Verrucous malformations: their presentation and management.

Verrucous hemangiomas are a distinct subset of vascular malformations that have not been described extensively in the plastic surgery literature. They are characterized by reactive epidermal acanthosis, papillomatosis, hyperkeratosis, and extension into the subcutaneous tissues. In response to injury, infection, or subtotal resection, they enlarge and become increasingly keratotic. In light of contemporary definitions of hemangiomas and malformations, the authors recommended that these lesions be renamed verrucous malformations. The authors review their evaluation and treatment of 6 patients with this lesion and offer an algorithm that emphasizes excision over ablative therapy. Of the 6 patients, 3 patients had failed either cryotherapy or laser removal by a nonsurgeon, with a consequent increase in lesion size and discomfort. The lesions were all subsequently excised. Because of the size and location of the verrucous malformations, staged removal was required in 3 patients. Patients have been followed for as long as 7 years. A single recurrence was controlled with reexcision. Excision of verrucous malformations, rather than laser ablation or cryosurgery, is supported by the authors' favorable results.

Nonlethal case of otocephaly and its implications for treatment.

Otocephaly is a craniofacial syndrome with a constellation of findings consistent with symmetrically deficient development of the first branchial arch. Characteristically, those affected have ventrally displaced ears, microstomia, and an atrophic to absent mandible. Secondary to oropharyngeal incompetence, ventilatory, difficulties typically lead to the imminent death of these infants shortly after birth. Here we present a patient who survived well beyond the perinatal period with the support of pediatric intensivists and advanced technology available for the diagnosis and treatment of such a complex crajniofacial syndrome.

Giant congenital nevocellular nevus overlying an encephalocele.

Discussion regarding the diagnosis, prognosis, and management of giant congenital nevocellular nevi (GCNN) has permeated the literature because of concerns regarding malignant potential. As a derivative of dysgenetic neural crest development, GCNN histology in the neonate may be quite variable and not easily discernible from malignant melanoma, presenting a diagnostic dilemma to both the seasoned pathologist and surgeon. Here we present an interesting case of GCNN arising from a large encephalocele in a neonate. In spite of a clinically aggressive course, histology repeatedly failed to meet the criteria for melanoma until the diagnosis of malignant melanoma was made at autopsy. Clinical suspicion for malignancy must be high when evaluating atypical large nevi, and prompt surgical management is necessary to protect against the risk for malignant melanoma.

Proteus syndrome: a newly recognized hamartomatous syndrome with significant craniofacial dysmorphology.

A new disorder describing multiple hamartomas distinct from neurofibromatosis and Klippel-Trenaunay-Weber syndrome was first reported in 1979. It was named Proteus syndrome after the Greek god Proteus, the polymorphous, who could change his shape at will to avoid capture. The clinical manifestations are extensive, including cranial exostoses; progressive enlargement, asymmetry and disfigurement of the skull; macrocephaly; exostoses of the ear canals, nasal bridge, and alveolar ridge; partial gigantism of the hands or feet, asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, and long bone overgrowth. A case report of Proteus syndrome is presented and discussed along with a review of the pertinent literature.

Surgical correction of pressure ulcers in an urban center: is it efficacious?

Recurrence following surgical correction of pressure ulcers remains one of the more formidable complications of their treatment. To assess the effect of surgical therapy on recurrence, thirty patients were evaluated between 1985 and 1990 at The Johns Hopkins Hospital. These data were compared to a prior study conducted from our institution between 1977 and 1980. Follow-up in this current study ranged from 1-108 months. Of the 22 paraplegic patients, pressure ulcers recurred at the same site of surgical correction in 82 percent (n = 18), or at a different site in 64 percent (n = 14), at an average of 18.2 and 20.2 months respectively. No recurrence was noted in the eight non-paraplegic patients. In an urban center, surgically corrected pressure ulcers have a high recurrence. Physician and patient must be willing to accept a high recurrence rate prior to attempted surgical closure.

Anatomic and clinical considerations of an internal mammary artery harvest.

In an effort to understand the perceived correlation of internal mammary artery harvesting and wound healing difficulties in the inferior margins of the sternotomy incision, we showed the cutaneous vascular perfusion in the sternal and xiphoid areas by India ink injection studies in cadavers. With these studies, we demonstrated an inherent paucity of nutrient supply to the inferior sternum and xiphoid area. The classic internal mammary artery harvest further compromises the blood supply to these areas. We believe that limiting the most inferior dissection of the internal mammary artery and not including the distal bifurcation leaves intact the lateral musculophrenic nutrient supply to the inferior sternum and xiphoid area and to the ipsilateral abdominal rectus muscle. These guidelines will help to prevent ischemic complications of this area and may aid in reconstruction. If the bifurcation is harvested, we believe that the removal of the avascular xiphoid cartilage at the time of the initial bypass procedure may eliminate this as a potential septic focus.

