OpenMed/multicare-articles huggingface dataset:
Clival Paraganglioma, Case Report and Literature Review
Authors: Rachel Moor, Michael Goutnik, Brandon Lucke-Wold, Dimitri Laurent, Si Chen, William Friedman, Maryam Rahman, Nichole Allen, Marie Rivera-Zengotita, Matthew Koch
Journal: OBM Neurobiol (2022)
Keywords: paraganglioma, catecholamine, clinical work up, radiation, surgical resection

Paragangliomas are rare tumors that may present with cranial neuropathies when located along the skull base. Supratentorial paragangliomas are less likely to secrete catecholamines but should be worked up, nonetheless. We highlight a case of a female in her fourth decade found to have a petroclival lesion following initial presentation that included one month of tooth pain, dysphagia, diplopia, hoarseness and right hemifacial hypoesthesia. Magnetic resonance imaging of the brain demonstrated a T2 hyperintense lesion favored to be a petroclival meningioma. Pre-operative angiography demonstrated a hypervascular tumor. She underwent a combined presigmoid craniotomy with posterior petrosectomy performed by both neurosurgery and neuro-otology. Pathology demonstrated paraganglioma. She had small volume residual tumor and is planned for continued outpatient radiotherapy. Paragangliomas should be on the differential for skull base lesions. Management paradigm involves multidisciplinary care and a combination of surgical resection and post-operative radiation. In this paper, we discuss underlying pathophysiology as well as appropriate workup and management.
Penetrating fish-hook ocular injury: management of an unusual intraocular foreign body
Authors: Ludovico Iannetti, Paolo Tortorella
Journal: Case Rep Med (2014)

Importance. Ocular penetrating fish-hook injuries represent an unusual and very dangerous ocular trauma. We report the management of an unusual case of a simple-single barbed fish-hook accident globe injury successfully treated with surgery. Observations. We described a case report of a caucasian 32-year-old man presented with a scleral perforation of the left eye caused by a fish-hook injury while fishing. The fish-hook penetrated the sclera, passed the trabecular meshwork, and exited into the anterior chamber. He underwent surgery under local anesthesia to remove the intraocular foreign body and to repair the wound. The hook was removed backing through the entrance wound, enlarge the primary scleral laceration. Final visual outcome, one month after trauma, was 0.0 LogMar. Conclusions and Relevance. Our unusual case shows a modified extraction technique of fish-hook from the eye. Although the fish-hook injury represents generally a serious occurrence, in some cases, a prompt and appropriate method of extraction can lead to a good final outcome.
Neonatal ilio-psoas abscess: report of two cases
Authors: Minakshi Sham, Dasmit Singh
Journal: J Neonatal Surg (2014)
Keywords: extraperitoneal surgical drainage, iliopsoas abscess (ipa), limb deformity, neonate
MeSH terms: Case Reports

Ilio-psoas abscess (IPA) is rare in children and exceptional in the neonate. However, we recently managed two consecutive male neonates with right-sided IPA. The first baby was born two days after rupture of the membranes and had thick meconium-stained amniotic fluid. There was no such high risk factor in the second child. Diagnosis was made by ultrasonography in both the patients. Extraperitoneal surgical drainage was done and systemic antibiotics were given. Delay in presentation and uncontrolled sepsis, led to mortality in the first case. On the contrary, relatively early presentation, prompt drainage of the abscess and good response to higher antibiotics, lead to successful salvage of the second baby.
Hyperaesthesia following genital herpes: a case report
Authors: Catriona Ooi, Vijay Zawar
Journal: Dermatol Res Pract (2011)
MeSH terms: Case Reports

