Background: Frontonasoethmoidal encephalomenigoceles, rarely seen in the Western Hemisphere, remains a challenging clinical entity. This midline facial deformity involves a central herniation of a glial mass which �pushes outward' and deforms the medial orbit, medial canthus, nasomaxillary process and nasal structures without resulting in hypertelorbitism.� The �Chula' repair, named after King Chulalongkorn of Thailand, and other techniques focus on extracranial resection and nasal dorsal reconstruction.� In the HULA repair we describe resection of the deforming mass, repair of the cranial base defect and complete reconstruction of the midline hard and soft tissue structures.

Methods:� Filipino patients diagnosed with frontonasoethmoidal encephalomenigoceles were treated by a multidisciplinary team during joint civilian/military humanitarian missions at Tripler Army Hospital (n=12).� Demographic, medical history and CT scan information was recorded.� Operative technique evolved and varied slightly depending on the deformity but followed tenets of the HULA Frontoethmoidal Encephalocele Correction (Figure 1): H = Hard split cranial bone, pericranial flap, Fibrin Glue sealant after ligation of cyst stalk and dura repair; U = Unification of supraorbital bar after midline resection and inward rotation; L = Low nasal radix position for full thickness cantilever bone graft; A = Adjustment of medial canthal position and nasal maxillary process after resection of midline accessory nasal bone. Postoperative and follow-up assessment was based on examination, photographic images, CT scans, parental surveys, the Whitaker reconstructive score and developmental testing.

Results: Patients ranged in age from 5 to 12 years; there were 67% females and 33% males.� All cases involved frontoethmoidal or nasoethmoidal defects. Sixty-six percent of patients underwent central nasal skin excisions of �damaged,' or poor quality, hyperpigmented skin.� Patients with undamaged nasal skin did not require an external skin incision but instead had bilateral maxillary gingivobuccal sulcus incision for removal of the central mass.� In these patients an intercartilagenous nasal incision was used to place the cantelever bone graft.� After the HULA procedures no patients manifested cerebrospinal fluid leaks, infection, or elevated intracranial pressures.� Follow up at 6 months-4 years revealed satisfactory correction of the deformities (mean Whitaker score of 1.3=no or minor soft tissue revision only necessary) (Figure 2).� Parental survey showed very good to excellent aesthetic outcome with a high degree of satisfaction.� Follow-up developmental tests showed normal scores in preschool tests (mean score=102) and global evaluations with normal memory and attention skills.

Conclusion: Our outcomes demonstrated that the HULA technique was a safe and effective approach to correct the complex craniofacial anomaly of frontoethmoidal encephalomenigoceles.