OpenMed/multicare-articles huggingface dataset:
Ependymoblastoma with pulmonary metastasis in an adolescent: A case report
Authors: Xinmin Xu, Angcheng Li, Xia Xu, Qiangjun Gong, Shengjie Zhu, Wenya Chu, Shubo Ding
Journal: Front Neurol (2022)
Keywords: adolescent, case report, ependymoblastoma, metastasis, pulmonary
MeSH terms: Case Reports

Ependymoblastoma is a rare embryonal neoplasm of the nervous system, and the entity is even rare with distant metastasis. This case can help refine the existing literature and provide lessons for the management of other patients with ependymoblastoma. The present case concerns an adolescent with supratentorial ependymoblastoma, who received gross-total resection (GTR), postoperative radiotherapy, and six cycles of chemotherapy, with disease-free survival (DFS) of about 5.3 years. Subsequently, pulmonary metastasis occurred, but no intracranial lesion was found. Finally, combined treatment with radiotherapy and chemotherapy significantly reduced the lung lesions, with progression-free survival (PFS) of 10 months and long-term survival of 6.3 years. This case indicates that the lung metastases of ependymoblastoma are relatively sensitive to radiation, but lung metastases have not completely disappeared. Perhaps, increasing the radiation dose to lung metastases can improve the efficacy, which is worth exploring.
Case Reports: Management Challenges in Pediatric Germ Cell Tumors
Authors: Sophie Carr, Arash Safavi, Erik D Skarsgard
Journal: Front Pediatr (2021)
Keywords: alpha-fetoprotein, germ cell tumor, malignant, recurrence, retroperitoneal teratoma, surgery, teratoma
MeSH terms: Case Reports

Germ cell tumors in infants are most frequently extragonadal, benign, and amenable to surgical resection. An unusual feature of germ cell tumors is the potential coexistence of malignant with benign disease which makes it possible for patients with incompletely resected tumors to experience either a benign or malignant recurrence. A challenge to postoperative surveillance is the interpretation of serum alpha fetoprotein, a marker of malignancy, that is physiologically elevated during the first year of life. A rare subset of germ cell tumors occur in the retroperitoneum. Although the vast majority are benign, these tumors are often large and distort normal anatomy, and may demonstrate local invasiveness that increases risk of resection. The intent of these reports is to caution readers about these unusual features of germ cell tumors of infancy.
Encephalo-Arterio-Synangiosis with Cranioplasty after Treatment of Acute Subdural Hematoma Associated with Subcortical Hemorrhage Due to Unilateral Moyamoya Disease
Authors: Naoki Kato, Shota Kakizaki, Yusuke Hirokawa, Shotaro Michishita, Takuya Ishii, Tohru Terao, Yuichi Murayama
Journal: Case Rep Neurol Med (2023)
MeSH terms: Case Reports

Moyamoya disease is often diagnosed after intracranial hemorrhage in adult patients. Here, we report a case of unilateral moyamoya disease treated with indirect revascularization combined with cranioplasty after treatment for acute subdural hematoma and subcortical hemorrhage. A middle-aged woman with disturbed consciousness was transferred to our hospital. Computed tomography (CT) revealed an acute subdural hematoma with left temporoparietal subcortical hemorrhage. Three-dimensional CT angiography indicated a scarcely enhanced left middle cerebral artery (MCA) that was suspected to be delayed or nonfilling due to increased intracranial pressure. Subsequently, hematoma evacuation and external decompression were performed. Postoperative digital subtraction angiography (DSA) revealed stenosis of the left MCA and moyamoya vessels, indicating unilateral moyamoya disease. Forty-five days after the initial procedure, we performed encephalo-arterio-synangiosis (EAS) using the superficial temporal artery simultaneously with cranioplasty for the skull defect. The modified Rankin Scale score of the patient one year after discharge was 1, and the repeat DSA showed good patency of the EAS. Revascularization using EAS in the second step can be an option for revascularization for hemorrhagic moyamoya disease if the patient required cranioplasty for postoperative skull defect after decompressive craniotomy.
Mesocolic hernia following retroperitoneal laparoscopic radical nephrectomy: A case report
Authors: Naohiro Yoshida, Fumihiko Fujita, Kosuke Ueda, Suguru Ogata, Takahiro Shigaki, Takato Yomoda, Takafumi Ohchi, Tomoaki Mizobe, Tetsushi Kinugasa, Yoshito Akagi
Journal: Int J Surg Case Rep (2019)
Keywords: internal hernia, laparoscopic nephrectomy, mesocolon