We report an adult female patient who presented with sacral radiculopathy as incapacitating dysthesias following primary genital herpes simplex, which later recurred. Despite use of systemic antiviral treatment, the painful syndrome in our patient persisted. The success in treatment was seen only after the addition of amitriptyline hydrochloride. The case is being presented here for its rare manifestation and novel use of amitriptyline hydrochloride.
Early-Onset Alzheimer Disease (EOAD) With Aphasia: A Case Report
Authors: Marcus Kiiti Borges, Thais Nakayama Lopes, Marina Maria Biella, Alaíse Siqueira, Sivan Mauer, Ivan Aprahamian
Journal: Front Psychiatry (2018)
Keywords: alzheimer's disease, aphasia, case report, dementia, early onset
MeSH terms: Case Reports

Background: Alzheimer's disease (AD) is traditionally subdivided into early onset (EOAD) and late onset (LOAD). EOAD has an onset before age 65 years and accounts for 1-5% of all cases. Two main presentation types of AD are familial and sporadic. Case presentation: The authors present the case of a 68-year-old retired white man, with a college level educational background. At 55 years of age, the patient presented cognitive decline with short-term memory impairment and slowed, hesitant speech. At 57 years, he was unable to remember the way to work, exhibiting spatial disorientation. PET-CT: revealed hypometabolism and atrophy in the left temporal lobe and posterior region of the parietal lobes. Disease course: Evolving with difficulties in comprehension and sentence repetition over past 3 years and with global aphasia in past 6 months, beyond progressive memory impairment. Discussion: Possibly due to the young age and atypical presentation, and the diagnosis of EOAD is often delayed. To the best of our knowledge, this case can be classified as a sporadic EOAD with aphasia. Clinical variant and neuroimaging findings were crucial to the diagnosis and treatment of this atypical presentation of AD.
Hemophagocytic inflammatory syndrome in ADA-SCID: report of two cases and literature review
Authors: Elena Sophia Fratini, Maddalena Migliavacca, Federica Barzaghi, Claudia Fossati, Stefania Giannelli, Ilaria Monti, Miriam Casiraghi, Francesca Ferrua, Salvatore Recupero, Giulia Consiglieri, Valeria Calbi, Francesca Tucci, Vera Gallo, Maria Ester Bernardo, Sabina Cenciarelli, Monica Palmoni, Margherita Moni, Luca Galimberti, Marzia Duse, Lucia Leonardi, Elena Sieni, Elena Soncini, Fulvio Porta, Lucia Dora Notarangelo, Raffaella De Santis, Saverio Ladogana, Alessandro Aiuti, Maria Pia Cicalese
Journal: Front Immunol (2023)
Keywords: ada-scid, scid, hemophagocytic inflammatory syndrome, hemophagocytic lymphohistiocytosis (hlh), primary immunodeficiency
MeSH terms: Agammaglobulinemia / therapy, Lymphohistiocytosis, Hemophagocytic / diagnosis, Lymphohistiocytosis, Hemophagocytic / etiology, Lymphohistiocytosis, Hemophagocytic / therapy, Severe Combined Immunodeficiency / diagnosis, Severe Combined Immunodeficiency / therapy, Review, Case Reports, Research Support, Non-U.S. Gov't, Child, Cyclosporine, Humans, Cyclosporine, Severe combined immunodeficiency due to adenosine deaminase deficiency

