A giant occipital encephalocele in neonate with spontaneous hemorrhage into the encephalocele sac: Surgical management

Abstract

The presence of giant occipital encephalocele represents a surgical challenge. However, preoperative magnetic resonance imaging with venography can help in delineating relation of venous sinus, content of the sac and help classify occipital encephalocele into infra-torcular and torcular depending on the relation with position of torcula. However, the presence of old hemorrhage into encephalocele sac is extremely rare and in the detailed PubMed search, the authors could find one such case, reported by Nath et al. The author reports a case of giant occipital encephalocele; during surgery, evidence of old bleed was noted. Pertinent literature and management are reviewed briefly.

Occipital Encephalocele: Cause, Incidence, Neuroimaging and Surgical Management

“…In the USA, the incidence is between 1 in 3000 to 1 in 10,000 live births; approximately 90% of them involve the midline [ 1 - 3 , 19 ], and 70% of them occur in females [ 6 , 7 ]. Solitary encephalocele is a sporadic phenomenon and many of them may be part of the genetic syndrome (Meckel-Gruber syndrome, Von Voss-Cherries syndrome, Valker-Varburg syndrome, Knobloch syndrome, Jouberg syndrome, Valker Varburg syndrome) [ 2 , 6 , 19 ]. Hydrocephalus is an important clinical manifestation and is estimated to be identified in about 60% of all newborns with OE [ 4 ].…”

“…Posterior encephaloceles are frequently associated with neurological problems. Some infants may be asymptomatic on physical examination [ 6 , 11 , 19 ], but others might harbor a numerous and different signs and symptoms such as delays in reaching developmental milestones, intellectual disability, learning disabilities, growth delays, seizures, vision impairment, uncoordinated voluntary movements (ataxia), hydrocephalus, spastic paraplegia or quadriplegia and microcephaly [ 3 , 5 , 17 ].…”

“…MRI coupled with magnetic resonance angiography (MRA) is a beneficial method in the visualization of the contents of the sac and its relationship to venous sinuses [ 22 ]. MRI can usually depict the dural venous anatomy, and with MRI venography additionally improving the interpretation of the relationship between OE and venous sinuses, MRI represents a superior method in the diagnosis of dilated venous sinuses [ 2 , 19 , 21 ] (Figs. 1,2,5 ).…”

“…The amount of brain tissue in the sac of OE, the state of CSF pathways, the degree of neurological deficit of the patients, and the existence (or non-existence) of the associated congenital malformation elsewhere in the body are the relevant factors for the management of the newborns with this lesion [ 2 ]. Surgical indication involves several conditions and should be considered in cases where there is a risk of rupture of the bag, leakage of CSF, meningitis, the content of prolapsed brain tissue, the presence of venous sinus, hydrocephalus, associated brain anomalies and aesthetic-cosmetic appearance [ 4 , 19 ]. Dysplastic, gliosed, non-functional brain tissue should be excised, taking care of the essential vascular and brain structures [ 6 , 11 , 17 , 19 ].…”

“…Surgical indication involves several conditions and should be considered in cases where there is a risk of rupture of the bag, leakage of CSF, meningitis, the content of prolapsed brain tissue, the presence of venous sinus, hydrocephalus, associated brain anomalies and aesthetic-cosmetic appearance [ 4 , 19 ]. Dysplastic, gliosed, non-functional brain tissue should be excised, taking care of the essential vascular and brain structures [ 6 , 11 , 17 , 19 ]. Lesion of torcula may provoke cerebral deep venous thrombosis.…”