Septooptic Dysplasia with an Associated Arachnoid Cyst

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 re...

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Main Authors: Skyler V. McLaurin-Jiang, Julie K. Wood, David F. Crudo
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2016/5493769
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author Skyler V. McLaurin-Jiang
Julie K. Wood
David F. Crudo
author_facet Skyler V. McLaurin-Jiang
Julie K. Wood
David F. Crudo
author_sort Skyler V. McLaurin-Jiang
collection DOAJ
description 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.
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spelling doaj-art-d74796b5d11e4e9b9b1e950fb4ad66952025-02-03T05:58:52ZengWileyCase Reports in Pediatrics2090-68032090-68112016-01-01201610.1155/2016/54937695493769Septooptic Dysplasia with an Associated Arachnoid CystSkyler V. McLaurin-Jiang0Julie K. Wood1David F. Crudo2Department of Pediatrics, Wake Forest School of Medicine, Winston-Salem, NC, USASection on Hospital Medicine, Department of Pediatrics, Wake Forest School of Medicine, Winston-Salem, NC, USASection on Endocrinology, Department of Pediatrics, Wake Forest School of Medicine, Winston-Salem, NC, USAA 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.http://dx.doi.org/10.1155/2016/5493769
spellingShingle Skyler V. McLaurin-Jiang
Julie K. Wood
David F. Crudo
Septooptic Dysplasia with an Associated Arachnoid Cyst
Case Reports in Pediatrics
title Septooptic Dysplasia with an Associated Arachnoid Cyst
title_full Septooptic Dysplasia with an Associated Arachnoid Cyst
title_fullStr Septooptic Dysplasia with an Associated Arachnoid Cyst
title_full_unstemmed Septooptic Dysplasia with an Associated Arachnoid Cyst
title_short Septooptic Dysplasia with an Associated Arachnoid Cyst
title_sort septooptic dysplasia with an associated arachnoid cyst
url http://dx.doi.org/10.1155/2016/5493769
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AT juliekwood septoopticdysplasiawithanassociatedarachnoidcyst
AT davidfcrudo septoopticdysplasiawithanassociatedarachnoidcyst