Faculty Bibliography

BACKGROUND: The classic Le Fort III procedure was recommended in syndromic craniosynostotic children to reduce exorbitism, improve airway function, and decrease dysmorphism. This study reports on a cohort of syndromic craniosynostosis patients who have undergone early primary subcranial (classic Tessier) Le Fort III advancement and who have been followed longitudinally through skeletal maturity and beyond. METHODS: In this study, the Le Fort III advancements all occurred between the ages of 3 to 5 years, with a mean age of 4.6 years. Subsequently, these early Le Fort III patients were followed throughout development with longitudinal dental, medical, radiographic, and photographic evaluations conducted through skeletal maturity and beyond. For study inclusion, the patients had to have preoperative medical photographs and cephalometric studies at 6 months and 1, 5, and 10 years postoperatively after the primary Le Fort III advancement as well as cephalometric documentation 6 months and 1 year after the secondary midface advancement after skeletal maturity. RESULTS: After early or primary Le Fort III advancement, there was no evidence of relapse and only minimal anterior or horizontal postoperative growth of the midface. However, there was also a return of occlusal disharmony from "anticipated" mandibular growth, approaching a maximum at skeletal maturity. The dysmorphic concave facial profile and malocclusion, and airway and ocular considerations, provided the impetus for secondary midface surgery after skeletal maturity was attained. CONCLUSION: The data demonstrate that early Le Fort III advancement performed before the age of mixed dentition does not obviate the need for a secondary advancement after skeletal maturity is reached. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

BACKGROUND: The purpose of this study was to characterize soft-tissue profile changes following Le Fort III (midface) distraction in growing patients with syndromic craniosynostosis. METHODS: The cohort consisted of 20 syndromic patients who underwent Le Fort III osteotomy with midface advancement using a rigid external distraction device. The mean age at surgery was 5.7 years (range, 3 to 12.5 years). Lateral cephalograms were obtained preoperatively (time 1), after distraction device removal (time 2), and 1 year after distraction (time 3). Ten skeletal hard-tissue and 11 soft-tissue profile landmarks were identified and digitized at time points 1, 2, and 3. The x and y displacement of each landmark was studied to determine the ratios for soft- to hard-tissue change. RESULTS: The horizontal ratio of soft- to hard-tissue change for nasal dorsum to orbitale was 0.73:1 and the soft-tissue tip of nose to the anterior nasal spine was 0.86:1. The horizontal ratio of soft-tissue A point to hard-tissue A point was 0.88:1. The horizontal ratio of the upper lip position to the labial surface of maxillary incisor was 0.88:1. The ratio for nasal tip elevation to the anterior nasal spine advancement was 0.27:1. CONCLUSIONS: The result of this study supported the hypothesis that there exists a linear relationship between soft- and hard-tissue changes in the horizontal direction for the midface landmarks following Le Fort III distraction. However, there was a nonlinear relationship between soft- and hard-tissue changes in the vertical direction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

BACKGROUND AND PURPOSE: Treacher Collins syndrome is an autosomal dominant craniofacial disorder affecting derivatives of the first and second branchial arches. Given the conflicting reports in the literature regarding the extent of anterior-posterior and vertical (superior-inferior) dysplasia of the cranial base, maxilla, and mandible, this study was designed to provide a comprehensive lateral cephalometric assessment of the craniofacial morphology of unoperated patients with Treacher Collins syndrome. METHODS: The records of 45 patients with Treacher Collins syndrome registered at the Institute of Reconstructive Plastic Surgery of the NYU Langone Medical Center from the period of 1975 to 2008 were reviewed. Inclusion criteria included (1) patients between with the ages of 5 and 17 years, (2) no history of prior surgical intervention to correct skeletal deformity, and (3) diagnostic quality lateral cephalograms. Twenty patients satisfied the inclusion criteria with 12 male and 8 female patients in the sample and a mean age at the time of the lateral cephalogram of 8.8 years (range 5.2 to 16.7 years). Thirty angular and linear variables were measured to assess anterior-posterior and vertical (superior-inferior) position of the cranial base, maxilla, and mandible. Each patient's measurements were compared to age-matched and sex-matched control samples derived from the Moyers growth studies. The mean and standard deviation for each variable was then determined. RESULTS: Lateral cephalometric analysis showed decreased anterior, posterior, and total cranial base lengths and a reduced cranial base angle. The anteroposterior dimension or length of the maxilla is deficient and the maxilla is positioned posteriorly with respect to the cranial base. Both anterior and posterior facial heights (superior-inferior dimension) are decreased. Lower face height is increased, whereas total face is decreased. The maxillary and functional occlusal planes are tipped upwards posteriorly. The mandibular morphology is characteristically small in both body length and total mandibular length, and the maximum ramus width is also deficient. The mandibular plane angle and gonial angle are increased. The mandible is retropositioned. CONCLUSIONS: A lateral cephalometric analysis is described that provides a skeletal basis for the hallmark clinical findings associated with Treacher Collins syndrome. This is of importance because an understanding of the underlying skeletal dysmorphology may shed light on the etiology and growth pattern, and impacts the overall treatment planning for skeletal correction.