Introduction:
Small bowel obstruction (SBO) caused by an internal hernia through a mesocolon after retroperitoneal laparoscopic nephrectomy (RLN) is rare.
Presentation of case:
A 66-year-old man who had undergone RLN with bladder cuff excision for a left renal pelvic cancer. After the surgery, he experienced SBO repeatedly. Contrast-enhanced computed tomography (CT) and gastrografin contract radiography through a long tube showed an internal hernia through the mesocolon to the retroperitoneal space where the resected left kidney had been located. We performed a subsequent surgery for the internal hernia. Postoperative course was uneventful and currently he has no recurrence of herniation 6 months post-operatively.
Discussion:
Mesenteric defects that cause an internal hernia can be created inadvertently during RLN when the colon is mobilized medially, and the kidney is being detached from retroperitoneum. The removal of a kidney leads to a potential retroperitoneal space to which small intestine can migrate. While there is no absolute necessity in mobilizing the colon during the retroperitoneal laparoscopic approach, there is still a risk of making mesocolic defects directly in the retroperitoneal space.
Conclusion:
We need to perform operations with sufficient anatomical knowledge of retroperitoneal fascia and careful surgical techniques. The critical thing to prevent an internal hernia following RLN is to close the mesenteric defects intraoperatively. It is also important to suspect an internal hernia and do proper examinations promptly when patients had the symptoms of SBO after nephrectomy.
Dignity Therapy in Pediatrics: A Case Series
Authors: Taryn Schuelke, Jared Rubenstein
Journal: Palliat Med Rep (2020)
Keywords: dignity therapy, legacy, pediatric palliative care

Objective: To report our first case series of Dignity Therapy modified for a pediatric palliative care population. Background: Dignity Therapy has been utilized successfully with terminally ill adult patients to help restore a sense of dignity and personhood as well as cope with existential distress near the end of life. To our knowledge, there are no published reports of this treatment modality in pediatric patients. Methods: The authors report the experience of a single-center case series of Dignity Therapy in a pediatric palliative care population. The adult Dignity Therapy process was adapted to fit the pediatric population and their families. Modifications are explained in some detail, and specific cases are shared to illustrate the process. The goal of this case series is to report on the application of Dignity Therapy to the pediatric population. Setting/subjects: Inclusion criteria for the cases series consisted of children and their families who were aware that death may occur soon, were English speaking, admitted to the hospital, and receiving care from the palliative care service. Results: Eight patients or their caregivers have completed Dignity Therapy thus far through our program. Four consented to publication of their experience. Three patients were adolescents and told their own story and the story of one younger nonverbal child was told by her family. All four participants reported that the intervention was acceptable and expressed gratitude for their final generativity document. No patient or family reported distress or negative effects from participation in Dignity Therapy. Conclusions: This case series describes how Dignity Therapy is possible with adaptations in the pediatric population, and how Dignity Therapy by proxy may be possible for caregivers of patients unable to tell their own story.
Diagnosis and Surgical Management of Congenital Intranasal Teratoma in a Newborn: A Rare Case Report
Authors: W X Yeo, K K Tan
Journal: Case Rep Otolaryngol (2018)
MeSH terms: Case Reports

Teratomas are the most common germ cell tumors of childhood. Head and neck teratomas, however, account for less than five percent of all teratomas. Considered rare at an incidence of 1 in 20,000 to 40,000 live births, they may occur in the cervical region, nasopharynx, brain, orbit, or oropharynx. Teratoma presenting as an isolated intranasal mass is extremely rare. In this report, we describe a case of a mature teratoma arising from the roof of the nasal cavity presenting as an isolated intranasal mass, the first of its kind from our literature review. The tumor was resected endoscopically with no recurrence detected.
Sinonasal glomangiopericytoma treated with preoperative embolisation and endoscopic sinus surgery
Authors: Elizabeth Psoma, Petros D Karkos, Stamatia Dova, Michail Gavriilidis, Konstantinos Markou, Constantinos Kouskouras, Afroditi Haritanti, Stefanos Finitsis
Journal: Ecancermedicalscience (2016)
Keywords: embolization, endoscopic sinus surgery, glomangiopericytoma, nose, sinus
MeSH terms: Case Reports

