Plastic and reconstructive surgery in epidermolysis bullosa: clinical experience with 110 procedures in 25 patients.

We present clinical experience with 110 reconstructive procedures in 25 patients with epidermolysis bullosa, a group of rare heritable disorders characterized by marked fragility of the skin and mucosa. We discuss management of hand and foot deformities unique to epidermolysis bullosa patients, excision of squamous cell carcinoma, and reconstruction of oral, nasal, and ocular tissues. All patients underwent these procedures without major surgical or anesthetic complications. We analyze surgical outcome and formulate guidelines to avoid damaging the skin and mucosa.

Squamous cell carcinoma of the breast after augmentation with liquid silicone injection.

Squamous cell carcinoma of the breast is an extremely rare neoplasm. Approximately 50 cases have been reported in the English literature since 1917. The pathogenesis of squamous cell carcinoma of the breast is puzzling because epithelial elements are not normally identified in breast tissue. It has been suggested that epithelial cells are derived from epidermoid cysts deposited during early embryological development, from metaplastic transformation of ductal cells, or after trauma or surgical manipulation. Although no evidence has been published to support a casual relationship between liquid silicone-induced mastopathy and breast carcinoma, squamous cell cancers are known to arise in the setting of prolonged inflammation often seen after liquid silicone injection. This case of primary squamous cell carcinoma of the breast, arising 25 years after augmentation with liquid silicone injections, occurred in a 70-year-old patient with silicone-induced mastopathy.

Atypical melanocytic lesions in epidermolysis bullosa.

We report a 6-year-old female with recessive dystrophic epidermolysis bullosa (RDEB) who presented with a very large acquired melanocytic lesion. The lesion demonstrated many features both clinically and histologically that made the distinction from malignant melanoma difficult. The pathogenesis of this lesion and other unusual melanocytic lesions seen in the setting of acute and chronic blistering disorders seems related to repeated episodes of disruption of the dermal-epidermal junction.

Anesthetic management in epidermolysis bullosa: review of 129 anesthetic episodes in 32 patients.

BACKGROUND: Anesthetic and monitoring instrumentations such as endotracheal intubation may cause skin and mucosal damage with potentially serious consequences in patients with epidermolysis bullosa (EB). OBJECTIVE: This study defines the risks of skin and mucosal damage from anesthetic and monitoring techniques in patients with EB and formulates management guidelines. METHODS: We retrospectively analyzed the outcome of 129 anesthetic episodes in 32 patients with various types of EB. RESULTS: Serious complications did not occur in any patient with EB from the use of endotracheal intubation, face mask, nerve blocks, local anesthetics, and intravenous or intramuscular anesthetic agents. CONCLUSION: With appropriate precautions, patients with EB can undergo standard anesthetic techniques with only minor and infrequent complications.

Gastrocnemius muscle flap coverage of exposed or infected knee prostheses.

A retrospective analysis was performed of 12 patients who required soft-tissue coverage of an exposed or infected total knee prosthesis between 1982 and 1989. All knees had skin closure with medial gastrocnemius muscle flaps. At a mean follow-up period of 41 months, all patients who were treated for infected prostheses with removal of the implant, intravenous antibiotics, and muscle flap closure had an excellent clinical result with complete skin coverage and no infection. Five of the six patients went on to successful reimplantation. Of the patients with an exposed prosthesis, five of six had an excellent outcome with retention of the prosthesis. Thus, 11 of 12 patients (92%) who had medial gastrocnemius flap coverage of an exposed or infected knee prosthesis had an excellent outcome, with ten of 12 patients (82%) retaining their prostheses or having a successful reimplantation. No medial gastrocnemius flap failed after standard primary or revision total knee arthroplasty. Gastrocnemius muscle flaps provide excellent soft-tissue coverage of exposed or infected knee prostheses and facilitate surgical care of this difficult problem.

Hyperosmotic mannitol and collateral blood flow to ischemic myocardium.

Elevation of extracellular osmolality reduces the extent of myocardial and endothelial cell swelling that accompanies acute ischemia, and the reduction of cell swelling is associated with an increase in collateral blood flow to the ischemic area. However, little is known about the effects of hyperosmolality on the vascular resistance of the collateral coronary vasculature. We compared the effects of hyperosmolar mannitol with those of nitroglycerin and dipyridamole on the vascular resistance of large collateral coronary vessels and of the small arterial vasculature in an isolated heart model of regional ischemia. Elevation of osmolality by mannitol increased collateral blood flow to the ischemic region through at least two mechanisms. First, increasing osmolality resulted in dilation of large arterial conductance vessels, similar to that produced by nitroglycerin. In addition, mannitol produced an effect on the coronary circulation at a microvascular level which, per se or in combination with its effect on larger collateral conductance vessels, increased collateral blood flow to ischemic regions.

A comparison of the open and closed methods in the initial treatment of sternal wound infections.

To determine the optimal initial treatment method for sternal wound infections, a retrospective review of 3,229 consecutive adult open heart operations was performed. There were 40 deep sternal wound infections (1.2%). All types of open heart procedures had approximately the same rate of infection except for left ventricular aneurysm repair plus coronary artery bypass grafting, which had a sternal wound infection rate of 5.8% (p less than 0.01). The use of single and double internal mammary artery grafts did not increase the rate of sternal wound infections. Treatment outcome was available for 39 patients. Twenty-eight patients were initially treated with debridement and closed mediastinal irrigation, which had a failure rate of 42% and a mortality rate of 35%. Six patients were initially treated with debridement, open packing, and muscle flap closure of the sternal wound. No deaths or treatment failures occurred in this group. The mortality and failure rates for the open treatment method were significantly better than for the closed treatment method in the initial treatment of sternal wound infections (p less than 0.05). These data, as well as a review of the literature, suggest that the open method is superior to the closed method in the initial treatment of deep sternal wound infections.

Computed tomography--its principles and application to the diagnosis of facial fractures.

The introduction of microcomputers with their chips of integrated circuits and ability to rapidly perform thousands of complex mathematical instructions has enabled the clinician to readily use and manipulate tomographically acquired data from relatively narrow body slices. These data allow identification of complex injuries and disease states through the construction of detailed anatomic images and the differentiation of structures of low densities and those of subtle density differences.

Computer use in the medical specialties.

This article summarizes new and innovative uses of the computer in various medical applications. It highlights the use of this technology in the fields of Cardiology, Radiology, Rehabilitative Medicine, Laboratory Medicine, and so on. It also discusses future uses in aiding the handicapped patient to ambulate, the deaf to hear, and the blind to see.

Free flap neo-vascularisation: case report.

This report records the unusual occurrence of occlusion of the artery of a free flap on the ninth post-operative day. Careful clinical observation strongly suggested that good neo-vascularisation had already occurred at this early date. The pattern of capillary refill suggested that neo-vascularisation originated from the periphery of the recipient site. The decision was made not to re-explore the flap vessel. The flap survived.

Cleft lip: morbidity and mortality in early repair.

A fifteen-year survey of primary lip repairs done at The New York Hospital revealed that lip closure can be accomplished during the first week of life with no mortality and with major morbidity slightly higher than that in repairs done after one week of age. Modern anesthetic techniques have markedly reduced respiratory complications from lip closure. Adequate postoperative sedation coupled with careful patient monitoring should appreciably reduce the incidence of wound dehiscence.