Fetal surgery for occipital encephalocele: A case report

Abstract

Background: Occipital encephalocele is a congenital defect of the neural tube at the level of the cranial midline, which results in herniation of meninges and brain tissue. The results of the management of myelomeningocele study determine the maternal and fetal risks for an open fetal surgery and have motivated the constant review of the concepts and strategies which the pediatric neurosurgeon can employ for the treatment of neural tube defects in the prenatal period.

Case description: We present a case of a female patient in utero of 26 gestational weeks with the diagnosis of an occipital encephalocele treated by open fetal surgery. During week 20 of gestation, the diagnosis of occipital encephalocele was made by ultrasound, which was corroborated by fetal magnetic resonance that showed cranial protrusion of neural and meningeal content in the occipital region, measuring 1.6 × 2.8 × 3.3 cm with an approximate volume of 7.7 cc through a bone defect of 6 mm. The closure of the defect was performed by the postnatal surgical technique adapted to the open fetal surgery. Later, the patient was born transabdominal with a 2.8 cm occipital wound, with suture points and approximated borders, normocephalic, without clinical signs of sepsis, hydrocephalus, or overt neurologic compromise.

Conclusion: Open fetal surgery is a therapeutic option in the face of an isolated occipital encephalocele. This case report demonstrates the viability of the surgical procedure by the adaptation of a postnatal surgical technique to a prenatal surgery. Further studies are needed to evaluate the long-term functional results, comparing them with those seen in patients who undergo a postnatal procedure.

Keywords: Cranial dysraphism; Fetal surgery; Occipital encephalocele.

Figure 1:

Obstetric ultrasound during week 20 of gestation with occipital encephalocele.

Figure 2:

Sagittal view of fetal magnetic resonance T2 weighted image showing an occipital protrusion measuring 1.6 × 2.8 × 3.3 cm

Figure 3:

Axial view of fetal magnetic resonance T2 weighted image showing an occipital protrusion with a bone defect of 6 mm.

Figure 4:

Coronal view of fetal magnetic resonance T2 weighted image showing an occipital protrusion with an estimated volume of 7.7 cc.

Figure 5:

Surgical technique. (a) Localization of placenta by transoperative echography, planning of mini hysterotomy. (b) A 3 cm mini hysterotomy and widening by a Weitlaner retractor. (c) Exposure of occipital encephalocele. (d) Midline incision of encephalocele sac and exposure of meninges. (e) Tight closure of meninges by a 5-0 propylene running suture. (f) Complete closure of occipital defect without leak of cerebrospinal fluid (CSF). (g) Postnatal wound of clinical case with borders closed with a running suture, without leak of CSF.