Giant Occipital Encephalocele in an Infant: A Surgical Challenge.

Giant occipital encephalocele is an uncommon condition. The sheer size of the lesion poses a challenge to the surgeon and the anesthetist. Here, we present a 4-month-old girl child presenting with a progressively increasing swelling in the occipital region since birth. The child after evaluation underwent repair of the encephalocele. The purpose of this report was to highlight the difficulties faced during surgery while operating on such a huge occipital encephalocele. Copyright:

Introduction

Encephalocele is a broad term for herniation of cranial contents through the defect in cranium. If only meninges and cerebrospinal fluid (CSF) are herniated, they are referred to as meningocele. If both the meninges and neural elements are herniated, they are referred to as meningomyelocele. The incidence of encephalocele is 1 in 5000 live births.[1] Occipital encephalocele is the most common variety of encephalocele. In case the size of the head is same or smaller to the encephalocele sac, it is referred to as giant encephalocele.[1]

Here, we present the challenges faced during surgery while operating a 4-month-old girl child with a giant occipital encephalocele.

Case History

A 4-month-old girl child presented with a history of swelling in the back of the head since birth. The child was delivered by cesarean section at term. The parents were counseled about the swelling in the head in the prenatal period. The swelling was gradually increasing in size. The child was examined at birth at their hometown, but was advised to wait till 3 months by a pediatrician. She was taking feeds normally and moving all the four limbs. She did not have any other complaints.

On examination, the child was active. A large swelling was present in the occipital region measuring 20 × 15 × 18 cm [Figures 1 and 2]. The overlying skin was intact, and in few areas, it showed pressure necrosis. The swelling was tensely cystic and transilluminant [Figure 3]. The anterior fontanel was wide open. On palpation, there was a breach in the underlying cranium at the occipital region. Visual examination of the child was normal. She was moving all the four limbs, and the power in the limbs was normal. With the aforementioned clinical findings, a diagnosis of occipital encephalocele was made.

Figure 1

Occipital encephalocele

Figure 2

Lateral view of encephalocele

Figure 3

Transillumination of encephalocele

Blood investigations were within normal limits. Magnetic resonance imaging performed was suggestive of occipital encephalocele with small amount of brain tissue. After obtaining an informed consent from the parents, the child was taken for surgery.

The child had to be taken to the edge of the table with head hanging for intubation. After intubation, the child was placed in prone position. The encephalocele was excised and the dura was identified and a watertight closure was carried out. Postoperatively, the child was extubated, and she was started on feeds on postoperative day 2. It was noticed on postoperative day 8 that the anterior fontanel of the child was tense and bulging. A plain computed tomography (CT) of the brain was suggestive of hydrocephalus. The child underwent a medium-pressure ventriculoperitoneal (VP) shunt. After shunt surgery, she had an uneventful recovery [Figure 4]. She was discharged on postoperative day 15 of the first surgery. She is on regular follow-up since 2 years after surgery. Recent CT of brain carried out was normal [Figure 5]. The child’s condition is stable with mild developmental delay [Figure 6].

Figure 4

After excision and VP shunt placement

Figure 5

CT scan after two years showing VP shunt in situ

Figure 6

Child at two years of follow-up

Discussion

Encephalocele accounts for 10%–20% of all craniospinal dysraphisms.[2] A total of 70% of occipital encephaloceles occur in females.[3] These lesions are usually covered either with normal skin, dysplastic skin, or a thin, distorted meningeal membrane. Encephaloceles could be pedunculated or sessile.[4] The content of occipital encephalocele may include meninges, occipital lobes, or ventricles, and may rarely contain cerebellum, brain stem, or torcula.[5] The neurological prognosis in children depends on the amount of neural tissue that has herniated through the sac. The large-sized swellings may have significant brain herniation, abnormality of the underlying brain, microcephaly, and ventriculomegaly. Such patients usually have poor prognosis.

Surgery in these children is a challenge to both the anesthetist and the operating surgeon. The decision regarding surgery depends on various factors including the amount of neural tissue in the sac and other congenital anomalies. Large encephalocele with minimal or no neural tissue has excellent prognosis. Surgical management requires careful attention to pediatric anesthetic and surgical principles. The loss of large quantity of CSF causes electrolyte disturbances, which need to be corrected perioperatively. Other considerations to be taken care of include blood loss, maintenance of body temperature, prone position and its associated complications, and careful securing of the endotracheal tube.[6] All gliotic tissues and ischemic neural contents are excised taking care of the sinuses that may course through it. Great emphasis is laid on obtaining a two-layered watertight closure of the dura. In our case, the problems faced were because of large size of encephalocele, prone position, intubation, blood loss during surgery, and redundant skin. All the aforementioned difficulties were addressed and the child had an uneventful recovery. Postoperatively, in our case, she developed hydrocephalus, which required VP shunt. This is due to the changes in CSF dynamics after surgery.

Conclusion

The overall prognosis of occipital encephalocele depends on the extent and nature of herniated contents and associated anomalies. Many large encephaloceles have an excellent prognosis despite their size.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.