2025 Proffered Presentations
S280: INTERDISCIPLINARY MANAGEMENT OF A TRANSETHMOIDAL MENINGOCELE IN A NEWBORN
Rose Fluss, MD; Anne R Lally, BA; Jinu Kim, MD; Andrew J Kobets, MD; Albert Einstein College of Medicine
Introduction: Encephaloceles are a type of open neural tube defect in which there is herniation of brain matter and meninges through a structural deformity in the skull; meningoceles describe herniation of meninges alone. Basal encephaloceles are an extremely rare congenital abnormality that frequently present with congenital dyscrasias and ocular anomalies. Comparably, meningoceles are unusual lesions that often present with midfacial anomalies and hypertelorism. Here, we describe a case of a preterm newborn monochorionic-diamniotic twin with a transethmoidal meningocele, midline cleft lip and palate, slow feeding, absence of the right orbit, and microphthalmia, who could not maintain her airway and was managed by an interdisciplinary surgical team.
Case Presentation: At 34 weeks gestation, an elective cesarean section was performed for a monochorionic-diamniotic gestation. The second twin had a large facial opening with a suspected encephalocele. This opening was draining clear, yellow fluid, which was concerning for cerebral spinal fluid. In addition, the patient was born with a patent ovale and patent ductus arteriosus. Due to the absence of spontaneous breathing, copious fluid drainage, and a facial abnormality which prevented the utilization of mask ventilation, the patient was immediately intubated.
Imaging with MRI revealed a severe malformation of the left face, left orbitofrontal region, as well as the left mandibular body. A discrete left globe was not identified. A dysplastic left nasal cavity and nasopalatine cleft was apparent. Arising from the left nasal cavity was a T2 hyperintense fluid-filled structure extending from a short segment calvarial defect along the left anterior cranial fossa, that appeared to communicate with the meninges. Since there was no evidence of brain herniation, the lesion was then suspected to be a meningocele.
After the first 15 days of life in the neonatal intensive care unit, the patient underwent stage 1 of her operative procedures with neurosurgery, otolaryngology, and plastic surgery all present. Otolaryngology created a tracheostomy for a more sustainable airway and larger operating surface area. Plastic surgery worked within the oral cavity to separate the meningocele sac from oral mucosa. Otolaryngology worked transnasally. A midline nasal split was performed to facilitate dissection through the open cleft lip and nose. Plastics opened a portion of the face, which facilitated better access to the lesion. The protruded meningocele was isolated and sutured off at its origin in the left anterior cranial fossa. Bipolar cautery was used and the lesion was removed en bloc. Fibrin glue and a vascularized graft were used to seal off the area. Sutures were placed to secure this and secure the skin of the face.
Discussion: In neonates, surgical repair of a cephalocele is rare. The risks of hypothermia and blood loss outweigh the benefits of immediate repair unless the infant has CSF rhinorrhea, difficulty breathing, or difficulty feeding. By intervening very early, the team could not perform the typical surgical repair: a craniotomy, pericranial graft, skull base and transoral mucosal repair. This repair, although not the classical approach to definitive repair, was the safest option given our patient’s age.