal neovascularization (34 eyes), serous detachment of neurosensory epithelium (38 eyes), detachment of the nerve fiber layer (8 eyes), and hemorrhagic and serous detachment of retinal pigment epithelium (11 eyes and 9 eyes, respectively). The major characteristic of OCT of exudative AMD is fibrotic choroidal neovascularization combined with serous and hemorrhagic detachment of neurosensory epithelium or retinal pigment epithelium, which would be helpful for diagnosing and evaluating exudative AMD.

Visual prognosis of eyes with submacular hemorrhage associated with exudative age-related macular degeneration.

To study the retinal structural changes associated with submacular hemorrhage due to exudative age-related macular degeneration (AMD) and their relationships with visual prognosis. We retrospectively reviewed the medical records of 31 consecutive patients (31 eyes) with visual impairment due to an acute submacular hemorrhage associated with typical AMD (10 eyes) or polypoidal choroidal vasculopathy (21 eyes). Optical coherence tomography (OCT) revealed that submacular hemorrhage exhibited intense hyperreflectivity beneath the neurosensory retina and often seemed to infiltrate it. In the OCT sections, mild to moderate amorphous hyperreflectivity and/or hyperreflective dots were observed within the neurosensory retina, resulting in the loss of the junctions between the inner (IS) and outer (OS) segments of the photoreceptors. Of the 31 eyes, the foveal IS/OS line could be seen incompletely in 12 eyes and was totally absent in 16 eyes. The initial integrity of the foveal photoreceptor layer was correlated with the final visual acuity; the initial detection of the IS/OS just beneath the fovea was correlated with good final visual acuity (r = 0.375, p = 0.038). As a hallmark of integrity of the foveal photoreceptor layer, the initial detection of the IS/OS just beneath the fovea may predict good visual outcomes.

Posterior vitreomacular adhesion: a potential risk factor for exudative age-related macular degeneration?

To compare the state of the posterior vitreous in exudative age-related macular degeneration (AMD) with eyes with nonexudative AMD and controls. Prospective, observational case series. B-scan ultrasonography and optical coherence tomography (OCT) were performed in 163 eyes from 82 subjects older than 55 years, 50 eyes with exudative AMD, 57 with nonexudative AMD, and 56 control eyes. Main outcome measures were the number of eyes with complete posterior vitreous detachment (PVD) by ultrasound and the number of eyes with central vitreomacular adhesion by OCT. By ultrasonography, 17 (34.0%) of 50 eyes with exudative AMD had PVD as compared with 41 (71.9%) of 57 eyes with nonexudative AMD (P = .00002) and 34 (60.7%) of 56 controls (P = .017). OCT detected persistent central vitreoretinal adhesion surrounded by a detached posterior vitreous cortex in 18 (36%) of 50 eyes with exudative AMD, significantly higher than in nonexudative AMD (4/57 [7%]; P < .0001) and in controls (6/56 [10%]; P = .002). Persistent attachment of the posterior vitreous cortex to the macula may be another risk factor for the development of exudative AMD via vitreoretinal traction inducing chronic low-grade inflammation, by maintaining macular exposure to cytokines or free radicals in the vitreous gel, or by interfering in transvitreous oxygenation and nutrition of the macula. Inducing PVD may provide prophylactic benefit against exudative AMD.

[Optic disc drusen and acute vision loss].

Optic disc drusen (ODD) are calcified nodules within the optic nerve head. These are products of degenerated retinal ganglion cells axoplasmic transport, also known as hyaline or colloid bodies. They are mainly encountered as an incidental benign finding, or as a benign cause of swollen discs (in the differential diagnosis of papilledema). The majority of the patients are asymptomatic, and a slowly progressive sub-clinical visual field loss is found in about 80% of the patients. However, acute painless symptomatic visual field loss, occasionally massive, is rare. This is a case history of 4 patients who developed acute painless visual field loss which was attributed to optic disc drusen. The clinical features and proposed ischemic pathophysiology of acute vision loss and ODD are discussed.

[Clinical and pathological observation of seven cases of spontaneous sub-retinal hemorrhage].