Hemophagocytic inflammatory syndrome (HIS) is a rare form of secondary hemophagocytic lymphohistiocytosis caused by an impaired equilibrium between natural killer and cytotoxic T-cell activity, evolving in hypercytokinemia and multiorgan failure. In the context of inborn errors of immunity, HIS occurrence has been reported in severe combined immunodeficiency (SCID) patients, including two cases of adenosine deaminase deficient-SCID (ADA-SCID). Here we describe two additional pediatric cases of ADA-SCID patients who developed HIS. In the first case, HIS was triggered by infectious complications while the patient was on enzyme replacement therapy; the patient was treated with high-dose corticosteroids and intravenous immunoglobulins with HIS remission. However, the patient required HLA-identical sibling donor hematopoietic stem cell transplantation (HSCT) for a definitive cure of ADA-SCID, without HIS relapse up to 13 years after HSCT. The second patient presented HIS 2 years after hematopoietic stem cell gene therapy (GT), secondarily to Varicella-Zoster vaccination and despite CD4+ and CD8+ lymphocytes' reconstitution in line with other ADA SCID patients treated with GT. The child responded to trilinear immunosuppressive therapy (corticosteroids, Cyclosporine A, Anakinra). We observed the persistence of gene-corrected cells up to 5 years post-GT, without HIS relapse. These new cases of children with HIS, together with those reported in the literature, support the hypothesis that a major dysregulation in the immune system can occur in ADA-SCID patients. Our cases show that early identification of the disease is imperative and that a variable degree of immunosuppression could be an effective treatment while allogeneic HSCT is required only in cases of refractoriness. A deeper knowledge of immunologic patterns contributing to HIS pathogenesis in ADA-SCID patients is desirable, to identify new targeted treatments and ensure patients' long-term recovery.
The first case report of an intraosseous epidermoid cyst in the distal phalanx of the index finger with infection resulting in single clubbing finger: A case report and review of the literature
Authors: Ahmad Alhaskawi, Haiying Zhou, Yanzhao Dong, Jingtian Lai, Zewei Wang, Sohaib Hasan Abdullah Ezzi, Vishnu Goutham Kota, Mohamed Hasan Abdulla Hasan Abdulla, Zhenyu Sun, Hui Lu
Journal: Front Surg (2023)
Keywords: clubbing, distal phalanx, enchondroma, intraosseous epidermoid cyst, single clubbing finger
MeSH terms: Case Reports

An intraosseous epidermoid cyst at the distal phalanx of the index finger is extremely rare. These cysts are asymptomatic unless ruptured, severely infected, or transformed into malignant squamous cell carcinoma. We present a case of a single clubbing finger in an adult diagnosed with an intraosseous epidermoid cyst in the distal phalanx of the left index finger with no history of pulmonary or cardiovascular diseases. Preoperative MRI showed an expansile lytic lesion with a sclerotic margin. Histopathological examination indicates that there is keratinous cell debris in the cyst with a wall of stratified squamous epithelium, which was the key to the correct diagnosis of an intraosseous epidermoid cyst. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Postpriapism erectile dysfunction and shunt-related urethral stricture: long-term morbidity after proximal shunt for ischaemic priapism
Authors: Jibril Oyekunle Bello
Journal: Pan Afr Med J (2014)
Keywords: ischaemic priapism, postpriapism erectile dysfunction, cavernoso-spongiosal shunt, shunt-related urethral stricture
MeSH terms: Erectile Dysfunction / etiology, Ischemia / surgery, Penis / blood supply, Priapism / surgery, Urethral Stricture / etiology, Case Reports, Adult, Anastomosis, Surgical / adverse effects, Humans, Male, Time Factors, Urologic Surgical Procedures / adverse effects

Recent guidelines have advocated for step-wise treatment of increasing invasiveness in the management of ischaemic priapism though with low-level evidences. In the past, proximal shunts were favoured as first-line treatment. We present an African man who had proximal shunt (cavernoso-spongiosal) three decades ago for ischaemic priapism and subsequently had long-term morbidity over the three decades with adverse effect on his quality of life. Recent guidelines appear to be sound despite their limitations and more invasive cavernoso-spongiosal shunts may be associated with significant long-term morbidities and poor quality of life.
Perioperative Management of Subarachnoid Hemorrhage in a Patient with Alagille Syndrome and Unrepaired Tetralogy of Fallot: Case Report
Authors: Juan Fiorda-Diaz, Muhammad Shabsigh, Galina Dimitrova, Suren Soghomonyan, Gurneet Sandhu
Journal: Front Surg (2017)
Keywords: alagille syndrome, general anesthesia, ruptured aneurysm, subarachnoid hemorrhage, unrepaired tetralogy of fallot
MeSH terms: Case Reports