Sinonasal glomangiopericytoma is a benign rare tumour of pericytes that accounts for less than 0.5% of all sinonasal tumours. It is an indolent tumour with a macroscopic appearance of common inflammatory polyps. We report the case of a 55-year-old male who presented with right nasal obstruction. CT and MRI examinations demonstrated a soft-tissue mass that obstructed mainly the right nasal cavity. Biopsy revealed glomangiopericytoma. The tumour was treated with preoperative embolisation followed by complete endoscopic resection. Very few cases have been reported to be treated in this way.
Dynamic focal retinal arteriolar vasospasm in migraine
Authors: Anmar M Abdul-Rahman, Jagjit S Gilhotra, Dinesh Selva
Journal: Indian J Ophthalmol (2011)
MeSH terms: Arterioles / drug effects, Arterioles / pathology, Migraine Disorders / physiopathology, Retinal Vessels / drug effects, Retinal Vessels / pathology, Vasoconstriction / drug effects, Case Reports, Drug Administration Schedule, Follow-Up Studies, Fundus Oculi, Humans, Male, Middle Aged, Migraine Disorders / complications, Photography, Retinal Artery Occlusion / etiology, Retinal Artery Occlusion / pathology, Vasodilator Agents / administration & dosage, Vasodilator Agents

A 48-year-old man presented following an episode of sudden onset simultaneous inferior altitudinal visual loss in his left eye and visual obscuration with shimmering in the inferonasal quadrant of the right eye. Clinical examination demonstrated left superior hemiretinal artery occlusion and an area of focal dynamic spasm along the right superior temporal branch retinal artery, the arteriolar spastic cycle was about 2 sec in duration. Hematological (including complete blood count, thrombophilia screen, vasculitic screen and serum magnesium), carotid, and cardiac investigations were normal. He was given acetazolamide 500 mg orally, timolol maleate 0.5% eye drops once daily and sublingual amyl-nitrate 0.8 mg, and maintained on felodipine 10 mg/day and aspirin 100 mg/day. The area of focal arteriolar spasm in the right eye resolved over two months. To our knowledge there are no prior reports of photographically documented dynamic focal retinal vascular spasm on a MEDLINE and PUBMED search.
Early-Onset Diabetes Mellitus in Chromosome 8p11.2 Deletion Syndrome Combined With Becker Muscular Dystrophy - A Case Report
Authors: Conghui Cao, Xiaoli Wang, Xiaojuan Zhao
Journal: Front Endocrinol (Lausanne) (2022)
Keywords: becker muscular dystrophy, case report, chromosome 8p112 deletion syndrome, contiguous gene syndrome, early onset diabetes mellitus
MeSH terms: Diabetes Mellitus / genetics, Muscular Dystrophy, Duchenne / genetics, Spherocytosis, Hereditary / genetics, Case Reports, Research Support, Non-U.S. Gov't, Chromosomes, Exons, Humans, Quality of Life, Sodium-Phosphate Cotransporter Proteins, Type III / genetics, SLC20A2 protein, human, Sodium-Phosphate Cotransporter Proteins, Type III

Background:
Chromosome 8p11.2 includes several key genes in development such as the FGFR1, ANK1, KAT6A, and SLC20A2 genes. Deletion of this fragment causes a contiguous gene syndrome. Currently, few cases of interstitial deletion of whole 8p11.2 have been reported. We report a rare case of 8p11.2 deletion syndrome with the unique phenotypes, presenting with early-onset diabetes.
Case description:
A 20-year-old man with a 1-year history of diabetes mellitus was admitted to the Endocrinology Clinic. Physical examination revealed the dysmorphic facial features, and broad and foreshortened halluces. Laboratory examination indicated spherocytosis anemia, and hypogonadotropic hypogonadism. Bone mineral density analysis showed decreased bone density in the lumbar vertebrae. Brain CT showed calcification. Whole-exome sequencing revealed a 7.05-Mb deletion in 8p11 containing 43 OMIM genes, and a large in-frame deletion of exons 48-55 in the DMD gene. Metformin was given to the patient after which his blood glucose was well controlled. HCG was injected subcutaneously and was supplemented with calcium and vitamin D, which led to an improvement in the patient's quality of life.
Conclusion:
We report a rare case of 8p11.2 deletion syndrome with unique phenotypes, and early-onset diabetes. It is challenging for endocrinologists to simultaneously reconcile a combination of these diseases across multiple disciplines. We discussed the influencing factors of early-onset diabetes in this patient and speculated that it was caused by complex interactions of known and unknown genetic backgrounds and environmental factors.
Lung metastasis from perineal leiomyosarcoma: a case report and a review of the Japanese literature
Authors: Masamichi Itoga, Wataru Ito, Shigeharu Ueki, Masahide Takeda, Yuki Moritoki, Yoshiki Kobayashi, Mami Chihara, Naoshi Suzuki, Hideto Sasaki, Junichi Chihara
Journal: Case Rep Med (2013)

