scholarly journals Septooptic Dysplasia with an Associated Arachnoid Cyst

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Skyler V. McLaurin-Jiang ◽  
Julie K. Wood ◽  
David F. Crudo

A 4-week-old male infant presented with hypothermia, hypoglycemia, and hyperbilirubinemia. His medical history was remarkable for hydrocephalus secondary to an arachnoid cyst, intermittent hypoglycemia, hypothermia, and poor feeding requiring nasogastric tube for nutrition. Physical exam revealed retrognathia, mild hypotonia, micropenis, and clinodactyly. Ophthalmologic exam demonstrated bilateral optic nerve hypoplasia (ONH). Laboratory data confirmed inadequate cortisol and growth hormone response to hypoglycemia, a low thyroxine level, and direct hyperbilirubinemia. Magnetic resonance imaging of the brain confirmed the known history of arachnoid cyst with hydrocephalus but also revealed anterior pituitary hypoplasia, absence of the posterior pituitary bright spot, a thin pituitary stalk, and bilateral optic nerve hypoplasia. A diagnosis of septooptic dysplasia (SOD) was made. Hormone replacement with hydrocortisone and levothyroxine was started with improvement in the infant’s glycemic control, thermoregulation, feeding, and cholestasis. This case reinforces the importance of careful physical examination and laboratory review in a patient with known history of arachnoid cyst which has been previously described as an associated feature of optic nerve hypoplasia and hypopituitarism.

2010 ◽  
Vol 68 (3) ◽  
pp. 400-405 ◽  
Author(s):  
Karina de Ferran ◽  
Isla Aguiar Paiva ◽  
Daniel Luiz Schueftan Gilban ◽  
Monique Resende ◽  
Micheline Abreu Rayol de Souza ◽  
...  

Septo-optic dysplasia (SOD), also referred to as de Morsier syndrome, is a rare congenital condition, characterized by two of the classic triad features: midline brain abnormalities, optic nerve hypoplasia (ONH) and pituitary endocrine dysfunction. We report 5 children with SOD, originally referred to be evaluated due to short stature, who also presented bilateral optic nerve hypoplasia, nystagmus and development delay. In 4 of the patients, we identified neuroimaging abnormalities of the hypothalamo-pituitary axis such as anterior pituitary hypoplasia (3/5), ectopic posterior pituitary (4/5), thin or absent stalk (3/5) and empty sella (1/5). We also encountered diverse pituitary deficiencies: growth hormone (3/5), adrenocorticotropic hormone (3/5), thyroid-stimulating hormone (2/5) and antidiuretic hormone (1/5). Only one child presented intact pituitary function and anatomy. Although rare, SOD is an important cause of congenital hypopituitarism and it should be considered in children with optic nerve hypoplasia or midline brain abnormalities for early diagnosis and treatment.


Author(s):  
Christopher Lyons ◽  
Giovanni Castano ◽  
James E Jan ◽  
Michael Sargent

1996 ◽  
Vol 18 (3) ◽  
pp. 234-235 ◽  
Author(s):  
Takashi Ichiyama ◽  
Takashi Hayashi ◽  
Miki Nishikawa ◽  
Susumu Furukawa

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Christopher L. Siepker ◽  
Jennifer L. Zimmer ◽  
Kathleen M. Bedard ◽  
Kelsey A. Hart ◽  
Sarah L. Czerwinski ◽  
...  

Case Description. A two-month-old, female, Aberdeen-Angus calf was presented for congenital cataracts and blindness in both eyes (OU). The dam had a reported history of visual defects (not specified) and had produced other affected calves (per owner history). Ophthalmic examination revealed mature bilateral cataracts, attenuation of the iridic granules, persistent pupillary membranes, and dyscoric pupils. Additionally, the calf had a poor body condition, prognathism, dome-shaped head, excessive nasal drainage, limb contracture, and fever. Histopathology of both eyes revealed lenticular degeneration (congenital cataracts), retinal dysplasia, and optic nerve hypoplasia. BVDV IHC detected antigen within only the left eye (OS), consisting of intrahistiocytic and endothelial immunoreactivity within the ciliary body, iris, and choroid. No BVDV immunoreactivity could be detected in the right eye (OD). This case highlights the unique ocular changes present in in utero BVDV infection of cattle with a different immunohistochemical staining profile than previously described.


