Giant Frontoethmoidal Encephalocele in an Infant: A Case Report

TL;DR

A three-month-old infant with a rare giant brain tumor underwent successful surgery and showed normal development after seven years.

Contribution

This case report presents a rare and successful surgical management of a giant frontoethmoidal encephalocele in an infant.

Findings

The infant underwent two-stage surgery with no post-surgical complications.

Seven years post-surgery, the patient showed normal physical and cognitive development.

The craniofacial reconstruction was achieved using the same skin without additional flaps.

Abstract

Giant encephalocele is a rare pediatric surgical entity that poses unique challenges. Few cases have been reported in the medical literature, and its cause is unknown. Factors that increase the likelihood of developing this pathology are radiation, infections, hyperinsulinemia, vitamin deficiencies related to neural tube closure defects, maternal smoking, alcohol, and anticonvulsants. A three-month-old female infant, with no factors associated with the current condition, with poor prenatal control, diagnosed with giant frontoethmoidal encephalocele, and with the presence of a frontal tumor of approximately 15 cm in its major axis, underwent surgery by a neurosurgeon and a plastic surgeon to correct the craniofacial deformity. The first approach was performed by removing the tumor with the closure of the meninges and skull. Then in the second surgical time, craniofacial reconstruction was performed by the plastic surgery team, using the same skin without the need for flaps of any other structure and without the presence of complications. The patient had no post-surgical complications. After seven years of medical follow-up, she has a normal staturoponderal and psychomotor development, no intellectual deficit, and adequate aesthetic results.

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