Pulmonary metastasis from leiomyosarcoma is rare and its clinical management is challenging. A single lung metastasis from a perineal leiomyosarcoma occurred in a 79-year-old woman. Five months after resection of the lung metastasis, a new metastatic tumor developed in the contralateral lung. Since the patient did not desire to receive hospitalized treatment, TS-1 (an oral agent consisting of a combination of tegafur, gimeracil, and oteracil potassium) therapy was started on an outpatient basis. The lung metastasis has been successfully controlled for at least 17 months with excellent tolerability. The clinical features and the treatment of this case are discussed.
Severe hyponatremia as the initial sign preceding guillain-barré syndrome, an acute inflammatory demyelinating polyneuropathy: a case report
Authors: Benjamin Kloesel, Latonya J Hickson
Journal: Case Rep Neurol Med (2013)

Guillain-Barré syndrome is an immune-mediated polyneuropathy that frequently presents with progressive muscle weakness. Hyponatremia has recently been described as a feature of this condition, generally appearing over the course of the illness and following the diagnosis of this demyelinating process. We report a case of Guillain-Barré syndrome presenting with severe hyponatremia that is further exacerbated by intravenous immune globulin therapy. Awareness should be raised for consideration of both Guillain-Barré syndrome and its treatment with intravenous immune globulin therapy as the cause of hyponatremia.
Successful use of differential target multiplexed spinal cord stimulation for chronic postsurgical abdominal pain
Authors: Ryusuke Tanaka, Kenji Shinohara, Yohei Hidai, Chiaki Kiuchi, Satoshi Tanaka, Mikito Kawamata, Junichi Sasao
Journal: Pain Rep (2023)
Keywords: chronic postsurgical pain, differential target multiplexed stimulation, neuropathic pain, spinal cord stimulation
MeSH terms: Case Reports

Introduction:
Recent advances in stimulation techniques have improved the efficacy and expanded the applicability of spinal cord stimulation (SCS). Among these techniques, there are no reports on the efficacy of differential target multiplexed (DTM) SCS for chronic postsurgical pain (CPSP) after abdominal surgery. Therefore, we present the successful use of DTM SCS for CPSP after distal pancreatectomy.
Methods:
A 49-year-old man with hypertension and severe chronic low back pain presented with neuropathic CPSP involving the left abdomen in the area of a laparotomy incision. His pain was refractory to conservative treatment and was rated 10 on a numerical rating scale (NRS). He underwent permanent implantation of a pulse generator after a 14-day trial stimulation.
Results:
Chronic postsurgical pain was well controlled (NRS 1-2) at a 3-month follow-up with DTM SCS.
Conclusion:
Differential target multiplexed SCS can be a new treatment option for neuropathic CPSP that is resistant to conservative treatment. It is important to further examine the characteristics of CPSP and identify appropriate candidates for the successful use of DTM SCS.
Spontaneous Hepatic Rupture with Intraperitoneal Hemorrhage without Underlying Etiology: A Report of Two Cases
Authors: Kristin Klein, A M James Shapiro
Journal: ISRN Surg (2011)
MeSH terms: Case Reports

Spontaneous hepatic rupture is a rare event most often occurring in association with underlying liver disease or pregnancy. We report two unusual cases of hepatic rupture without any identifiable pathology, trauma, or comorbid conditions.
Bilateral Hypothenar Hammer Syndrome Case Presentation and Literature Review
Authors: Andrés-Trasahedo Estíbaliz, Labrador-Sánchez Eztizen, Salazar-Asencio Osman-Alberto, Flores-Robles Bryan-Josué, Pinillos-Aransay Valvanera, Laura Torrecilla Lerena, López-Martín Juan-Antonio
Journal: Case Rep Vasc Med (2022)
MeSH terms: Case Reports

Hypothenar hammer syndrome is a rare cause of vascular insufficiency. Generally, patients report a history of repetitive trauma to the hypothenar region of the hand. Symptoms often consist of cold intolerance, pain, paleness, and paresthesia due to digital ischemia. The severity of these symptoms will depend on the extent of ulnar artery occlusion and the presence or absence of collaterals between this artery's superficial and deep branches. It is a rare clinical entity, which on multiple occasions requires a surgical approach. We present a 63-year-old man with bilateral Raynaud's phenomenon secondary to hypothenar hammer syndrome successfully treated by vascular repair surgery. In patients with Raynaud's phenomenon, it is important to know that there are reversible causes such as hypothenar hammer syndrome.
Port Placement via the Anterior Jugular Venous System: Case Report, Anatomic Considerations, and Literature Review
Authors: Gernot Rott, Frieder Boecker
Journal: Case Rep Radiol (2017)
MeSH terms: Case Reports