ABSTRACT: Among the craniosynostosis syndromes, Pfeiffer syndrome is notable because of high mortality and the need for multiple surgical interventions. However, it is variable in severity. We propose a new classification of Pfeiffer Syndrome to define pathology and function. A retrospective review was conducted of 42 patients with Pfeiffer syndrome treated from 1975 to 2010, the largest series reported to date. The classification was based on a functional assessment of patients in terms of respiratory, ocular, otological, and neurological status. This classification was tested by scoring and stratifying patients as follows: type A (mild problems), B (moderate problems), or C (severe problems). Patients were scored both at the time of presentation and after all surgical interventions to assess change in functional outcome. The functional classification system was compared to another previously reported. Type A patients did not have any change in postoperative functional outcomes (mean preoperative score 1.6, mean postoperative score 1.6); type B patients showed functional improvement (mean preoperative score 4.1, mean postoperative score 3.4) but type C patients (mean preoperative score 7.7, mean postoperative score 4.8) demonstrated the greatest improvement in functional scores after surgical intervention. Suture pathology did not indicate the clinical severity of phenotype, a variance from a previously published classification. The proposed classification is useful to assess severity of phenotype: respiratory, ocular, otologic, and neurologic problems are key indicators of the need for treatment. The classification can provide a helpful guide in multidisciplinary treatment planning, in reporting outcomes, and in the sharing of data among craniofacial anomalies centers.

ABSTRACT: The incidence of postoperative complications in cleft care is low. In this 19-year retrospective analysis of cleft lip and palate patients treated with preoperative nasoalveolar molding, we examine the incidence of postoperative oronasal fistulae. The charts of 178 patients who underwent preoperative nasoalveolar molding by the same orthodontist/prosthodontist team and primary cleft lip/palate repair by the same surgeon over a 19-year period were reviewed. Millard, Mohler, Cutting, or Mulliken-type techniques were used for cleft lip repairs. Oxford-, Bardach-, or von Langenbeck-type techniques were used for cleft palate repairs. One nasolabial fistula occurred after primary cleft lip repair (0.56% incidence) and was repaired surgically. Four palatal fistulae (3 at the junction between soft and hard palate and 1 at the right anterior palate near the incisive foramen) occurred, but 3 healed spontaneously. Only 1 palatal fistula (0.71%) required surgical repair. All 5 fistulae occurred within the first 8 years of the study period, with 4 (80%) of 5 occurring within the first 3 years. Although fistula rate may be related to surgeon experience and the evolution of presurgical techniques, nasoalveolar molding in conjunction with nasal floor closure contributes to a low incidence of oronasal fistulae.

ABSTRACT: Despite significant advances in cleft lip and palate treatment, anatomical controversies remain. Some have proposed that the width of the cleft is due to alveolar segmental displacement. Others suggest that the width is due to palatoalveolar hypoplasia. Improving our understanding of cleft anatomy may have implications for presurgical orthopedics and tissue engineering therapies. Palatoalveolar impressions of 17 noncleft children and 11 children with complete (alveolar, primary, and secondary) unilateral cleft palates were taken. Maxillary tuberosity positions and maxillary volumes were compared. Tuberosity position was determined by facebow transfer of palatoalveolar casts into geodetic datum boxes, and identification of the Cartesian coordinates (x, y, z) of the tuberosities relative to the box surfaces and Frankfurt horizontal. Maxillary volume was determined by immersing the palatoalveolar casts and measuring sand displacement. A significant difference was noted in the average tuberosity to contralateral tuberosity distance between cleft and noncleft cohorts. On average, cleft palate tuberosities were laterally displaced 8.7 mm compared with noncleft palates (P < 0.05). There was neither statistically significant alveolar segment elevation nor retroversion. A significant difference was noted in the average palatoalveolar volumes. The cleft palatoalveolar volume was 5.7 cm, and the noncleft palatoalveolar volume was 7.2 cm (P < 0.05). A palatal cleft is due to both alveolar tissue displacement and deficiency. Therefore, ideal cleft palate care should involve the correction of a displaced and deficient alveolus.

ABSTRACT: Preoperative nasoalveolar molding (NAM) in combination with primary gingivoperiosteoplasty (GPP) reduces the need for secondary alveolar bone grafting by 60% in patients with unilateral cleft lip and palate (CL/P). Herein, we investigate the efficacy of NAM and primary GPP in patients with bilateral CL/P. All patients (n = 38) with bilateral CL/P who underwent NAM and primary GPP from 1988 to 1998 with at least 14 years of follow-up were included in this study. Panoramic and periapical radiographs were used to assess dentoalveolar bone formation. A total of 38 patients were identified with median follow-up of 18 years (range 14-26 years). Of the 27 patients who underwent bilateral GPP, 14 (51%) patients had successful dentoalveolar bone formation bilaterally and 13 (49%) had unilateral bone formation. No patient had a bilateral failure. Of the 11 patients who underwent unilateral GPP, 7 (63%) patients had successful dentoalveolar bone formation. Bilateral successful dentoalveolar bone formation following primary bilateral GPP has a dependent probability of 52% and a conditional probability of 82%.