Correction of frontonasoethmoidal encephalocele: the HULA procedure.
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.


CONCLUSIONS
The authors' outcomes demonstrated that the HULA technique was a safe and effective approach for the complete correction of frontonasoethmoidal encephalomeningoceles.
Kumar
Anand
A
Los Angeles, Calif.; Honolulu, Hawaii; and Bethesda, Md. From the Division of Plastic and Reconstructive Surgery, David Geffen School of Medicine at the University of California; Bethesda Naval Hospital; Tripler Army Hospital; and West Los Angeles Kaiser Permanente.
Helling
Eric
E
Guenther
Dax
D
Crabtree
Tom
T
Wexler
Andrew W
AW
Bradley
James P
JP
D002648:Child; D002675:Child, Preschool; D004677:Encephalocele / Q000601:surgery*; D005004:Ethmoid Bone / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005624:Frontal Bone / Q000601:surgery*; D006801:Humans; D008297:Male; D009666:Nose / Q000601:surgery; D009915:Orbit / Q000601:surgery; D017060:Patient Satisfaction; D019651:Plastic Surgery Procedures / Q000379:methods*