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Prenatal exposure to nitrofen is known to cause multiple malformations in mice. The reported malformations include lung hypoplasia, diaphragmatic hernia, cardiovascular defects, skeletal malformations, cleft palate, and renal abnormalities. The authors present detailed findings of craniofacial defects after prenatal exposure to nitrofen, and propose that together with the previously reported malformations, nitrofen exposure induces a Fryns phenotype in mice. Fryns syndrome is a rare human genetic syndrome that is an autosomal recessive disorder characterized by lung hypoplasia, diaphragmatic hernia, craniofacial malformations, skeletal malformations, cardiovascular malformations, and genitourinary malformations. Timed-pregnant Swiss Webster mice were gavage-fed 25 mg of nitrofen on day 8 of gestation. Control animals received olive oil. Osteogenesis and chondrogenesis were studied in fetuses recovered on day 17 after Alcian blue-Alizarin red staining. Approximately 26% of the nitrofen-exposed embryos had severe craniofacial defects, and there was generalized delay in chondrogenesis and osteogenesis throughout the skeleton. No such defects were noted in the control group. The authors propose that prenatal exposure to nitrofen induces a Fryns phenotype in mice, and thus speculate that nitrofen may target similar molecular mechanisms to those that lead to Fryns syndrome.

Frontoethmoidal encephaloceles are herniations of the intracranial contents through a defect in the skull at the junction of the frontal and ethmoidal bones. They are generally classified as nasofrontal, nasoethmoidal, and naso-orbital, although there may be some overlap or multiplicity. The records of 35 patients treated for frontoethmoidal encephaloceles were examined. Of these, 12 cases with complete and accurate medical records were evaluated in detail. The successful correction of frontoethmoidal encephaloceles was shown to depend on the following: a detailed understanding of the pathological anatomy (such as interorbital hypertelorism rather than true orbital hypertelorism and the presence of secondary trigonocephaly), careful planning of the bone movements to correct these deformities, and attention to detail regarding the placement of scars, positioning of the medial canthi, and the nasal reconstruction. Avoiding the "long-nose" deformity often seen after repair should be a priority. In general, the authors recommend a one-stage repair with both a transcranial and external approach.

The tuberous breast deformity is one of the most challenging congenital breast anomalies. The nomenclature, classification, and treatment of this pathological condition have varied considerably. In this study, 16 patients with 23 tuberous breast deformities are evaluated. The breast deformities are classified according to the three-tier classification system used at the authors' institution. The treatment pattern is evaluated and a flexible algorithm is discussed for the treatment of the tuberous breast deformity.

The VACTERL association is an acronym for a constellation of abnormalities affecting the spinal column, anus, heart, trachea, esophagus, kidneys, and limbs that are seen in newborns. It has been shown that prenatal rats exposed to Adriamycin demonstrate a similar series of anomalies. It is the aim of this study to characterize the spectrum of limb anomalies in this model. Female Sprague-Dawley rats (N = 18; 14 experimental and 4 control) were used. After mating, pregnant animals received intraperitoneal injections of Adriamycin (2 mg per kilogram) on days 6, 7, 8, and 9 of gestation. Fetuses were recovered on day 21 of gestation. Ten experimental litters (42 fetuses) and two control litters (18 fetuses) underwent skeletal staining with Alizarin Red S stain. Four experimental litters (N = 18) and two control litters (N = 17) underwent histological study including staining for vascular and neural structures. Upper limb anomalies were found in 45% of the experimental animals, all including either hypoplasia or bending of the radius. The ulna was involved in 37% of the affected limbs. There were no isolated ulnar deformities. There were no histological differences noted. The spectrum of upper limb anomalies seen in the Adriamycin-exposed prenatal rat closely approximates, in incidence and morphology, the upper limb deformities seen with the VACTERL association in children. It is a reliable model for use in additional studies of the embryological mechanism by which these defects arise.

The great advantages of the radial forearm fasciocutaneous flap are offset by the dilemmas associated with its donor site. The most commonly encountered problems are the functional and cosmetic sequelae of skin graft loss with consequent exposure of volar forearm tendons, the superficial sensory branch of the radial nerve, and the radius. Techniques that minimize donor site morbidity are those that provide a well-vascularized bed for the protection of exposed tendon, nerve, and bone, and for the acceptance of skin grafts. A technique involving the mobilization of the flexor pollicis longus muscle belly in conjunction with the abductor pollicis longus with approximation to the flexor digitorum superficialis muscle belly provides stable, well-vascularized coverage of the radial forearm flap donor site, with reliable skin graft acceptance and no functional sequelae.

