Repair of Frontoethmoidal Encephalocele in the Philippines: An account of 30 cases between 2008-2013

• Published in: World neurosurgery 2017-07, Vol.103, p.19-27
• Main Authors: Marshall, Amanda-Lynn, M.P.H, Setty, Pradeep, D.O, Hnatiuk, Mark, M.D, Pieper, Daniel R., M.D
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Arachnoid Cysts - epidemiology; Arnold-Chiari Malformation - epidemiology; Child; Child, Preschool; Comorbidity; Encephalocele - diagnostic imaging; Encephalocele - epidemiology; Encephalocele - surgery; Ethmoid Bone - diagnostic imaging; Ethmoid Bone - surgery; Female; Foramen cecum; Frontal Bone - diagnostic imaging; Frontal Bone - surgery; Frontoethmoidal encephalocele; Humans; Hydrocephalus - epidemiology; Infant; Male; Medical Missions, Official; Nasal Bone - diagnostic imaging; Nasal Bone - surgery; Neural tube defect; Neurosurgeons; Neurosurgery; Operative Time; Oral and Maxillofacial Surgeons; Otolaryngologists; Patient Care Team; Pediatric craniofacial reconstruction; Philippines; Postoperative Complications - epidemiology; Prefrontal Cortex - diagnostic imaging; Prefrontal Cortex - surgery; Reconstructive Surgical Procedures; Skull base; Surgery, Plastic; Tomography, X-Ray Computed; Treatment Outcome; Young Adult
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2017.03.063

Abstract Background Frontoethmoidal encephalocele is a congenital abnormality of the anterior skull base involving herniation of cranial contents through a midline skull defect. Patency of the foramen cecum, along with other multifactorial variables, contribute to the development of frontoethmoidal encephaloceles. Due to limited resources, financial constraints, and lack of surgical expertise, repair of frontoethmoidal encephaloceles are limited in developing countries. Methods Between 2008-2013 an interdisciplinary team composed of neurosurgeons, craniofacial surgeons, otolaryngologists, plastic surgeons and nursing personnel, conducted surgical mission trips to Davao City in Mindanao, Philippines. All patients underwent a combined extracranial/intracranial surgical approach, performed in tandem by a neurosurgeon and a craniofacial surgeon, to detach and remove the encephalocele. This was followed by reconstruction of the craniofacial defects. Results A total of 30 cases of frontoethmoidal encephalocele were repaired between 2008-2013 (20 male: 10 female). The average age at operation was 8.7 years, with seven patients over the age of 17 years. Of the three sub-types, the following breakdown was observed in patients: 18 nasoethmoidal; 9 nasofrontal; and 3 naso-orbital. Several patients demonstrated concurrent including enlarged ventricles, arachnoid cysts (both unilateral and bilateral), gliotic changes, as well as orbit and bulbus oculi (globe) deformities. There were no operative-associated mortalities or neurological deficits, infections or hydrocephalus on follow up during subsequent trips. Conclusions Despite the limitations of performing advanced surgery in a developing country, the combined interdisciplinary surgical approach has offered effective treatment to improve physical appearance and psychological well-being in afflicted patients.