Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings.

Benign vascular lesions can be classified into two categories depending on clinical behaviour and endothelial cell characteristics: neoplasms (haemangiomas) and vascular malformations. However, intraosseous vascular anomaly, previously called intraosseous haemangioma, is a very rare malformation. In our previous study, we described the first hereditary form of intraosseous vascular malformation of the craniofacial region, vascular malformation osseous (VMOS). Characteristic findings are autosomal recessive inheritance, severe and diffuse intraosseous vascular malformation in all craniofacial bones without soft tissue involvement and associated mid-line abnormalities such as umbilical hernia and supra-umbilical raphe. In this paper, we discuss the imaging findings of this new disorder in detail.

A new flap model in rats: iliac osteomusculocutaneous flap.

Although osteomusculocutaneous flaps are used frequently in clinical practice to repair defects involving soft tissue and bone, there are still many questions that remain to be answered regarding their basic physiology. To accomplish such basic science studies, simple and reliable animal osteomusculocutaneous flap models are needed. The purpose of this study was to describe a new flap model in rats--namely, the iliac osteomusculocutaneous flap. Thirty adult Wistar rats weighing 200 to 250 g were used in this experiment. In 15 rats, the vascular anatomy of the iliolumbar vessels and their relation with adjacent soft tissues and the iliac bone was determined by anatomic dissection. Based on this anatomic study, the iliac osteomusculocutaneous flap model was created in rats. The flap is comprised of a skin island (3 x 3 cm) in the flank region, a 1 x 1-cm segment of iliac bone, and an abdominal wall muscle cuff. In 10 rats, the flap was raised as an island flap based on its vascular pedicle of iliolumbar vessels, and was replaced in situ. In the remaining 5 rats, the flap was transferred to the groin region as a free flap. Direct observation on postoperative day 7 revealed that the skin island of all the flaps was completely viable. Bone scintigraphy performed on postoperative day 3 in free flaps demonstrated radionuclide uptake, indicating viability of the bony segment. The dye injection study revealed ink staining within blood vessels of the bone, confirming its viability. Microangiography of the flap demonstrated vascularity of each component of the flap by the iliolumbar vessels, including a distinct branch to the iliac bone. The authors conclude that the iliac osteomusculocutaneous flap of the rat is a simple and reliable flap model that offers the following advantages: (1) It is a true osteomusculocutaneous flap, (2) it can be used as a free flap without the need for an isogeneic rat, (3) the vascular pedicle is consistent, and (4) it is harvested from a small-animal species.

Unilateral virginal breast hypertrophy.

A case of unilateral virginal breast hypertrophy with a review of the etiological factors and treatment modalities is presented. A 16-year-old girl presented with progressive enlargement of the left breast of 5 months' duration. The result of the mammographic examination was consistent with cystosarcoma phyllodes. Fine-needle aspiration biopsy revealed giant fibroadenoma. Although some of the characteristics of the fine-needle aspiration biopsy specimen were suspicious for cystosarcoma phyllodes, there were no adequate epithelial structures, which are obligatory for the diagnosis. The patient was treated with subcutaneous mastectomy and subpectoral insertion of a silicone gel implant. The histopathological examination was consistent with virginal hypertrophy. The breast maintained its volume with no further growth in the affected or in the normal breast after 4 years of follow-up.

Lymphatic malformation causing intractable chylorrhagia.

Lymphatic malformation is a developmental error usually noticed at birth or in early childhood. Lesions of the upper leg and lower trunk are the most difficult to remove, because they are often the largest ones encountered and also because they tend to extend proximally into the retroperitoneal tissues. Chyle reflux, usually associated with lymphedema of the extremity, has not been reported to be caused by lymphatic malformation. We report a case of intrapelvic retroperitoneal lymphatic malformation with an extension of gluteal-thigh soft-tissue involvement causing intractable chylorrhagia. The tumor was subtotally excised, and the defect was closed by a distally based, peninsular latissimus dorsi myocutaneous flap. The flap served both as a filling material and as a "bridge" between the residual tumor, including abnormal lymphatics, and normal lymph flow.

Intrasphenoidal migration of a premaxillary Kirschner wire.

OBJECTIVE: The use of Kirschner wire for the fixation of premaxilla is a well-known method in bilateral cleft lip surgery. We report a case in which the Kirschner wire of the premaxillary fixation had migrated intrasphenoidally. RESULTS AND CONCLUSIONS: The foreign body was accidentally discovered during a cephalometric analysis and was taken out surgically through an upper lip sulcus incision. Although the wire remained asymptomatic for 10 years, it constituted a potential danger for intracranial migration.

Comparison of immune response to nerve allograft segments in fetal and adult rabbits: a histological study.

Fetuses, as opposed to adults, are immature immunologically and it has been proved that they can tolerate allograft materials much better than adults. In this study the rejection phenomenon of nerve allografts was compared histologically in fetuses and adults. The study was performed in 60 New Zealand rabbits (30 pregnant and 30 nonpregnant), and allograft nerve segments were obtained from Chinchilla rabbits. The animals were divided into fetal and adult groups. Each group was studied at various time periods. Nerve allografts were placed under the panniculus carnosus in the interscapular region of the fetuses and adults. In both fetal and adult groups, the nerve allograft segments were assessed histologically after 1, 7, and 30 days. The criteria used during the evaluation were the degenerative findings in the myelinated axons (large, medium, and small axons), changes in Schwann's cells, and the quantity of infiltrating cells. The changes were graded microscopically from 0 (no change) to 3 (severe destruction and cellular infiltration). Cellular infiltration was more extensive in the adult groups than in the fetal groups. Earlier fetal groups showed minimal infiltration, but the response became more extensive in the later fetal groups. This is probably related to the removal of the fetuses from their intrauterine environment. When comparing fetal and adult groups, the results were significant (p < 0.05). The fetuses tolerated the nerve allograft segments better than the adults. This may be related to the immature immune system of the fetuses.