1995 ◽  
Vol 17 (5) ◽  
pp. 379
Author(s):  
Takashi Ichiyama ◽  
Takashi Hayashi

MedPharmRes ◽  
2018 ◽  
Vol 2 (3) ◽  
pp. 22-26
Author(s):  
Uyen Vo ◽  
Duc Quach ◽  
Luan Dang ◽  
Thao Luu ◽  
Luan Nguyen

Budd–Chiari syndrome (BCS), a rare and life-threatening disorder due to hepatic venous outflow obstruction, is occasionally associated with hypoproteinemia. We herein report the first case of BCS with segmental obstruction of the intrahepatic portion of inferior vena cava (IVC) and hepatic veins (HVs) successfully treated by endovascular stenting in Vietnam. A 32-year-old female patient presented with a 2-month history of massive ascites and leg swelling. She refused history of oral contraceptives use. Hepatosplenomegaly without tenderness was noted. Laboratory data showed polycythemia, mild hypoalbuminemia and hypoproteinemia, slightly high total bilirubin and normal transaminase level. The serum ascites albumin gradient was 1.9 g/dL and ascitic protein level was 1.1 g/dL. The other data were normal. BCS was suspected because of the discrepancy between mild liver failure and massive ascites; and the presence of hepatosplenomegaly and polycythemia. On abdominal magnetic resonance imaging, the segmental obstruction of three HVs and IVC was 2-3 cm long without thrombus. Cavogram revealed the severe segmental stenosis of intrahepatic portion of IVC with no visualized HV and extensive collateral veins. A Protégé stent was deployed to IVC. Leg swelling and ascites were completely resolved within 3 days after stenting. During 1-year follow-up, edema was not recurred and repeated laboratory results were all normal.


2019 ◽  
Vol 16 (1) ◽  
pp. 40-46
Author(s):  
Rui Guo ◽  
Ruiqi Chen ◽  
Chao You ◽  
Lu Ma ◽  
Hao Li ◽  
...  

Background and Purpose: Hyperglycemia is reported to be associated with poor outcome in patients with spontaneous Intracerebral Hemorrhage (ICH), but the association between blood glucose level and outcomes in Primary Intraventricular Hemorrhage (PIVH) remains unclear. We sought to identify the parameters associated with admission hyperglycemia and analyze the impact of hyperglycemia on clinical outcome in patients with PIVH. Methods: Patients admitted to Department of Neurosurgery, West China Hospital with PIVH between 2010 and 2016 were retrospectively included in our study. Clinical, radiographic, and laboratory data were collected. Univariate and multivariate logistic regression analyses were used to identify independent predictors of poor outcomes. Results: One hundred and seventy patients were included in the analysis. Mean admission blood glucose level was 7.78±2.73 mmol/L and 10 patients (5.9%) had a history of diabetes mellitus. History of diabetes mellitus (P = 0.01; Odds Ratio [OR], 9.10; 95% Confidence Interval [CI], 1.64 to 50.54) was independent predictor of admission critical hyperglycemia defined at 8.17 mmol/L. Patients with admission critical hyperglycemia poorer outcome at discharge (P < 0.001) and 90 days (P < 0.001). After adjustment, admission blood glucose was significantly associated with discharge (P = 0.01; OR, 1.30; 95% CI, 1.06 to 1.59) and 90-day poor outcomes (P = 0.03; OR, 1.27; 95% CI, 1.03 to 1.58), as well as mortality at 90 days (P = 0.005; OR, 1.41; 95% CI, 1.11 to 1.78). In addition, admission critical hyperglycemia showed significantly increased the incidence rate of pneumonia in PIVH (P = 0.02; OR, 6.04; 95% CI 1.27 to 28.80) even after adjusting for the confounders. Conclusion: Admission blood glucose after PIVH is associated with discharge and 90-day poor outcomes, as well as mortality at 90 days. Admission hyperglycemia significantly increases the incidence rate of pneumonia in PIVH.


Author(s):  
Hatice Ferhan Kömürcü ◽  
Gıyas Ayberk ◽  
Ömer Anlar

Introduction: Meningiomas are the third most common intracranial tumors in adults after glial tumors and metastases. Olfactory groove meningiomas often grow without symptoms due to their slow growth rates and location in the frontal lobe. Optic nerve sheath meningiomas are benign neoplasms of the meninges surrounding the optic nerve. The coexistence of olfactory groove and optic nerve sheath meningiomas without any history of neurofibromatosis or radiotherapy has never been reported in the literature. Case Report: A 36-year-old female patient presenting with anosmia, headache, memory disturbance, and visual impairment and operated with the diagnosis of olfactory groove meningioma was reported. In the postoperative period, optic nerve sheath meningioma was detected in the imaging performed due to the persistence of visual impairment. Conclusion: Olfactory groove and optic nerve sheath meningiomas are rare tumors and can be diagnosed late because they progress slowly. Early diagnosis and treatment may affect the prognosis and morbidity of these patients favorably.


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