The use of immediate grafting in facial fracture management. Indications and clinical considerations.

One of the goals of reconstruction of the craniomaxillofacial skeleton following major trauma has been the one-stage repair of form and function. This goal can be achieved by the application of craniofacial techniques, careful interfragmentary stabilization, and primary bone grafting even in the most severe of injuries. Primary bone grafting, only recently achieving wide acceptance, has proven to be an important clinical advance allowing definitive one-stage repair in previously extremely difficult situations. The results are reproducible and predictable. In many centers, consistently superior end results are achieved for patients after the primary reconstruction. Future trends in facial fracture and trauma management now incorporate these concepts into the armamentarium of the reconstructive surgeon.

The effect of rigid fixation on growth of the neurocranium.

The effects on skull growth of plating the coronal suture and frontal bone were studied in New Zealand White rabbits. Three-dimensional coordinate landmarks were digitized and analyzed to determine the differences in form between operated and unoperated animals using Euclidian distance matrix analysis. This method compares sets of interlandmark distances in three dimensions and was used to demonstrate changes induced by plating. We interpret these changes in morphology to be the result of differences in growth between the operated and unoperated groups. Periosteal elevation alone (n = 6) resulted in a minimal local growth increase. Coronal suture plating (n = 8) resulted in local growth restriction with contralateral and adjacent size increases. Frontal bone plating (n = 6) without crossing a suture line also resulted in local growth restriction and adjacent bone size increases. The timing of intervention in relation to the completion of bone growth may explain the magnitude of clinically apparent effects. Changes in bones adjacent to those directly manipulated may be an attempt to maintain a normal skull volume.

Can infected prosthetic grafts be salvaged with rotational muscle flaps?

The conventional approach to prosthetic graft infection, including graft removal and extraanatomic reconstruction, conveys a substantial risk of limb loss and death and mandates more innovative solutions. From January 1985 to January 1989 eight rotational muscle flaps were performed on four men and three women with prosthetic graft infection who ranged in age from 42 to 79 years (mean, 67 years). The grafts involved included aortofemoral (three patients), femoropopliteal (two patients), femorofemoral (one patient), and subclavian-carotid-carotid (one patient) and were composed of Dacron (five grafts) or polytetrafluorethylene (two grafts). Infections were in the groin in six patients and in the neck in the other patient; all patients had anastomotic exposure. Clinical presentations included abscess/purulent drainage (four patients), anastomotic hemorrhage (two patients), and anastomotic false aneurysm (one patient) and was associated with fever and/or leukocytosis in all patients. Positive bacterial cultures were obtained from all patients. Rotational muscle flaps performed included rectus abdominis (five grafts), pectoralis major (one graft), gracilis (one graft), tensor fascia lata (one graft); in two patients, rotational muscle flaps were performed after failed local sartorius muscle transfer. No major complications of the RMF procedures were encountered. One patient died 4 months after the operation of complications of ischemic colitis/perforation. The other six patients were discharged with completely healed wounds. One patient developed recurrent infection 12 months after the rotational muscle flap procedure; five (83%) patients have been followed for 12 to 51 months (mean, 27 months) without evidence of recurrent infection. These preliminary results suggest that rotational muscle flaps are a safe and effective treatment for prosthetic graft infection, even when local sartorius muscle coverage has failed.

Separation of craniopagus Siamese twins using cardiopulmonary bypass and hypothermic circulatory arrest.

Occipitally joined craniopagus Siamese twins were separated with the use of cardiopulmonary bypass and hypothermic circulatory arrest. The 7-month-old infants shared a large sagittal venous sinus that precluded conventional neurosurgical approach because of risk of exsanguination and air embolism. After craniotomy and preliminary exposure of the sinus, each twin underwent sternotomy and total cardiopulmonary bypass with deep hypothermia. Hypothermic circulatory arrest allowed safe division and subsequent reconstruction of the sinus remnants. Several unusual problems were encountered, including transfusion of a large blood volume from one extracorporeal circuit to the other through the common venous sinus, deleterious warming of the exposed brain during circulatory arrest, and thrombosis of both pump oxygenators. Both infants survived, although recovery was complicated in each by neurologic injury, cranial wound infection, and hydrocephalus. This case demonstrates the valuable supportive role of cardiopulmonary bypass and hypothermic circulatory arrest in the management of complex surgical problems of otherwise inoperable patients.

Rigid fixation: variations in osteotomy design and technique.

The application of rigid fixation devices has expanded the craniomaxillofacial surgeon's armamentarium and allows greater versatility of osteotomy design and fixation. Further studies are under investigation to determine the degree of "interference" with growth as a result of application of rigid fixation devices. Our preliminary thought is that bone is dynamic and "liquid" enough to allow the driving forces of the developing cerebral tissue to remodel and recontour the bone tissue into a normal configuration and contour. Problems with relapse and resorption appear greatly reduced for both bone grafts and bone flaps. As the biomaterials industry continues to perfect rigid fixation devices, further advances will be forthcoming.

