Orthodontic and surgical treatment of a patient with Apert syndrome.

The aim of this case report was to present the combined orthodontic and surgical treatment of a patient with Apert syndrome in an adult stage. A 15 years old male patient with Apert syndrome was concerned about the appearance of his face and malocclusion. His profile was concave with a retruded maxilla and prominent lower lip. He had an Angle class I molar relationship with a 9.5 mm anterior open bite. The amount of crowding was 20.4 mm in the maxilla and 6 mm in the mandible. Cephalometric analysis revealed a skeletal Class III relationship due to maxillary hypoplasia with a dolichofacial growth pattern. Orthodontic treatment and orthognathic surgery were planned for the patient. After 45 months of presurgical orthodontics, the patient underwent two surgeries sequentially. The first surgery was performed to advance the maxilla and the second surgery was performed to correct the mandibular rotation and increase the overbite at the time of removing halo device. The amount of maxillary advencement was 8 mm. Mandibula was moved 1.5 mm anteriorly and rotated 1° to 1.5° (SNB and facial depth) in a counterclockwise direction. After a relatively long treatment, an esthetically pleasing and functional occlusion and correction of the skeletal problem was achieved in this adult case.

A new concept in the treatment of extravasation injury: controlled drug delivery systems.

PURPOSE: To investigate the effectiveness of the intralesionally injected controlled granulocyte-monocyte colony stimulating factor (GM-CSF) releasing system in widening refractory extravasation wounds. METHODS: The determination of in vitro GM-CSF release from chitosan gel was the first, and in vivo effect of the molecule was the second step of the study. Thirty-five Wistar-Albino rats were randomly divided into 5 groups: 1) control group (adriamycin group) (n=7); 2) adriamycin+normal saline group (n=7); 3) adriamycin+chitosan group (n=7); 4) adriamycin+1 µg/mL GM-CSF-loaded chitosan group (n=7); and 5) adriamycin+10 µg/mL GM-CSF loaded chitosan group (n=7). The wound area was measured macroscopically and histological examination was carried out for wound healing and tissue response to the polymer. RESULTS: The best healing process was observed with the controlled released GM-CSF groups (groups 4 and 5). The 1 µg/mL GM-CSF loaded group showed superior wound healing than that of 10 µg/mL GM-CSF loaded gels. This result was correlated with the in vitro study which also showed increased drug release in the 1 µg/mL GM-CSF loaded group than the 10 µg/mL GMC-SF loaded gels. CONCLUSION: This study suggests that GM-CSF, applied with controlled drug delivery system, can supply dynamic treatment options with long-lasting activity in single-dose administration.

Congenital isolated absence of the nasal columella: reconstruction with an internal nasal vestibular skin flap and bilateral labial mucosa flaps.

Congenital aplasia of the nasal columella is a very rare anomaly. The deformity is characterized by the isolated absence of the columella from the nasal tip to the root of the philtrum, including the medial crura of the alar cartilages; surrounding structures such as the septum, nose, and upper lip are normal. To the best of our knowledge, only four such cases have been described to date. The embryopathogenesis for this uncommon disease is presently unknown. Our report describes a 14-year-old girl with congenital agenesis of the columella as an isolated anomaly. Her family history was positive for the presence of the same congenital deformity, which also affected her older brother; there was, however, no consanguinity between the parents. The columella defect was reconstructed with an internal nasal vestibular skin flap and bilateral upper labial mucosa flaps. There are many techniques available to repair columella defects, including free grafts from the ear, local flaps from the forehead, face, upper lip, and nose, distant flaps such as tube pedicle flaps, and free flaps from the ear. Each of these techniques has advantages and disadvantages. Because of this, the treatment of columella defects should be individualized.

Upper lip flap for reconstruction of a full-thickness ala nasi defect.

Aesthetic reconstruction of full-thickness ala nasi defects has always been a challenge to the surgeon. Although several techniques are available, none has proved to be ideal. In this paper we describe a flap from the upper lip for reconstruction of defects involving the alar base and full-thickness defects of ala nasi. A flap based on the columella-labial junction is rotated upward for reconstruction of the alar base or ala nasi. The donor site of the flap can be closed primarily. When ala reconstruction is indicated, an upper lip flap forms the inner lining of the nostril, and a skin graft is applied over it for skin coverage. With this flap, an appropriately positioned ala with natural contours may be reconstructed in one stage.

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.

Modified Charles operation for primary fibrosclerotic lymphedema.