Alagille syndrome (ALGS) is a genetic disorder associated with multisystem dysfunction involving the hepatic, cardiovascular, and neurologic systems. Tetralogy of Fallot (TOF), a congenital cardiac anomaly, is commonly found in these patients. Patients with ALGS may also have an increased risk of cerebrovascular abnormalities and bleeding. Ruptured cerebral aneurysm and subarachnoid hemorrhage (SAH) may be developed, increasing the incidence of morbidity and mortality. Advances in neuroimaging and neurosurgery have allowed early identification and treatment of such vascular abnormalities, improving patients' outcomes and reducing life-threatening complications such as intracranial bleeding. Authors describe the perioperative management of a patient with ALGS and TOF who was admitted to the emergency department due a ruptured intracranial aneurysm with concomitant SAH. Surgical treatment included diagnostic cerebral arteriography with coil embolization of a left posterior communicating artery aneurysm, and placement of right external ventricular drain (EVD). The combination of neuroprotective anesthetic techniques, fast emergence from anesthesia, and maintenance of intraoperative hemodynamic stability led to a successful perioperative management. A multidisciplinary approach in specialized centers is essential for the treatment of patients with SAH, especially in patients with ALGS and complex congenital heart disease such as TOF.
Zinner Syndrome in Pediatric Age: Issues in the Diagnosis and Treatment of a Rare Malformation Complex
Authors: Valentina Cascini, Dacia Di Renzo, Vittorio Guerriero, Giuseppe Lauriti, Pierluigi Lelli Chiesa
Journal: Front Pediatr (2019)
Keywords: zinner syndrome, conservative treatment, cyst, minimally invasive treatment, renal agenesis, seminal vesicle
MeSH terms: Case Reports

Zinner syndrome (ZS) is the association of congenital seminal vesicle cysts and ipsilateral upper urinary tract anomalies, such as multicystic displastic kidney (MCDK). This condition is rare in pediatric age and both diagnosis and treatment are challenging. The aim of this study was to analyze the issues in diagnosis, management, and treatment of ZS in pediatric age. The medical records of two patients with ZS were examined. Furthermore, a review of the literature on this topic in pediatric age was performed. In our experience the diagnosis of ZS was incidentally achieved in the first months of life, as a consequence of studies performed for a prenatal diagnosis of MCDK. The first patient presented unspecific and transient symptoms, the second infant was completely asymptomatic. They were conservatively treated, with a long-term follow-up planned at least until adolescence. Fifty cases of ZS in pediatric age have been reported in the literature up to now. Only 12/50 were diagnosed in the first year of life. The diagnosis was demanding, as the clinical presentation was unspecific and the results at imaging studies needed a differential diagnosis with other retrovesical masses. More than 80% of these cases were asymptomatic at long-term follow-up. Therefore, a conservative management of ZS has been accepted for asymptomatic or poorly symptomatic patients, with occasional, transient, and unspecific symptoms, such as urinary tract infections or orchyepididimytis. As the surgical management is challenging, it is proposed only in those symptomatic patients. In conclusion, ZS is rare in pediatric age. However, it should be considered in the differential diagnosis of cystic masses within the pelvis in males with ipsilateral renal anomalies. A conservative treatment with a long-term follow-up is a safe option in the management of asymptomatic or poorly symptomatic patients, thus reserving the surgical approach only in those cases with symptoms.
Unilateral Asterixis in Arm and Leg Caused by Internal Capsula Stroke
Authors: Katharina Feil, Marion Huber, Nicolina Goldschagg, Lars Kellert
Journal: Case Rep Neurol Med (2018)
MeSH terms: Case Reports

