Staged Surgical Correction of Severe Panfacial and Transverse Nasal Root Asymmetry in Unicoronal Synostosis.

Nasal asymmetry is widely acknowledged to be one of the most difficult deformities to manage. Most reports in the literature pertain to corrective methods in relation to isolated deformity of the dorsum in the posttraumatic patient. There is a paucity of literature relating to management of nasal radix asymmetry, and still less in the context of severe panfacial asymmetry.

Spectrum of Antley-Bixler syndrome.

Antley-Bixler syndrome (ABS) is an exceptionally rare craniosynostosis syndrome characterized by radiohumeral synostosis present from the perinatal period. There is a wide spectrum of anomalies seen within ABS, and other features include midface hypoplasia; choanal stenosis or atresia; multiple joint contractures; visceral anomalies, particularly of the genitourinary system; and impaired steroidogenesis. The condition of ABS is curious in that mutations of 2 separate genes have been identified and that there seem to be subtle phenotypic differences between the 2 genotypes. Mutations of the P450 oxidoreductase gene have been reported in those patients with genital anomalies and/or impaired steroidogenesis, and the S351C mutation of the fibroblast growth factor receptor 2 gene has been reported predominantly in those patients with normal genitalia and steroidogenesis. We report a series of 4 patients with ABS and review their main findings and management.

Nasolabial angle and nasal tip elevation changes in profile view following a Le Fort I osteotomy with or without the use of an alar base cinch suture: a long-term cohort study.

OBJECTIVES: Cinch sutures attempt to counteract alar base widening but may lead to unintended increases in the nasolabial angle and nasal tip elevation. The aim of this investigation was to assess nasolabial angle changes after maxillary osteotomies with and without alar base cinch sutures in the short and long terms. STUDY DESIGN: Seventy-eight patients were assessed, with 51 in the cinch group (38 females, 13 males; age range 16-39 years) and 27 in the no-cinch group (12 females, 15 males; age range 17-27 years). The upper component (nasal tip elevation), lower component (lower lip inclination), and the overall nasolabial angle were measured on preoperative, postoperative, and long-term follow-up lateral cephalometric radiographs. RESULTS: The overall nasolabial angle (P = .006) and its upper component (P < .001) increased significantly in the cinch group immediately postoperatively but resolved by 6 to 12 months for the overall nasolabial angle and by 12+ months (up to 5.7 years) for the upper component. There were no significant changes in the no-cinch group. CONCLUSIONS: In the short term, the alar base cinch suture increases nasal tip elevation and the overall nasolabial angle. In the long term, there was no significant difference, suggesting that the initial nasal tip elevation resolves over time and that the cinch suture may have a limited effect on nasal tip elevation in the longer term.

The evaluation of bony union after frontofacial distraction.

Frontofacial advancement by distraction osteogenesis using the rigid external distraction device has become an accepted treatment for the deformity associated with craniofacial dysostoses (e.g., Crouzon, Apert, and Pfeiffer syndromes). The technical and physiological principles of osteogenesis distraction are well understood. This study documents the pattern of calcification at the osteotomy sites after distraction by analysis of serial three-dimensional computed tomography (CT) scans. The CT scans of 25 patients (11 with Crouzon, 6 with Apert, and 8 with Pfeiffer syndrome) were analyzed. Eleven individual areas along the osteotomy lines were assessed for evidence of bone formation. Scores were assigned within 4 categories ranging from no bone, calcification without bridge formation, a bony bridge, to complete bony infill (>95%). The scans were reviewed on 2 separate occasions by 2 independent assessors. There was high concordance both for intraobserver and interobserver scores. Rigid external distraction frame removal was undertaken after a 6-week consolidation period. All CT scan timings were calculated from this date. Of the 25 patients studied, 16 patients had CT scans available at 3 to 6 months, 12 at 9 to 12 months, and 7 at or more than 18 months. The scans were available in standard coronal slices with three-dimensional reconstructions. Bone formation is most consistently seen in the pterygoid region with calcification consistently occurring earlier and more completely in this area. Bone formation was often delayed in the orbital region and severely delayed or absent in the frontal region and zygomatic arches. There was no significant difference in the order or quality of bony union for the 3 underlying craniofacial dysostoses. This preliminary study confirms the clinical impression that bone formation after distraction is greatest in the pterygoid regions. The clinical implications of these findings are discussed.

Monobloc distraction in an infant, using the rigid external distractor: problems and solutions--a case report.

