Correction of frontonasoethmoidal encephalocele: the HULA procedure

Abstract

Background: The frontonasoethmoidal encephalomeningocele deformity involves central herniation of a glial mass that "pushes outward" and deforms the medial orbit, medial canthus, nasomaxillary process, and nasal structures without resulting in hypertelorbitism. The authors studied a modification of the "Chula" repair, called the HULA procedure (H = hard-tissue sealant, U = undermine and excise encephalocele, L = lower supraorbital bar, A = augment nasal dorsum), which provided complete correction of the midline hard and soft-tissue structures using an intracranial and extracranial approach.

Methods: Filipino patients with frontonasoethmoidal encephalomeningoceles were treated by a civilian/military humanitarian team at Tripler Army Hospital (n = 12). Operative technique followed the four steps of the HULA frontoethmoidal encephalocele procedure. Postoperative and follow-up assessments were based on examination, photographic images, computed tomography scans, parental surveys, Whitaker score, and developmental testing.

Results: Patient ages ranged from 5 to 12 years; 67 percent were female and 33 percent male. Sixty-seven percent required excisions of poor-quality, hyperpigmented skin along with the large glial mass; the other 33 percent had a "closed" resection of the smaller mass through a gingivobuccal sulcus incision. No patients manifested cerebrospinal fluid leaks, infection, or elevated intracranial pressures postoperatively. Skeletal correction showed improved medial orbit distance, with a mean correction of 14 mm (42 percent). Whitaker score was 1.3 (no or minor soft-tissue revision necessary). Parental survey showed a high degree of satisfaction with the aesthetic and functional outcomes. Follow-up developmental tests showed normal global evaluations for all but one child with normal memory and attention skills.

Conclusion: The authors' outcomes demonstrated that the HULA technique was a safe and effective approach for the complete correction of frontonasoethmoidal encephalomeningoceles.