Scalp erosion in ankyloblepharon-ectodermal defect-cleft lip and/or palate (AEC syndrome): treatment with acellular dermal matrix.

Ankyloblepharon-ectodermal defect-cleft lip and/or palate (AEC syndrome, also known as Hay-Wells syndrome) is an autosomal dominant disease caused by mutation in the p63 gene that is primarily characterized by facial clefting, presence of ankyloblepharon, ectodermal dysplasia, and scalp erosion. Scalp erosion is perhaps the most debilitating manifestation of AEC due to its problematic treatment that is fraught with failure given the underlying pathology of the p63 mutation causing dysfunctional wound healing. Management is often targeted in a stepwise fashion, beginning with daily baths, light debridement, and emollients and progressing to extensive skin excision. Skin grafting has limited success and, inevitably, infections requiring aggressive debridement and antibiotic therapy result from dysfunctional healing. The use of acellular dermal matrix for treatment of scalp erosion is a novel approach attempted in a patient with severe scalp disease. Here we report her case and the failure of treatment, along with possible explanations and suggestions for future therapy.

Multifocal lipoblastoma of the face.

Lipoblastoma is a rare benign neoplasm found exclusively in the pediatric population that can occur anywhere in the body, most commonly seen in the extremities but also found in the face. We report an 8-month-old female subject who presented with multifocal soft tissue masses of the face. The diagnosis of lipoblastoma was made in 2 separate locations after surgical resection. Subsequent surgery was performed at the cheek site in an attempt to remove further mass, resulting in discovery of 2 other discrete tumors found to be lipoblastomas. Although the literature reports recurrence rates ranging from 14% to 27%, to our knowledge, aside from a case of Proteus syndrome, there are no known reports of multiple lipoblastomas in the literature. Presentation of the case, review of pertinent literature, and consideration of congenital infiltrative lipomatosis of the face follow.

Posterior pharyngeal augmentation in the treatment of velopharyngeal insufficiency: a 40-year experience.

BACKGROUND: Velopharyngeal insufficiency (VPI) remains a common problem in the care of patients with cleft palate and other related conditions. It may be successfully corrected with augmentation of the posterior pharynx. The purpose of this study was to review the experience and results of posterior pharyngeal augmentation over a 40-year period at one cleft center in the United States. METHODS: All patients from 1968 to 2008 who underwent posterior pharyngeal augmentation for the treatment of VPI were reviewed retrospectively. Diagnosis, age at the time of operation, type of implant used, duration of follow-up, speech performance both pre- and postoperatively, and complications were analyzed. Speech performance preoperatively was assessed by video fluoroscopy and/or nasoendoscopy. Resonance was assessed by both the surgeon and speech pathologist using a 4 point grading scale. RESULTS: One hundred eleven patients underwent posterior pharyngeal augmentation. Thirteen patients required removal of the implant because of extrusion (n = 12) or postoperative sleep apnea (n = 1). Five patients with failed implants at the primary operation had successful replacement at a second operation, resulting in a retained implant success rate of 93%. Of the 103 patients who were successfully augmented, 15 were lost to follow-up, leaving 88 patients with full postoperative speech evaluations. Of the 88 patients evaluated, 64 exhibited normal or near normal speech (73%), 22 patients had mildly nasal speech (25%), and 2 patients (2%) were not improved. Highest extrusion rates were seen for smooth surface silicone and rolled gortex implants. CONCLUSION: Augmentation of the posterior pharyngeal wall is a safe and effective treatment for patients with VPI. Implants are well tolerated and speech is substantially improved.

Metastasis of hepatocellular and renal cell carcinoma to the hand.

SUMMARY: Malignant carcinoma metastasis to the skeleton is the third most common site of metastasis after lung and liver, usually involving the axial skeleton and the proximal ends of long bones, ribs, and vertebrae. Acrometastasis, metastasis to hands or feet, comprises only 0.1% of all metastases. Here, we present 2 cases of acrometastasis, one from hepatocellular carcinoma and the other from renal cell carcinoma. We describe the presentation, radiographs, surgical treatment, and outcomes for each patient. Patients presented with swelling and pain at the tumor sites and were treated with amputations. The second patient's disease progressed resulting in death shortly after amputation indicating acrometastasis may be a poor prognostic indicator of survival. We review the literature and discuss the importance of disease process recognition and prognosis.

The role of prophylactic antibiotics in elective hand surgery.

The current literature has poorly defined the role of antibiotic agents in elective hand surgery. The reason for this may be due to a small number of well-designed studies specifically addressing the efficacy of antibiotics in hand surgery. Our purpose is to critically review the current literature in an attempt to more precisely define the role of prophylactic antibiotics in elective hand surgery.