Patient-reported quality of life in highest-functioning Apert and Crouzon syndromes: a comparative study.

BACKGROUND: Crouzon and Apert syndromes are the most common syndromic forms of craniofacial dysostosis. Apert syndrome has a broad clinical spectrum, including complex craniofacial involvement, as well as limiting deformities of the hands, feet, and other joints that require multiple surgical procedures when compared with Crouzon syndrome, which is generally less severe. The authors hypothesized that the quality of life of Apert syndrome patients is inferior to that of Crouzon syndrome patients. METHODS: The quality of life of Apert (n = 8) and Crouzon (n = 12) syndrome patients was assessed using the World Health Organization Quality of Life-100 questionnaire. The Mann-Whitney test was used to compare the quality-of-life scores between Apert and Crouzon patients. Values were considered significant for a confidence interval of 95 percent (p < 0.05). RESULTS: Apert patients showed an overall higher (score > 60 percent) quality of life in most World Health Organization Quality of Life-100 facets (68 percent) and domains (83.33 percent), with significance (p < 0.05) in three facets (energy and fatigue, mobility, and environment in the home), compared with Crouzon patients. CONCLUSION: Contrary to the authors' initial hypothesis, both the highest-functioning Apert patients and the Crouzon patients presented a satisfactory quality of life, demonstrating that these syndromic patients had acquired the necessary repertoire to manage the adverse daily situations of their lives.

The inverted-T mammaplasty: a modified winch suture to reduce horizontal scar length.

Mammaplasty performed with an inverted-T skin resection pattern is a useful technique to treat moderately or severely ptotic breasts. This method of skin resection is conducted via 3 incisional components: periareolar, vertical, and horizontal. In this report, the authors describe a simple modified winch suture that can be inserted with the inverted-T technique to reduce the length of the horizontal incision.

Two-stage surgical treatment of severe cherubism.

Cherubism is an autosomal-dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration followed by fibrous tissue hyperplasia. Eight patients (age, 6-15 years; mean, 11 years) with severe cherubism were treated with a 2-stage operation by the same senior surgeon (C.M.R.-A.) over an 18-year period, 1987 through 2005. Severity was based on a modified Motamedi classification system. The diagnosis was established clinically, radiographically, and histologically. Postoperative follow up ranged from 2 to 18 years (mean, 5.1 years). All patients underwent 2-stage surgical treatment. In the first stage, the anterior wall of the maxilla was osteotomized and removed (n = 5). The cherubic lesion was curetted from the maxilla and orbits. The maxilla was recontoured on the back table and then fixed orthotopically with wires. Alternatively, cortical bone windows were created, the maxillary and orbital lesions were curetted, and the maxilla was infractured and recontoured in situ with manual pressure (n = 3). Six months after the maxillary/orbital procedure, all patients underwent second-stage curettage and recontouring of the mandible using bony access windows and manual infracture. Satisfactory esthetic results were achieved in all patients. The patients remained clinically and radiographically disease-free throughout the follow-up period. The most profoundly affected patient sustained bilateral lower eyelid ectropion that resolved with full-thickness skin grafting. Although cherubism tends to abate by the fourth decade of life, early 2-stage surgical curettage provides a simple and reliable treatment that not only delivers immediate results, but also seems to arrest the growth of any remaining cherubic tissue.

Bilateral lumbar hip dermal fat rotation flaps: a novel technique for autologous augmentation gluteoplasty.

BACKGROUND: The present study reports a novel technique for augmentation and/or correction of deformities in the gluteal region. This technique is applicable to patients with skin excess, skin flaccidity, and/or ptosis, and for redundant skin folds in the lower back region, that often occur following the surgical treatment of morbid obesity. METHODS: In a retrospective review, nine female patients, aged 27 to 64 years, underwent the bilateral lumbar hip dermal fat rotation flaps procedure for gluteal augmentation. The patients were observed over a period of 3 months to 4 years. Gluteal projection was measured with a standardized computed tomographic scan in two patients. Complications were analyzed. RESULTS: The postoperative photographs and the measurements taken by computed tomographic scan proved that the gluteal projection had been increased satisfactorily with this innovative procedure. Three complications were observed, the major one being a hematoma that required reoperation. CONCLUSION: Bilateral lumbar hip dermal fat rotation flaps is an ultimate technique that should be performed in selected patients to create a harmonic autologous augmentation of the buttocks region. Although the complication rate of this technique was high, it was similar to that in some authors' reports for belt lipectomy.