We report an unusual clinical manifestation of ischemic stroke with acute right-sided asterixis affecting the arm as well as the leg due to a lesion in the left posterior limb of the internal capsula. After treatment with intravenous thrombolysis the patient made a good recovery. Notably, in this case unilateral asterixis affected the arm as well as the leg, resulting in postural and gait instability. In addition, damage in the basal ganglia-thalamo-cortical network, as in our patient, has to be distinguished from other supratentorial causes of acute asterixis like thalamic or frontal lobe lesions linked to the cerebello-brainstem-thalamo-frontal lobe circuits.
Nasopharyngeal Vascular Hamartoma in a Dog
Authors: Annalisa N Judy, Alexander I Krebs, Joseph Haynes, Nina R Kieves
Journal: Case Rep Vet Med (2020)
MeSH terms: Case Reports

An 8-year-old spayed female 32 kg Labrador retriever was presented for further investigation into the underlying cause of dyspnea, stertor, and sleep apnea present for three months and worsening over 30 days. There were significant reduction in airflow through the nares and loud inspiratory stridor. Thoracic and cervical radiographs made were normal. A skull CT and retrograde rhinoscopy showed a mass occluding the majority of the nasopharynx above the caudal third of the hard palate. The main differential diagnoses included a neoplastic mass vs. inflammatory mass vs. cyst vs. mucous obstruction. There was no destruction of nasal turbinates, making a benign etiology more likely. Biopsy of the mass showed an inflammatory process. En bloc excision of the mass was performed via ventral rhinotomy without complication. Histopathology of the excised mass revealed it to be a mucosal vascular hamartoma. The dog recovered uneventfully and had no further respiratory issues, short or long term. Although vascular hamartomas are a rare finding in veterinary medicine, they can be found in a wide variety of species and anatomic locations. They should be considered when naming differentials for benign mass lesions throughout the body, including the nasopharynx. Although they are benign masses in nature, they can be clinically significant and should be addressed. Prognosis after removal in this location is excellent.
Narrow-band imaging with magnification for the diagnosis of colorectal adenoma in a patient with Cronkhite-Canada syndrome
Authors: Hitoshi Fukase, Munenori Honda, Hideaki Miyamoto, Masatoshi Nakashima, Ryosuke Gushima, Hideaki Naoe, Rin Yamada, Yoshihiro Komohara, Yasuhito Tanaka
Journal: DEN Open (2023)
Keywords: cronkhite–canada syndrome, adenoma, colonoscopy, hamartoma, narrow‐band imaging
MeSH terms: Case Reports

Cronkhite-Canada syndrome (CCS) is a rare disease characterized by gastrointestinal polyposis, skin pigmentation, alopecia, and abnormal nailfolds. Although colorectal cancer has been reported in patients with CCS, reports are limited regarding the effectiveness of the usage of image-enhanced endoscopy in CCS lesions. Here, we report a case of CCS in which narrow-band imaging (NBI) magnifying endoscopy was applied to detect an adenomatous component in multiple hamartomatous polyps. A 79-year-old female complained of taste disorder, anorexia, and weight loss over several months. Endoscopic examination revealed multiple reddened polyps in the stomach and colon, leading to a diagnosis of CCS. Narrow-band imaging magnification showed sparse and dilated round pits on the CCS polyps. Furthermore, 12 out of the numerous colorectal CCS polyps had a coexisting light reddish elevated component with a regular distribution of microvessels and a regular reticular pattern. This pattern satisfied the criteria for Type 2A of the Japan Narrow-band-imaging Expert Team classification, indicating adenoma. After resection, these twelve polyps were subject to pathological analysis, which confirmed they were all hamartomatous polyps with low-grade adenoma on the superficial layer. Immunohistochemical analysis revealed a significant increase in the Ki-67 index and p53 staining only in the adenomatous lesions. We conclude that narrow-band imaging magnifying endoscopy would be useful in differentiating adenoma from CCS-related polyps, which thereby facilitates early detection and treatment of precancerous lesions.
Therapeutic effects of saireito (chai-ling-tang), a traditional Japanese herbal medicine, on lymphedema caused by radiotherapy: a case series study
Authors: Aiko Nagai, Yuta Shibamoto, Keiko Ogawa
Journal: Evid Based Complement Alternat Med (2013)