Radical excision of lymphedematous tissue with skin grafting (Charles operation) may be required for patients with advanced fibrosclerotic lower extremity lymphedema. Complications of this procedure include papillomatosis, wart formation, intractable skin ulcerations and weeping of lymph and are often considered major drawbacks of the operation. We have largely circumvented these sequelae by burying a strip of shaved split-thickness skin graft into the deep subcutaneous tissue thereby modifying the Charles operation. The strip of deepithelialized skin seemingly connects the superficial dermal lymphatics with subfascial deep lymphatics thereby facilitating lymph drainage and minimizing lymphedema accumulation and the complications outlined above. We have now treated 4 patients with advanced primary fibrosclerotic lymphedema using this modified technique. Not only were the patients improved in appearance and function with less trophic changes, but lymphscintigraphy using 99mTc-dextran also suggested improved interstitial tracer transport.

Further experience with upper lip flaps for reconstruction of the ala nasi and alar base defects.

Reconstruction of the ala nasi and the alar base presents problems because of complex contours, skin color and texture, and limited availability of mobile, adjacent skin. A large percentage of basal cell carcinomas occur on the nose. When excised with adequate safety margins, these lesions create a defect too large for direct repair. This paper describes the use of the upper lip flap for reconstructing alar base defects and reconstructing the inner lining of the ala nasi in full-thickness defects of the ala nasi as an alternative to other procedures proved to be effective on this region. The base of the flap rests on the base of the columella. It is raised just over the underlying orbicularis oris muscle. The resulting donor defect is closed primarily. The outer aspect of defects of the ala nasi are covered with either a full-thickness skin graft, a forehead flap, or a cheek flap. The upper lip flap was used on 10 patients to reconstruct full-thickness defects of the ala nasi, for which an alar base needed to be reconstructed. The results were satisfactory in most patients. An upper lip flap can be used safely to reconstruct the ala nasi and alar base defects either alone or in combination with other flaps.

Titanium screw implants for intermaxillary fixation of partially edentulous jaw.

Establishment of the best possible relationship between upper and lower teeth is very important when treating jaw fractures and orthognathic deformities in partially edentulous patients. Many surgeons use arch bars and acrylic splints for intermaxillary fixation (IMF) to obtain the best occlusal relationships after the operation. In patients with sufficient teeth, IMF is not so difficult to realize. However, in partially edentulous patients, the available teeth may not be sufficient to apply arch bars or splints. This paper describes a system for IMF of a partially edentulous jaw. Screws made of medical-grade titanium are implanted into the alveolar ridges where two or more teeth are missing. Arch bars or acrylic splints secured on these implants and available teeth can be used safely for IMF. In vitro axial pull-out tests demonstrated that these implants can withstand the traction forces generated by elastics. Five partially edentulous patients, three with mandibular fractures and two with orthognathic problems, were treated with these implants. All patients healed without any complications and with the best possible occlusal relationships.

Open rhinoplasty through a forked flap incision.

Surgical elongation of the short columella is a challenging problem for the surgeon. Although some flaps from the upper lip are successfully used to correct this deformity on cleft lip patients with a scarred upper lip, these methods cannot be applied to noncleft patients with a smooth upper lip. Distant flaps and composite grafts do not give the best aesthetic results. The use of an external approach for rhinoplasty is preferred by many surgeons, especially for difficult or secondary cases. Most incisions for open rhinoplasty are placed on the columella. This report describes a new incision for open rhinoplasty to be used on patients with a short columella. The incision is a standard forked flap with a columellar base but the legs of the flap extend to the nostril bases instead of to the upper lip. This method was used on eight aesthetic rhinoplasty patients with a short columella between March 1995 and March 1998. The results of the method are discussed.

Use of umbrella graft for nasal tip projection.

An adequate nasal tip projection is of the utmost importance for good nasal aesthetics. Conventional rhinoplasty procedures are not adequate for achieving nasal tip projection in "tips with inadequate projection" (TIP). This article describes our technique of using an umbrella-shaped cartilage graft to the tip. The graft is carved from caudal septal and alar cartilages. The results and advantages of the technique are discussed.

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.

Dermatofibrosarcoma with lymph node involvement.

Dermatofibrosarcoma protuberans is a slow-growing, locally aggressive, fibrous tumor that, on rare occasions, metastasizes to distant sites or regional lymph nodes. We have found 28 patients with metastasis in the literature; 9 of these patients had lymph node metastasis. In this report we present a case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to inguinal lymph nodes appearing 10 months after wide excision and skin grafting of the primary lesion. The clinical and pathological features of this case are presented. Although, along with our patient, 10 cases of regional lymph node metastasis are not justification for regional lymph node dissections, they do emphasize the need for regional lymph node examinations in the long-term follow-up of dermatofibrosarcoma protuberans cases.

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.