Neurosurgical interventions for occipital encephalocele

Abstract

Aims and Objectives:The aim of this study is to find the outcome of repair and resection of the occipital encephalocele.Study Design:Case series.Materials and Methods:The clinical data of fifty consecutive occipital encephalocele patients were retrieved from medical records including operative notes, postoperative follow-up visits, and postsurgical complications were noted for analysis from November 2009 to November 2013 at the Department of Neurosurgery, Jinnah Postgraduate Medical Centre, Karachi, Pakistan. All patients were assessed by computed tomography scan, magnetic resonance imaging brain, and ultrasound when needed. Physician's assessment, physical examination, and his/her questions to the family at follow-up were used as a tool to determine if there was a developmental delay rather than quantitative analysis like hydrocephalus questionnaires. Patients who developed complications and delayed milestone were regarded as no improvement and those who did not develop complications and achieved appropriate milestone were regarded as improved at 18 months follow-up.Results:Of 50 patients, 17 were males and 33 were females. The average age at presentation was 2.4 months. 16 (32%) patients had increased head circumference and hydrocephalus, 2 (4%) had associated Dandy–Walker cyst, 3 (6%) developed developmental delays, and 8 (15%) had a seizure disorder. None of our patients had neurological deficits. The size of the sac ranged from 2 cm × 3 cm to 27 cm × 15 cm. 9 (18%) patients were admitted with the complication of sac rupture and 2 (4%) patients sac ruptured after admission. Only one patient (2%) had a cerebrospinal fluid leak postoperatively that was repaired primarily without patch graft or dura seal while 4 (8%) developed hydrocephalus after repair of the sac which was treated with placement of ventriculoperitoneal shunt. One (2%) patient did not recover from anesthesia and expired.Conclusion:Encephalocele is commonly seen in the practice of neurosurgery in the world as well as in Pakistan. Modern neuroimaging, neurosurgical techniques, and neonatal neurological intensive care have greatly improved morbidity and mortality in the care of encephalocele.

Occipital Encephalocele: Cause, Incidence, Neuroimaging and Surgical Management

“…Even certain toxins or infections might play a role in its evolution [ 3 ]. Maternal levels of folate, as a protective factor, are relevant to myelomeningocele but its relevance for E is still ‘not clear’ [ 17 ]. The use of folic acid (400 micrograms of folic acid daily) in the diet of the pregnant women’ might lower the risk of some neural tube defect’ [ 3 ].…”

“…Encephalocele account for 10% - 20% of all craniospinal dysraphism [ 6 ]. The published estimates vary in the range of 1/5000 live births to approximately 1 in 10,000 babies each year; or about 0.8–5.6/10,000 live births [ 1 , 3 , 11 , 17 , 18 ]. Occipital encephalocele is the most common form of the anomaly and is present in the range of 80-90% of all (E).…”

“Cable suturing technique” a dural obliteration method for the prevention of cerebellar herniation through a large occipital meningocele

“…Encephalocele (also known as cranium bifidum) is considered as incomplete cranial neuropore closure during neural tube formation (day 24 gestational age) where intracranial structure herniates outside skull confines,[123456] including brain tissues, meninges, and cerebrospinal fluid (CSF). [578]…”

“…But, although autologus (expansile) cranioplasty has good outcome in preserving brain parenchyma,[1012] it has a disadvantage of relative risk of bleeding, donor site morbidity as well as risk of resorption, thus some authors would advocate a later - staged - cranioplasty. [13514151617]…”

“…The prognosis of occipital encephalocele mainly depends on the size and the amount of herniated tissues, as large amount can increase the risk of mental and physical retardation,[1521] chances of CSF leakage, and wound infection. [3522]…”