Purpose: This study attempted to identify differential cytokeratin expression in cystic jaw lesions using immunohistochemical staining., Patients and Methods: The charts from selected patients treated between 1983 and 1994 for jaw cysts were evaluated. Twenty-four paraffinized specimens were selected randomly for investigation with 5 immunohistochemical stains. The 4 diagnostic categories included ameloblastoma, dentigerous cyst, odontogenic keratocyst (OKC), and recurrent odontogenic keratocyst in patients with nevoid basal cell carcinoma (NBCC) syndrome. The 5 immunohistochemical stains included antibodies to cytokeratins 13, 17, and 18; CAM 5.2; AE 1/3; and carcinoembryonic antigen (CEA)., Results: Differential staining of OKCs from patients with and without NBCC syndrome was found only with the antibody to cytokeratin 17. Furthermore, staining of OKCs in syndromic patients appeared to be stronger and more uniform than in nonsyndromic patients., Conclusions: These findings suggest that immunohistochemical staining for cytokeratin 17 may aid in the diagnosis of OKCs and may be used to further subdivide these lesions based on the presence or absence of NBCC syndrome.

The lips are a complex laminated structure. When lost through injury or disease, they present a complex reconstructive challenge. The facial artery musculomucosal (FAMM) flap is a composite flap with features similar to those of lip tissue. In this article, the anatomy, dissection, and clinical applications for the use of the FAMM flap in lip and vermilion reconstruction are discussed. A series of 16 FAMM flaps in 13 patients is presented. Seven patients had upper-lip reconstruction and six had lower-lip reconstruction. Superiorly based FAMM flaps were used in eight patients, and eight inferiorly based flaps were performed in five patients. Three patients had bilateral, inferiorly based flaps. In summary, the FAMM flap is a local flap that can be used for lip and vermilion reconstruction. Although not identical to the lip, it has many similar features, which make it an excellent option for lip reconstruction.

Giant congenital pigmented nevi pose a substantial reconstructive challenge for the treating physician. Due to the increased risk of malignant transformation in such lesions, complete excision with tissue expansion or skin grafting is the generally accepted treatment. These modalities can, however, leave the patient with secondary deformities that also require complex reconstructive procedures. The following case details a patient requiring secondary reconstruction with large-volume tissue expansion 12 years after excision of a giant nevus, and split-thickness skin grafting. This patient illustrates a severe secondary deformity and the usefulness of large-volume serial expansion in such patients.

Degloving injuries range from the occult, easily missed injury to obvious massive tissue damage. The serious nature of these wounds is exacerbated by mismanagement. It is generally accepted that the degloved tissue should be excised, defatted, fenestrated, and reapplied as a full-thickness skin graft. Dressings are required that provide gentle, evenly distributed pressure and avoid shear stress to the newly grafted skin. Numerous types of dressings have been devised but all are cumbersome and time-consuming. We have found the Vacuum-Assisted Closure device to be a rapid, effective, and easy-to-use alternative to traditional methods. The authors examine their experience using a vacuum-assisted closure device to treat nine degloving injuries in 5 patients and discuss the important aspects in using this technique.

Cardiac metastasis from head and neck cancer is rarely encountered. We present a base-of-tongue squamous cell carcinoma with metastasis to the heart that was diagnosed antemortem. Autopsy series indicate that tongue cancer may metastasize more frequently to the heart than from other head and neck sites. However, none of these studies was controlled. Most importantly, cardiac metastasis should be suspected in any patient with cancer in whom new cardiac symptoms develop. The diagnosis is best confirmed with two-dimensional echocardiography or cardiac MRI. A myocardial or endocardial biopsy specimen can be obtained with angiographic guidance. Despite the improvement in diagnostic capability, available treatments are only palliative. All patients eventually die of their metastatic disease.