We report on a patient who was referred for port implantation with a two-chamber pacemaker aggregate on the right and total occlusion of the central veins on the left side. Venous access for port implantation was performed via left side puncture of the horizontal segment of the anterior jugular vein system (AJVS) and insertion of the port catheter using a crossover technique from the left to the right venous system via the jugular venous arch (JVA). The clinical significance of the AJVS and the JVA for central venous access and port implantation is emphasised and the corresponding literature is reviewed.
Difficulty in Fixation of the Volar Lunate Facet Fragment in Distal Radius Fracture
Authors: Hiroyuki Obata, Tomonori Baba, Kentaro Futamura, Osamu Obayashi, Atsuhiko Mogami, Hideki Tsuji, Yoshiaki Kurata, Kazuo Kaneko
Journal: Case Rep Orthop (2017)
MeSH terms: Case Reports

Recent reports suggest the presence of a rare fracture type for which reduction and fixation cannot be achieved with volar locking plate (VLP). In particular, it is difficult to achieve reduction and fixation with volar lunate facet (VLF) fragments present on the volar ulnar aspect of the lunate facet, because of the anatomical structure and biomechanics in this region. Herein, we report two challenging cases of difficulty in fixation of the VLF fragment in distal radius fracture. For this fracture type, it is most important to identify the volar ulnar bone fragment before surgery; it may also be necessary to optimize distal placement of the VLP via a dual-window approach and to apply additional fixations, such as a small plate, anchor, and/or external fixation.
Erythema multiforme: integrating the superfluous diagnosis to a veracious conclusion (case report)
Authors: Inukonda Lakshmi Mounica, Katne Tejaswi, Tirumala Chennaiah, Janumpally Varshitha Thanmai
Journal: Pan Afr Med J (2020)
Keywords: herpes simplex virus, drug induced, erythema multiforme, pathogenesis
MeSH terms: Erythema Multiforme / diagnosis, Mouth Diseases / diagnosis, Case Reports, Erythema Multiforme / pathology, Humans, Male, Mouth Diseases / pathology, Young Adult

Erythema multiforme (EM) is an acute, self-limiting hypersensitivity reaction that ranges from a mild cutaneous, exanthematous variant with minimal oral involvement to a progressive fulminating severe variant with extensive mucocutaneous epithelial necrosis (SJS & TEN). Knowing the etiological spectrum is the key for management in EM where sometimes leads to mortality. There are no specific diagnostic tests for EM, diagnosis is mainly clinically supported. Here in we report series of cases of EM with different etiological spectrum.
Psychosis in an adolescent girl: a common manifestation in Niemann-Pick Type C disease
Authors: Sara Wouters, Linda De Meirleir, Edward Campforts, Annik Lampo
Journal: Child Adolesc Psychiatry Ment Health (2014)
Keywords: adolescents, children, niemann-pick type c, psychiatric signs, psychosis
MeSH terms: Case Reports

Niemann-Pick disease type C (NP-C) is a rare autosomal-recessively inherited lysosomal storage disorder. It is caused by mutations in the NPC1 (95%) or NPC2 gene. It is a progressive and highly heterogeneous disease, characterized by the presentation of visceral, neurological, and psychiatric symptoms. Apart from the patients that die early from organic failure, most of the patients with juvenile and adolescent/adult onset of the disease, develop neurological and psychiatric symptoms. In some cases psychiatric signs, mostly psychosis, can be the first sign of the disease. A delay in diagnosis is often seen. By describing the case of a 16-year old girl, we would like to highlight current opinion about NP-C disease and resume recent findings on the clinical presentation, diagnosis and treatment. We focus on the psychiatric signs, and most important the specific combinations that are typical for the disease. There is no curative treatment for NP-C. Miglustat is used to modify neurological signs in NP-C.
A Case of Peripheral Ulcerative Keratitis Associated with Autoimmune Hepatitis
Authors: Hamoon Eshraghi, Aria Mahtabfar, Mohammad H Dastjerdi
Journal: Case Rep Med (2017)
MeSH terms: Case Reports

Purpose:
To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis.
Case report:
A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determined to be peripheral ulcerative keratitis. The patient was treated with topical and systemic steroids, immunosuppressant drugs (azathioprine and mycophenolate mofetil), a biologic (rituximab), and surgery (conjunctival resection), and the peripheral ulcerative keratitis epithelialized but ultimately led to corneal perforation.
Conclusion:
In this unique case, a patient with peripheral ulcerative keratitis who underwent treatment ultimately had a corneal perforation. This case may suggest a possible relationship between autoimmune hepatitis and peripheral ulcerative keratitis.