Despite the development of radiotherapy machines and technologies, a proportion of patients suffer from radiation-induced lymphedema. Saireito (SRT) is a traditional Japanese herbal medicine that has been used for treating edema and inflammation in conditions such as nephritic disease. This study investigated the effect of SRT on lymphedema caused by radiotherapy. Four patients were treated with SRT at a dose of 9 g/day. The severity of lymphedema was evaluated using the Common Terminology Criteria for Adverse Events version 4 and Numerical Rating Scale before and after SRT treatment. After the treatment with SRT, 2 of 4 patients (50%) showed apparent improvement in lymphedema. One of the cases had difficulty in wearing the custom-made thermoplastic cast, but after SRT administration, he could wear the mask easily. One case decided to stop taking SRT 3 days after initiation because cough and fever appeared. In conclusion, it is important to control the side effects of radiotherapy, which leads to improved tumor control rates. Prospective randomized studies are necessary to confirm the findings of this case series study.
Mixed neuroendocrine and squamous cell carcinoma of the colon: A case report and literature review
Authors: Adel Elkbuli, Brianna Dowd, Mark McKenney, Dessy Boneva
Journal: Int J Surg Case Rep (2019)
Keywords: case report, mixed neuroendocrine and squamous cell carcinoma, neuroendocrine carcinoma, primary colon cancer, squamous cell carcinoma

Introduction:
The occurrence of Neuroendocrine Carcinoma (NEC) of the colon is <2% of all colon cancers. Squamous Cell Carcinoma (SCC) is even more unusual and has an occurrence in the colon as low as 0.1% of colon cancers. Coupled together mixed NEC-SCC, is nearly unheard of. SCC of the gastrointestinal tract is usually found in the esophagus and rectum, rarely are there squamous cells even present in the colon.
Presentation of case:
A 62-year-old female presented with abdominal pain and a palpable mass in the left lower quadrant. CT scan revealed a poly-lobulated mass in the left abdomen attached to the left colon. A left hemicolectomy was performed. There were no distant metastases at the time of diagnosis. Microscopic examination revealed NEC mixed with SCC demonstrated by keratin pearl formation.
Discussion:
NEC and SCC individually are unusual in the colon but together are extraordinarily rare. Histologically, the NEC in this case demonstrated squamous cell differentiation with keratin pearl formation. The median survival in studies of patients with colonic NEC is 5-10 months due to the aggressive behavior and lymph node metastases, which were present in this case. There is insufficient literature addressing ideal adjuvant therapy after resection of mixed NEC and SCC of the colon.
Conclusion:
We present the first case of primary colon mixed NEC and SCC which presented as abdominal pain. There is a current absence of ideal therapy recommendations in the medical literature following resection of mixed NEC and SCC of the colon.
Noninvasive Management of Fractured Indwelling Tunneled Pleural Catheter Valve
Authors: Azib Shahid, Harpreet Singh, Toni-Denise Espina, Mohammad Abdalla, Uzair Ghori, Bryan S Benn
Journal: Case Rep Pulmonol (2022)
MeSH terms: Case Reports

Tunneled indwelling pleural catheters (IPCs) are frequently used to palliate symptomatic dyspnea due to recurrent pleural effusions. The drainage valve of IPCs is an important component of the catheter as fracture of the valve leads to malfunctioning of the IPCs. Replacement of the catheter includes risks such as pain, infection, pneumothorax, and procedure cost. We report two cases of malfunctioning tunneled IPC drainage valves repaired by our noninvasive method and discuss the need for a repair kit and a standardized approach to this repair in case of nonavailability of repair kits.