Novel Surgical Technique for Correction of Incomplete Median Cleft Lip Deformity in Oral-Facial-Digital Syndrome Type II.

ABSTRACT: Oral-facial-digital syndromes (OFDSs) represent a heterogenous group of embryonic development disorders characterized by malformations of the face, oral cavity, and extremities. Oral-facial-digital syndrome type II is an autosomal recessive disease characterized by median cleft lip, gingival frenula, cleft lobulated tongue, and polydactyly. There are few reports on surgical techniques for correction of incomplete median cleft lip. Here we describe a novel surgical method that we used to correct an incomplete median cleft lip in a 5-year-old girl with oral-facial-digital syndrome type II. She had previously undergone surgery for congenital heart disease, oral anomalies, and polydactyly. Cheiloplasty was performed at 5 years and 8 months using a surgical approach that focused on repair of the median tubercle using lateral labial elements. A reasonably good Cupid's bow and median tubercle were achieved. Our technique for surgical correction of moderate incomplete median cleft lip provides adequate philtral height, vermillion fullness, and a good-shaped Cupid's bow.

Ulcerated Infantile Hemangioma of the Hard Palate: Diagnostic Treatment With Oral Propranolol.

Infantile hemangiomas arising in the palate are rare. The authors describe a case of ulcerated infantile hemangioma of the hard palate with feeding difficulty. To our knowledge, this is the first reported case of immunohistochemically diagnosed palatal infantile hemangioma successfully treated using oral propranolol.

Dental arch relationship outcomes in one- and two-stage palatoplasty for Japanese patients with complete unilateral cleft lip and palate.

OBJECTIVE: To compare dental arch relationship outcomes following one- and two-stage palatal repair. DESIGN: Nonrandomized, clinical trial with concurrent control. SETTING: Hokkaido University Hospital. PATIENTS: Sixty-eight consecutively treated Japanese patients with complete unilateral cleft lip and palate. INTERVENTIONS: Thirty-one of the 68 patients underwent two-stage palatoplasty with delayed hard palate closure, and 37 patients underwent one-stage pushback palatoplasty. MAIN OUTCOME MEASURES: Dental casts were taken at 4.9 to 6.3 (mean: 5.2) years of age in the two-stage group and at 4.0 to 6.3 (mean: 5.1) years of age in the one-stage group, and dental arch relationships were assessed using the 5-Year-Olds' Index (5-Y) by four raters and the Huddart/Bodenham Index (HB) by two raters. RESULTS: Intrarater and interrater reliabilities evaluated using weighted kappa statistics were good or better for the 5-Y and HB ratings. The mean 5-Y score was 2.94 in the two-stage group and 3.13 in the one-stage group (P value was not significant). However, there was a significant difference in distributions between the groups (P < .05). The HB scores of molars were significantly greater in the two-stage group than in the one-stage group (P < .05). The rank correlation coefficients between the 5-Y and total HB score (ρ = -0.840, P < .01) and between the 5-Y and the score of the incisors in the HB (ρ = -0.814, P < .01) were significantly increased. CONCLUSIONS: These results suggest that the anteroposterior relationship was not significantly different between the groups, but the transversal relationship was better in the two-stage group than in the one-stage group.

Effectiveness and safety of percutaneous sclerotherapy using absolute ethanol and/or polidocanol for maxillofacial venous malformations involving the masticatory muscles: A case series.

OBJECTIVE: This study evaluated the effectiveness and safety of percutaneous sclerotherapy for maxillofacial venous malformations. STUDY DESIGN: Patients who had venous malformations involving the masticatory muscles and who underwent sclerotherapy were enrolled in this retrospective study. RESULTS: Twenty-four patients (13 female, 11 male; mean age 21 years) were analyzed. Major clinical symptoms were swelling (100%) and intralesional pain (54%). Intramuscular lesions involved the masseter muscle only in 38% of cases, both the masseter and temporalis muscles in 33%, all masticatory muscles in 21%, and the temporalis muscle only in 8%. Extramuscular involvement was observed in 58% of patients. Absolute ethanol and polidocanol were used as sclerosants. The mean number of sclerotherapy sessions per patient was 6.6 (range, 1-32). The mean follow-up duration after the first sclerotherapy session was 64.8 months (range, 6-178). The complications included paralysis of the facial nerve (25%), intraoral ulceration (8%), and hemoglobinuria (8%). The effectiveness of treatment was rated as excellent in 33% of cases, good in 46%, and fair in 21%. Better results were obtained in patients without extramuscular involvement. CONCLUSION: Percutaneous sclerotherapy can be effective and safe for maxillofacial intramuscular venous malformations, especially for localized lesions of the masseter muscle.

Combination of Tessier clefts 3 and 4: case report of a rare anomaly with 12 years' follow-up.

Tessier cleft types 3 and 4 are both rare craniofacial anomalies. Here we present the first case of a girl born with a combined anomaly of Tessier clefts 3 and 4 with severe bilateral cleft lip, a displaced premaxilla, and three-dimensional underdevelopment of the hard and soft tissues of the maxilla and zygoma. This type of rare facial cleft poses a major operative challenge. Over a period of years, presurgical alveolar molding with an active appliance was followed by seven operations. A satisfactory esthetic outcome was obtained. A multidisciplinary approach to treatment with a plastic surgeon in charge of the operations and an orthodontist in charge of the cleft deformity is essential.