The odontogenic keratocyst (OKC) is a jaw cyst with a proclivity for local invasion and recurrence. This 20-year retrospective study was conducted to evaluate methods of treatment and recurrence rates. Forty-nine patients were identified with an average age at presentation of 39.5 years. The molar region of either the mandible or maxilla was the principal primary location; the maxillary antrum was also a common site. The majority of cysts were unilocular and associated with adjacent dentition. Initial therapy was typically enucleation with or without extraction of associated teeth; seven cases of recurrent or second primary odontogenic keratocysts required more extensive surgery. Follow-up ranged from 1 to 15 years with an average duration of 4.3 years. The overall recurrence rate was 35%, and the average time to recurrence 4 years. A recurrence rate of 60% was documented for patients with basal cell nevus syndrome or a family history thereof. Long-term follow-up is necessary following initial OKC treatment. The high rate of recurrence in patients with documented or suspected basal cell nevus syndrome suggests the need for more aggressive initial surgical management in this selected patient population.

Purpose: This study evaluated the positional relationship of the descending palatine artery to the Le Fort I osteotomy., Materials and Methods: Three separate examinations were performed. In the first, 30 human skulls were used, and measurements were made of the greater palatine canal and foramen in relation to maxillary landmarks pertaining to the Le Fort I osteotomy. In the second, 40 patients with normal or minimal sinus mucosal thickening were selected from a pool of patients who underwent computed tomography (CT) scanning for sinus evaluation. These patients were scanned on a Somatome Plus spiral CT scanner as part of a routine sinus protocol, with the addition of an axial image 3 mm above the nasal floor where the Le Fort I osteotomy is usually performed. The distance from the greater palatine canal to the piriform rim was measured. In the third, eight fresh cadavers were used, and the distance from the internal maxillary artery to the nasal floor was measured., Results: The internal maxillary artery enters the pterygopalatine fossa approximately 16.6 mm above the nasal floor and gives off the descending palatine artery. The descending palatine artery travels a short distance within the pterygopalatine fossa and then enters the greater palatine canal. It travels approximately 10 mm within the canal in an inferior, anterior, and slightly medial direction to exit the greater palatine foramen in the region of the second and third molars., Conclusion: Injury to the descending palatine artery during Le Fort I osteotomy can be minimized by not extending the osteotomy more than 30 mm posterior to the piriform rim in females. This distance can be extended to 35 mm in males. Pterygomaxillary separation should be made by closely adapting the cutting edge of a curved osteotome or right-angled saw to the pterygomaxillary fissure while avoiding excessive anterior angulation. Furthermore, the superior cutting edge of the osteotome or saw blade should be less than 10 mm above the nasal floor.

Objective: To review the characteristics and treatment of odontogenic keratocysts in the pediatric population at our institution in light of a comprehensive literature review of odontogenic keratocysts in the general population in the hope of elucidating clinical, radiological, or pathological factors that would suggest a different therapeutic approach to odontogenic keratocysts in the pediatric as opposed to the adult population., Design: A 19-year retrospective medical chart review of children with mandibular or maxillary masses of odontogenic keratocyst origin., Setting: Two academic tertiary care institutions., Patients: Eleven children had pathologically confirmed odontogenic keratocysts. Age at diagnosis ranged from 8 to 18 years (mean, 13.4 years)., Results: A cystic mass with dentition displacement was characteristic clinically and radiographically. Treatment principally consisted of enucleation with or without extraction of teeth. Follow-up ranged from 1 to 8 years. Seven patients remained free of disease. Recurrences or second primary lesions occurred in 4 patients, all of whom had a family history of nevoid basal cell carcinoma syndrome or multiple cysts suggestive of this diagnosis. The maximum 8-year interval between initial treatment and recurrence is noteworthy., Conclusions: The diagnosis of odontogenic keratocyst deserves consideration in children who have a mass of the mandible or maxilla. The clinical behavior of this lesion in its initial occurrence and response to conservative treatment seems to be similar to that reported in adults. Odontogenic keratocysts, especially those that are multiple or recurrent, should alert the clinician to the possible underlying diagnosis of nevoid basal cell carcinoma syndrome.

Background: The placement of osseointegrated dental implants is considered a minimally invasive procedure with a low complication rate. Reported complications include local trauma to neurovascular structures, mandible fractures, sinusitis, and localized gingivitis. Major life-threatening complications are extremely rare. Severe infection has not been reported in a review of the English literature., Method: Case study., Results: We present a case of life-threatening deep neck space infection resulting in descending necrotizing mediastinitis following osseointegrated dental implant placement. Treatment included intravenous antibiotics, aggressive neck debridement, and removal of the infected dental implants., Conclusion: Severe infection can result from the placement of dental implants. Principles of treatment include antibiotics, surgical drainage and debridement, and careful assessment and removal of the involved implants.