Bartsocas-Papas syndrome with fusion of the lips and posterior fusion defects of the thoracic vertebrae.

Bartsocas-Papas syndrome is a rare popliteal pterygial syndrome with multiple anomalies including microcephaly, facial clefts, filiform bands, ankyloblepharon, syndactyly, and other ectodermal anomalies. Affected infants usually die perinatally. The authors present an 8-month-old female infant with manifestations of this syndrome and some additional traits including fusion of the lips, intraoral filiform bands, alopecia totalis, and posterior fusion failure of the vertebrae. The fused lips were opened by incising the fibrotic bands closing her mouth. Details of this patient and a brief review of the literature is presented.

Malignant melanoma in xeroderma pigmentosum patients: report of five cases.

Xeroderma pigmentosum is a rare genetic disease transmitted via a recessive gene with an altered reaction of the epidermis to light. Fifty per cent of patients develop a skin tumour by 8 years of age. The majority of patients may have multiple tumours, but metastasis is rare. In the last 25 years we have treated 24 xeroderma pigmentosum patients in our clinic. Only five patients had developed cutaneous malignant melanoma during their follow-up. Three of the patients were from the same family, melanoma occurring in three of five affected individuals. All xeroderma pigmentosum patients with malignant melanoma had received classical treatment modalities. Except one case of fulminant pattern, all four patients had long disease-free survival. Although early detection and treatment of these cutaneous malignancies will reduce morbidity and mortality, genetic counselling remains the most important protective measure for xeroderma pigmentosum.

Semicircular lipoatrophy after intragluteal injection of benzathine penicillin.

A 3-year old girl had localized lipoatrophy after an intragluteal injection of benzathine penicillin G. No associated abnormalities were found.

Repeated mandibular lengthening in Treacher Collins syndrome: a case report.

A patient with mandibular hypoplasia associated with Treacher Collins syndrome was treated by bilateral distraction osteogenesis. Since less than optimal length was provided by the first distraction, a second corticotomy was performed in the newly formed bone 6 months after the first distraction. Thus bone gained by distraction osteogenesis was subjected to distraction once again. New bone formation occurred after the second lengthening. This case illustrated that distraction osteogenesis may be applied to the mandible at the site of previous distraction.

A case report of penis reconstruction for partial penis necrosis following circumcision.

Circumcision is one of the most common operations performed world-wide and is usually done for religious and social reasons. Although it is a simple procedure, serious complications may result. These complications may range from hemorrhage to total amputation. Reconstruction of a partial amputation of phallus due to circumcision by full-thickness skin grafting is presented in this paper.

Modified split-level lid resection for blepharoptosis.

Mustardé's split-level lid resection surgery was popular through the 1980s for the correction of blepharoptosis with 7 mm or more of levator function. Although the aesthetic results gained with this technique were good, last line deformity and eyelid margin irregularities, such as central peaking at forward and upward gaze, were experienced. A simple modification of the tarsal resection pattern of Mustardés operation has solved these complications. In the treatment of 24 ptotic eyelids (12 unilateral, 6 bilateral with a modified split-level lid resection procedure), symmetrical appearance and level eyelids were obtained without lid margin peaking at 5 years' follow-up. The only persistent complication was lid lag at down-gaze in 10 eyelids.

Unilateral absence of the soft palate: case report of reconstruction with a mucoperiosteal island flap.

Congenital absence of half of the soft palate is a rare deformity. There is little in the literature about its definition and management. This article presents a case with velopharyngeal insufficiency caused by unilateral absence of the soft palate. The patient was treated with a modification of the mucoperiosteal island flap, first designed by Millard, to provide nasal lining during pushback lengthening of a short cleft palate. The speech quality of the patient improved noticeably after the operation. Although island flap has limited use in primary cleft palate surgery, it may be effective in reconstruction of soft palate defects, when standard pushback procedures are not adequate for solving the problem.

Use of a subfascial pocket on the contralateral calf for salvage of an avulsed foot.

Cross-leg fasciocutaneous flaps have long been used for reconstruction of defects on the contralateral foot. This report describes the use of a cross-leg subfascial pocket for preservation of avulsed metacarpal and phalangeal bones and reconstruction of both plantar and dorsal aspects of the contralateral foot. The avulsed metacarpal bones and phalanges of the foot were embedded into a subfascial pocket prepared on the contralateral calf in the first operation. The gastrocnemius fasciocutaneous flap was then used for reconstruction of the contralateral foot. The avulsed structures were kept vital during the 20 days that elapsed between these two procedures.

Reconstruction of postburn breast deformities.

Postburn breast deformity is a sequela of severe scar contraction of the burned chest. During the past 3 years, 24 female patients with such deformities required reconstruction, the surgery was performed in our department. These patients, the types of the deformities and the techniques used for reconstruction have been reviewed. For mild deformities (10 patients) reconstructions with skin grafts and local skin flaps were found to be satisfactory. For deformities which affected the mammary development (14 patients), mammary prostheses directly or under the soft tissue obtained by skin expansion or musculocutaneous flaps were used. In three of our patients, reduction mammaplasty or mastopexy was needed to symmetrize the breasts.