INTRODUCTION: Patients with craniofacial dysostosis frequently develop functional problems including raised intracranial pressure, ocular dysfunction, obstructive sleep apnoeas and failure to thrive. These functional problems can be treated by a number of different techniques. The monobloc frontofacial advancement has the ability to correct all of these functional problems in one procedure, but can be associated with high morbidity particularly in the young infant. AIM: A case report of a 4-month-old infant with Pfeiffers syndrome and severe functional problems treated by monobloc osteotomy and distraction using the rigid external distractor is reported. The management, problems and complications encountered with this patient are discussed together with the role of monobloc distraction in the infant. Adaptation of the rigid external distraction (RED) technique using two titanium mesh sheets to prevent penetration of the skull by the cranial pins is described. CONCLUSIONS: Monobloc frontofacial advancement in the very young can be successfully achieved using the RED frame, thus treating exophthalmus, raised intracranial pressure and upper airway problems in one operation. However, it may be associated with significant complications and should only be used for those extreme cases where the severity of the functional problems prevent treatment being delayed.

Transmigration of a maxillary canine. A case report.

In the oral cavity, transmigration is defined as a tooth that crosses the mid-line by more than half its length. Following extensive literature review, it was found that, although well documented with respect to mandibular canines, there were only two publications detailing maxillary cuspid transmigration. This report presents a case of transmigration where the left maxillary canine gradually crossed the mid-line and migrated to the right side in a patient with hemifacial microsomia and cleft palate. The Mupparapu classification of the migratory pattern of the mandibular canines is discussed. Various clinical considerations, as well as guidelines for general practitioners to diagnose and manage such a condition, are also discussed.

Oral manifestations of systemic diseases.

In the clinical examination for systemic diseases, inspection of the oral cavity is often overlooked. This area provides a wide array of clinical signs that may help the clinician establish a diagnosis. This article provides a summary of the more common oral manifestations of systemic disease.

Long-term complications associated with permanent dermal fillers.

We report a case series of patients with serious long-term complications associated with the injection of permanent dermal fillers. Although such complications are relatively rare, the consequences are potentially life-long, and the psychological and medical effects can often have a profound impact on the patient. The continued routine offering of these treatments will require doctors to communicate effectively with patients about the nature of the complications and the probability of risk compared with alternative treatments.

Effectiveness of botulinum toxin B in the treatment of drooling.

The aim of this audit was to assess the effectiveness of botulinum toxin B (NeuroBloc(®), Eisai Ltd.) in the treatment of drooling. Over a period of 18 months patients treated with NeuroBloc(®) botulinum toxin (BTX) for serious drooling (drooling score more than 7) were invited to complete a questionnaire on their experience of drooling and the impact of treatment. A total of 170 questionnaires were given to consecutive patients or carers and 145 (85%) responded. Of these, 128 (88%) reported that symptoms had improved by 6/10 or more on a visual analogue scale (VAS), and 54 (37%) reported compete resolution of drooling (10/10 VAS). A total of 139 (96%) would recommend the treatment to others.

Correcting the typical Apert face: combining bipartition with monobloc distraction.

BACKGROUND: Bipartition distraction is a novel procedure combining frontofacial bipartition and monobloc distraction. Apert syndrome and other syndromic craniofacial dysostoses are often characterized by hypertelorism, with a negative canthal axis and counterrotated orbits. Central midface hypoplasia can result in a biconcave face in both midsagittal and axial planes. Bipartition distraction can correct these facial abnormalities. METHODS: Twenty patients (19 Apert syndrome patients and one Pfeiffer syndrome patient, aged 1.6 to 21 years) underwent bipartition distraction. Severity of appearance was graded preoperatively and postoperatively as mild, moderate, or severe. Functional problems were documented by a multidisciplinary team. Central and lateral midface skeletal advancement were measured. Follow-up ranged from 15 months to 7 years. RESULTS: Bipartition distraction consistently produced more central than lateral facial advancement. Mean central advancement was 13.2 ± 5.9 mm at sella-nasion and 11.7 ± 5.4 mm at sella-A point. Lateral advancement was 4.7 ± 2.8 mm. Unbending the face improved aesthetic appearance. Airway function, eye exposure, and elevated intracranial pressure were improved. Complications included six temporary cerebrospinal fluid leaks (four needing a lumbar drain), five patients with postoperative seizures, five patients requiring Rigid External Distraction frame repositioning, one palatal fistula, one velopharyngeal incompetence, five pin-site infections, one abscess under frontal bone, three cases of sepsis, nine patients with worsened strabismus, two patients with enophthalmos, one patient with partial visual field loss, and three patients who required reintubation because of aspiration. CONCLUSIONS: : Bipartition distraction is an effective procedure with which to differentially advance the central face in Apert syndrome. It improves both function and aesthetics. CLINICAL QUESTION/LEVEL OF EVIDENCE: : Therapeutic, IV.