Three-dimensional computed tomographic reconstructions of intracranial meningiomas.

In a series of four patients with intracranial meningiomas, three-dimensional computed tomographic (CT) reconstructions were a useful diagnostic and surgical adjunct. Three-dimensional images are created from standard CT data by a boundary-detecting computer software program. Three-dimensional images of tumor invading or adjacent to the bony calvarium are projected about the x, y, and z axes. Axial and sagittal sections delineate the lesions. The images created allow a surgical view of the meningiomas in three-dimensional space and demonstrate the relationship of these masses to the skull. The use of three-dimensional reconstructions in craniofacial surgery and in neurosurgery is reviewed.

Early and late complications of orbital fractures.

Complications, unfortunately, arise as a result of traumatic or iatrogenic injuries. The sequelae can range from the most insignificant to the most debilitating. The most common complications noted in the diagnosis and treatment of orbital fractures are outlined as well as discussed as appropriate to their appearance during the healing process. With better understanding and better management, complications can be prevented.

Radiographical documentation of direct injury of the intracanalicular segment of the optic nerve in the orbital apex syndrome.

In the radiographical evaluation of the orbital apex syndrome, standard radiographs, tomograms, and computed tomographic scans have proved useful in the demonstration of the bony pathology, especially for optic canal fractures. The limitation of these methods, however, remains in their inability to provide accurate delineation of the associated soft tissue pathology, including the presence of optic nerve sheath hematoma. Recent developments in computer technology and graphic imaging are now available to provide an accurate three-dimensional radiographical analysis of the extent of skeletal and soft tissue injury in the orbital apex syndrome. The physician, in essence, can perform a radiographical "living autopsy". The technique was used to evaluate a patient with bilateral apex syndrome. It clearly showed that a severe direct injury to the intracanalicular portion of the optic nerve was responsible for the development of blindness in this patient. The progression of optic nerve injury, from perineural sheath hematoma to the ultimate development of optic nerve atrophy and fibrosis, was radiographically documented.

Pseudomalignancies.

The plastic surgeon encounters a variety of lesions that are benign histologically, but malignant in terms of their manifestations and clinical course. Many of these desperate pathologic situations present exceedingly difficult problems in terms of their diagnosis and management. Clinical entities that tend to be grouped within this type of pathologic phenomenon include giant pigmented nevi, arteriovenolymphatic malformations, neurofibromatosis, and localized overgrowth. The authors examine such lesions on the basis of their presentations, pathogeneses, and pathophysiology.

Volumetric quantification of intracranial and ventricular volume following cranial vault remodeling: a preliminary report.

This preliminary study documents preoperative and postoperative changes in cerebral tissue as well as intracranial and ventricular volume in patients who underwent cranial vault remodeling for craniosynostosis. The documentation and calculations were provided from CT data according to a craniofacial protocol. Three-dimensional images were then obtained of the preoperative and postoperative skulls and cerebral tissues. From these data, comparisons of preoperative and postoperative volumes of the cerebral tissue and ventricles could be examined. In one case, a frontal bone advancement combined with anterior cranial vault remodeling was associated with an increase in intracranial volume of 110 cc (8 percent) and a ventricular volume increase of 112 percent. The reported technique should allow more complete evaluation of the preoperative pathology and documentation and prediction of the projected intracranial and ventricular volume changes.

Rapid management of persistent seromas by sclerotherapy.

Postoperative lymphoceles and adventitious bursae are common occurrences. They are slow to resolve and often persist when traditional techniques of prolonged drainage, multiple aspirations, and local pressure are used. Tetracycline sclerotherapy provides a rational nonoperative alternative treatment that is simple and without major complications and provides a rapid resolution of persistent seromas. Based on our favorable limited experience we believe that this technique should be studied in patients with persistent seromas in a randomized prospective fashion.

Achalasia of the cardia associated with pulmonary sarcoidosis.

The case of a patient with sarcoidosis and symptoms suggestive of achalasia cardia is presented. Because of increasingly severe dysphagia, cardiomyotomy was performed and biopsy specimens of the esophageal wall were obtained. Light and electron microscopy revealed lesions of the nerves in Auerbach's plexus consisting of an inflammatory process and demyelinization of the nerve fibers. Since the operation, the patient has been swallowing without difficulty. Although previous reports have described dysphagia in patients with sarcoidosis, presumably secondary to mechanical compression by adjacent lymph nodes or infiltration of the esophageal wall by sarcoid granulomata, this report documents for the first time that dysphagia can also be caused by direct involvement of the innervation of the esophagus by sarcoidosis.