Giant Occipital Encephalocele Associated with Microcephaly and Micrognathia

Agrawal, Amit; Lakhkar, Bhavana; Lakhkar, Bhushan N.; Grover, Ankur

doi:10.1159/000187128

Cited by 9 publications

(11 citation statements)

References 9 publications

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“…Factors responsible for poor long-term prognosis are associated congenital anomalies, microcephaly, hydrocephalus, amount of herniated brain matter, large size of sac, and CSF leak. [ 9 10 ] Our case was fortunate to have minimal herniation of brain tissue with no associated anomalies and hydrocephalus.…”

Section: Discussionmentioning

confidence: 83%

“…Surgical strategies in these young neonates largely depend on the size of the sac, amount of herniated neural tissue, site of encephalocoele, state of CSF pathway, and relationship of superior sagittal sinus with the herniated sac. [ 10 11 ] Intraoperative complications such as blood loss, hypothermia, and electrolyte imbalance can be avoided by delaying the surgery for 7–8 months, which is not always possible, especially in the presence of CSF leak and in giant encephalocoeles, which ultimately end up in skin erosion with CSF leak. [ 1 2 ] To prevent bradycardia and cardiac arrest in a neonate due to sudden loss of CSF, slow controlled tapping of CSF is recommended.…”

Section: Discussionmentioning

confidence: 99%

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Giant anterior fontanel encephalocoele: A rare case report and review of literature

et al. 2020

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A BSTRACT An encephalocoele is a spectrum of cranial dysraphism caused due to defective closure of neural tube during early embryonic life, leading to formation of a cerebrospinal fluid (CSF)–filled sac containing brain tissue and meninges, all herniated through a bony defect of the skull. Anterior encephalocoeles, though rare in the Western world, are relatively common in southeast Asia, including some parts of India. Among anterior encephalocoeles, fronto-ethmoidal type is the most common followed by orbital encephalocoeles. Giant encephalocoeles are rare with few published short series, which are mostly located in occipital region. Giant interfrontal encephalocoele through a wide anterior fontanel is the rarest one and is limited to three case reports, including this index case. Here we report a case of giant anterior fontanel encephalocoele in a 20-day-old neonate and discuss various aspects of its management.

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Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Giant anterior fontanel encephalocoele: A rare case report and review of literature

et al. 2020

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“…When giant encephalocele are associated with microcephaly and micrognathia, it is a predictor of poor outcome. 5 In these patients, there can be associated lesions including agenesis of corpus callosum, hypoplasia of cerebellum, meningocele, and hydrocephalus. Coexistent hydrocephalus may occur due to torsion of the aqueduct or aqueductal stenosis.…”

Section: Discussionmentioning

confidence: 99%

“…7 Strategy regarding surgical intervention depends upon the amount of neural tissue in the sac, state of CSF pathway, neurological status of the patient and presence or absence of associated congenital anomalies elsewhere in body. 5,7 Presence of large occipital sac poses difficulty in positioning for intubation in these patients. To prevent any compression and rupturing the sac, the child cannot be positioned supine.…”

Section: Imjmmentioning

confidence: 99%

Surgical Management of Large Occipital Encephalocoele: A Case Report

Sim¹,

Theophilus²,

Azman³

2014

imjm

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Cranium dysraphism occur less commonly than spinal dysraphism. Overall, occipital encephalocoeles are more frequent than anterior encephalocoes. A large occipital encephalocoele possess difficulty in surgical intervention, not only the sac contains large amount of herniated brain tissue, but the appropriate positioning for successful intubation as well as the higher rate of surgical complications such as cerebrospinal fluid leakage and skin necrosis. In this case report, surgical management of large occipital encephalocoele is discussed. The use of antibiotics in ruptured encephalocoele is recommended.

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“…• May be progressive if hydrocephalus develops after birth. • May be associated with Chiari malformations or other syndromes (e.g., Meckel-Gruber syndrome) 9. …”

mentioning

confidence: 99%