To improve pathological understanding of massive sub-retinal hemorrhage. Retrospective case series study. The clinical and pathological data of 7 cases of massive sub-retinal hemorrhage which were examined in the Pathological Department of Tianjin Eye Hospital from May 1988 to April 2012 were collected. The serial section of eyeball specimens were made with HE and PAS staining. The pathological section were reviewed under the light microscope. Analysis were made again combining with patients' clinical history, imaging findings and the pathological features. In 7 patients, 6 patients were male, 1 patient was female. The age range was from 60 to 82 years old and the average age was 71.7 years old. Four cases were on the right eye and 3 cases were on the left eye. The main clinical feature was sudden loss of vision and 2 cases had acute glaucoma symptoms. Ultrasound examination showed choroidal tumor in 6 cases and retinal detachment with vitreous hemorrhage in one case. The color Doppler ultrasound examination demonstrated choroidal substantive occupying lesion in 3 cases and two of them were detected with blood flow signal. The MRI were examined in 3 cases which showed iso-high signal in T1W1 and iso-low signal in T2W1 suggesting the choroidal melanoma in 2 cases and sub-retinal hemorrhage in 1 case. Six cases were diagnosed clinically as choroidal tumor or melanoma and 1 case was diagnosed as acute angle-closure glaucoma. The enucleation were performed in 7 cases. In these cases, 6 cases were diagnosed pathologically as vitreous hemorrhage, hemorrhagic RPE detachment and massive subretinal hemorrhage. The related choroidal lesions included soft drusen of Bruch membrane in 3 cases, choriocapillaris wall degeneration in 2 cases, fibrovascular membrane formation under RPE in 2 cases, choroidal chronic non-granulomatous inflammation in 4 cases, choroidal vessels wall thickening and sclerosis in 2 cases and choroidal vessels anomaly in one case. The secondary angle-closure glaucoma or angle-open glaucoma were accompanied respectively in 2 cases. Another case of 7 cases was diagnosed pathologically as the rupture of retinal macroaneurysm with massive sub-retinal hemorrhage. Massive sub-retinal hemorrhage liked to occur in the elderly male patients. The main manifestations were sudden visual loss. A small number of patients had symptoms of acute glaucoma. Ophthalmic ultrasound and imaging characteristics were usually similar to the choroidal substantive neoplasm and were easily misdiagnosed as choroidal melanoma. Pathological examination revealed that the main reasons of massive hemorrhage were from the hemorrhagic RPE detachment and the rupture of retinal macroaneurysm.

Central serous papillopathy by optic nerve head drusen.

We report a 38-year-old man with a complaint of blurred vision in his right eye for the previous 5 days. He had bilateral optic disc drusen. Fluorescein angiography revealed multiple hyperfluorescent foci within temporal optic discs and temporal inferior arcade in late phase. Optical coherence tomography showed bilateral peripapillary serous detachment as well as right macular detachment. This is the first reported case of a concurrent peripapillary and macular detachment in a patient with central serous papillopathy by optic disc drusen. Central serous papillopathy is an atypical form of central serous chorioretinopathy that should be considered as a potential cause of acute loss of vision in patients with optic nerve head drusen.

Macular lesions predisposing to senile disciform macular degeneration.

Senile disciform macular degeneration (SMD) is a neovascular/exudative form of age-related macular degeneration (AMD). In this study 340 eyes were followed up to assess the progression of SMD. The 340 eyes consisted of two groups. Group 1 was composed of 157 eyes with age-related macular changes other than choroidal neovascular membrane. Group 2 was made up of the contralateral eyes of 183 unilateral SMD eyes. Average ages were 61 and 64 in groups 1 and 2, respectively, and respective follow-up periods were 44 and 52 months. Choroidal neovascular membrane developed in 12 eyes in group 1 (7.6%) and in 19 eyes in group 2 (10.4%), a total of 31 eyes (9.1%). Retinal pigment epithelium (RPE) detachment was found as a predisposing lesion in 25 of these 31 eyes. Choroidal neovascular membrane developed in 12 of the 24 eyes with large RPE detachments. In 3 eyes neovascular membrane developed from an RPE detachment which had evolved from soft drusen. There were 3 eyes among the 62 eyes with soft drusen in which neovascular membrane developed directly from soft drusen. Based on these results, we classified AMD into 3 types; 1) atrophic, 2) predisciform, which includes RPE detachment and soft drusen, and 3) SMD.