Prevalence: about 1/5000 births. Defect of closure of the cephalic part of the neural tube (4th week of gestation) which causes the protrusion of meninges (meningocele or encephalocele if they are accompanied by brain tissue) at the level of a portion of the skull.

The most frequent locations are:

-         the occiput (75%): the defect is located on the midline in 90 % of cases

-         the  fronto-ethmoidal region ( syncipital encephalocele): nasofrontal, nasoethmoidal, nasoorbital (figure)

-         the basal region (1-10%): transethmoidal: nasal obstruction (sometimes hypertelorism, cleft lip and palate, bifid nose)

-         the parietal region

The encephaloceles are associated with other neurological abnormalities in more than 60% of the cases: 

-         hydrocephalus, microcephaly

-         developmental disorders

-         convulsions

-         spina bifida

-         Dandy-Walker syndrome

-         Arnold-Chiari type II malformation.

Ocular abnormalities are frequent in case of encephalocele in the nasal region.

Except in case of leakage of CSF, the surgical closure is performed as an elective procedure. The most frequent postoperative complications are hydrocephalus and meningitis.

The cure of an occipital encephalocele is performed in the prone position.

Some teams correct nasal encephaloceles endoscopically (endonasal approach).

    nasal encephalocele

Anesthetic implications: 

-        in case of a defect in the  fronto-ethmoidal region: check the pituitary function (thyroid, growth hormone, diabetes insipidus, etc)

-        anticipate difficulties

1)        in case of encephalocele in the nasal region : for ventilation with a facemask

2)        in case of occipital encephalocele : for positioning for induction and intubation :

-        lateral decubitus position

-        or head held by a helper at over the edge of the operating table,

-        or partial needle decompression in case of a giant mass of fluid

-        compression of the mass can cause acute intracranial hypertension. There may be significant hemodynamic changes during the incision (abrupt loss of a large volume of CSF and pressure  changes at the level of the brainstem) and/or at the reintegration of the extruded part of the brain in the skull (intracranial hypertension).

References : 

-         Leelnukrom R, Wacharasint P, Kaewanuchit A. 
Perioperatve management for surgical correction of frontethmoidal cephalomeningocele in children: a review of 102 cases. 
Pediatr Anesth 2007; 17: 856-62.

-         Arora S, Bharti N, Bala I. 
Sudden cardiac arrest during repair of giant occipital encephalocele with microcephaly. 
J Anaesth Clin Pharmacol 2010; 26: 259-60.

-         Mahajan C, Rath GP, Dash HH, Bithal PK. 
Perioperative management of children with encephalocele: an institutional experience. 
J Neurosurg Anesthesiol 2011; 23: 352-6.

-         Vasudevan A, Kundra P, Priya G, Nagalakshmi P. 
Giant occipital encephalocele : a new paradigm. 
Pediatr Anesth 2012 ; 22 : 586-8

-         Black SA, Galvez JA, Rehman MA, Schwartz AJ. 
Images in Anesthesiology : airway management in an infant with a giant occipital encephalocele.
Anesthesiology 204: 120:1504

-         Nhushan RV, Pratibha CB, Balasubramanya AM, Nandakumar R, Reunold R. 
Endoscopic management of nasal encephalocele in a 4-month-old baby. 
Int J Pediatr Otorhinolaryngol Extra 2014; 9: 67-9. 

-        Fandino A, Herrera AJ, Feo OE, Ronderos AM, Ospina JC, De Leon O.
Congenital meningocele associated with corpus callosum agenesis and midline superior labial cleft due to Tessier 0-14 facial fissure : a case report.
Int J Pediatr Otorhinolaryngol 2020; 130: in press

Updated: February 2020