Faculty Bibliography

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: 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%.

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: 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.

BACKGROUND: Face transplantation allows the reconstruction of the previously nonreconstructible injury. Anthropometric landmarks are fixated to corresponding cephalometric landmarks to restore function and appearance, with emphasis on phonation, mastication, and functional upper airway. Currently, only a few face transplantations have been performed worldwide. A portion of these reconstructions involves combinations of hard and soft tissues of the midface. METHODS: Craniofacial and orthognathic considerations should be emphasized for functional effect in the planning and execution of face transplants that include both bone and soft tissue elements. These steps are taken to restore normal anatomy by fixating the midface into proper relationship with the skull base. Traditional orthognathic planning, using cephalometric parameters, often involves a line through sella and nasion as a reference for the skull base. Intraoperatively though, without a cephalograph, the sella-nasion plane is not accessible as a reference point. RESULTS: Postoperative analysis of our first face transplant recipient revealed that the Frankfort horizontal plane can alternatively serve as an accessible skull base reference point to guide the positioning of the midface. We have developed a technique to ensure fixation of the midface donor allograft in a proper functional relationship with the skull base, within 1 SD of Bolton normative data. CONCLUSIONS: "Reverse craniofacial planning" allows for precise fixation of the hard tissue components of the face transplant in relation to the skull base, as opposed to a "best fit" approach. We believe that this relationship results in the most anatomical restoration of occlusion, speech, and airway function.

BACKGROUND: : Patients with syndromic craniosynostosis and midface hypoplasia are often treated with Le Fort III advancement. The authors present four patients with extraordinarily long-term follow-up (in excess of 20 years). METHODS: : An institutional review board-approved retrospective chart review was performed on all patients with syndromic craniofacial synostosis who underwent Le Fort III advancement. Patients with greater than 20 years of cephalometric and photographic records were identified. Lateral cephalograms were obtained preoperatively, immediately postoperatively, at 1-year follow-up, and at long-term follow-up. Cephalograms were traced, digitized, and averaged. Fifty cephalometric landmarks were identified for serial measurements. RESULTS: : Of the four patients identified, one had Apert syndrome and three had Crouzon syndrome. Average age at the time of Le Fort III advancements was 11 years (range, 4 to 20 years). Average length of postoperative follow-up was 25 years (+/-5 years). No patient had significant anterior midfacial growth following Le Fort III advancement. Both young patients (<10 years) had substantial vertical inferior midfacial growth after advancement. CONCLUSIONS: : These data demonstrate that the Le Fort III segment of children with syndromic craniosynostosis does not grow significantly forward. Moreover, the traditional Le Fort III osteotomy does not provide the amount of midface advancement necessary to avoid phenotypic recidivism in these syndromic patients. This study also suggests that patients undergoing Le Fort III advancement appear to have zygomatic effacement and ptosis of the overlying soft tissue with deepening of the facial folds; collectively, it is suggested that these changes give the appearance of accelerated facial aging. CLINICAL QUESTION/LEVEL OF EVIDENCE:: Therapeutic, V

ABSTRACT: This article focuses on current practices and controversy in the area of presurgical infant orthopedics in patients born with cleft lip and palate.

BACKGROUND: Mandibular distraction has evolved from the use of external to the use of intraoral and semiburied devices. The authors highlight the evolution of the semiburied technique. The authors evaluate advantages and limitations, and report perioperative events for external and semiburied techniques to establish the indications for selection of the different devices. METHODS: A retrospective review was conducted of patients undergoing mandibular distraction at the New York University Langone Medical Center from the authors' introduction of mandibular distraction in May of 1989 to June 30, 2009. Perioperative events were stratified into three groups: minor incidents, moderate incidents, and major incidents. RESULTS: A total of 211 mandibular distraction procedures were performed: 129 external procedures on native bone, 37 external procedures on grafted bone, and 45 semiburied procedures on native bone. Minor incidents were more common with the semiburied device (62 percent) compared with external devices on native (26 percent) and grafted (38 percent) bone. There were fewer moderate incidents with the semiburied device (18 percent) than with the external device on native (22 percent) and grafted (30 percent) bone. In contrast to the external technique, no major incidents were seen with semiburied distraction. CONCLUSIONS: The semiburied device reduces scarring and has the mechanical advantages of being applied directly to the bone, less vulnerable to dislodgment, and more favorable for a vertical vector. However, its use requires more bone stock and it has the disadvantage of requiring a second operation for removal. Semiburied distraction is safe, reliable, and indicated for lengthening of the hypoplastic mandible where there is adequate bone stock for its attachment

BACKGROUND: Patients with Apert, Crouzon, and Pfeiffer syndromes who have severe midfacial hypoplasia are treated by Le Fort III midface advancement. The purpose of this study was to examine long-term (10-year) midface skeletal stability and growth following Le Fort III midface advancement in growing children. METHODS: A review of 192 patients with syndromic craniosynostosis treated by classic (nondistracted) Le Fort III advancement osteotomy between 1973 and 1998 was performed. Twenty-five patients met the inclusion criteria (age at surgery < 11 years and availability of cephalograms of diagnostic quality before treatment, after surgery, and at 1-, 5-, and 10-year follow-up). The mean age at the time of surgery was 5.8 years and the diagnosis was either Crouzon (n = 10), Apert (n = 9), or Pfeiffer (n = 6) syndrome. RESULTS: After surgery, point A advanced sagittally 10.72 mm and moved downward 3.77 mm. At 1 year, point A moved forward 0.10 mm and downward 0.47 mm. At 5 years, point A moved back 0.18 mm, whereas at 10 years it advanced 0.12 mm. During the same periods, however, pogonion came forward 5.72 mm and 7.32 mm, respectively. CONCLUSIONS: Le Fort III midface advancement in growing children with syndromic craniosynostosis is stable after the first year postoperatively. There is minimal horizontal growth of the midface between postoperative years 5 and 10, although the mandible continues to grow. Due to the differential growth rate of the midface and mandible, the facial profile becomes concave, thereby necessitating secondary midface surgery at the completion of skeletal growth

BACKGROUND:: The purpose of this study was to compare clinical outcomes and 1-year postsurgical stability with three different techniques of Le Fort III midface advancement. METHODS:: The records of 212 syndromic craniosynostosis patients were reviewed from the period 1973 to 2006. A total of 60 patients satisfied the inclusion criteria, and the mean age of the sample at surgery was 6.2 years. In group I (1977 to 1987), fixation was performed by interosseous wiring and intermaxillary fixation; in group II (1987 to 1996), fixation was achieved by only rigid plate fixation; and in group III (2000 to 2005), the patients underwent midface distraction with the rigid external distraction device. Cephalometric landmarks were identified and digitized at each of the time intervals (preoperatively, postoperatively, and 1 year postoperatively). RESULTS:: The mean advancement measured at point A in group I averaged 9.7 mm; in group II, it was 10.6 mm; and in group 3, it was 16.1 mm. There was no statistically significant difference in the amount of advancement between groups I and II. However, when groups I and II were compared with group III, there was a statistically significant difference (p < 0.05). No statistical significance was noted within and between all three groups at 1-year follow-up. CONCLUSIONS:: Significantly larger midface advancement was achieved with rigid external distraction (group III) compared with classic Le Fort III midface advancement with wire (group I) or plate (group III) fixation. At 1 year after surgery, the three groups showed relative stability of the advanced midface segment