Transfacial Two-pin External Mandibular Distraction Osteogenesis: A Technique for Neonatal Airway Obstruction from Robin Sequence.

Surgical management in those with moderate-to-severe airway obstruction includes tongue-lip adhesion, tracheostomy, and/or mandibular distraction osteogenesis. This article describes a transfacial two-pin external device technique for mandibular distraction osteogenesis, utilizing minimal dissection. Methods: The first percutaneous pin is transcutaneously placed just inferior to the sigmoid notch parallel to the interpupillary line. The pin is then advanced through the pterygoid musculature at the base of the pterygoid plates, toward the contralateral ramus, and exits the skin. A second parallel pin is placed spanning the bilateral mandibular parasymphysis distal to the region of the future canine. With the pins in place, bilateral high ramus transverse corticotomies are performed. Using univector distractor devices, the length of activation varies, with the goal of overdistraction to achieve a class III relationship of the alveolar ridges. Consolidation is limited to a 1:1 period with the activation phase, and removal is performed by cutting and pulling the pins out of the face. Results: To guide optimal transcutaneous pin placement, transfacial pins were then placed through twenty segmented mandibles. Mean upper pin (UP) distance was 20.7 ± 1.1 mm from the tragus. The distance between the cutaneous entry of the UP and lower pin was 23.5 ± 0.9 mm, and the tragion-UP-lower pin angle was 118.7 ± 2.9°. Conclusions: The two-pin technique has potential advantages regarding nerve injury and mandibular growth, given an intraoral approach with limited dissection. It may safely be performed on neonates whose small size may preclude the use of internal distractor devices.

Digital-Facial Translocation in Amniotic Band Sequence: Evidence of the Intrinsic Theory.

Amniotic band sequence is a complex congenital anomaly in which infants with typically no known genetic mutation have bands of maternal amniotic tissue wrapped around body parts, most commonly the limbs and digits. The authors report a novel variation on this presentation in 3 patients from 2 centers with complex craniofacial clefting and amniotic band sequence. They presented with hypertelorism, different forms of complex craniofacial clefting, and bands connecting ipsilateral hands to facial clefts, with digital-facial translocation in 2 cases. These findings support a model in which complex craniofacial clefts result in areas of exposed, sticky, and temporally and spatially coincident mesenchyme within the embryo that are susceptible to adherence of ipsilateral fetal hands. This strongly supports the intrinsic and adhesion theories of the etiology of amniotic band syndrome.

Management of Airway Obstruction in Infants With Pierre Robin Sequence.

BACKGROUND: Pierre Robin sequence (PRS)-related airway obstruction is often treated surgically; however, objective measures predicting the need for surgery are poorly defined. METHODS: A retrospective chart review was performed on 171 neonates with PRS. Infants were grouped based upon intervention modality: nonsurgical (conservative) or surgical [mandibular distraction osteogenesis (MDO) or tracheostomy]. Demographic data, physical examination findings, and study results were compared between groups to determine risk factors for surgical intervention, and to predict long-term success or failure of those interventions. RESULTS: The most significant, objective risk factor among those receiving surgery was a poor preintervention sleep study [obstructive index (OI): 42.4 versus 12.9 for the conservative treatment group; P < 0.001]. Only 11% of those treated conservatively had an OI >20, whereas 67.5% of those treated surgically met this severity measure. Of those receiving surgery, tracheostomy was associated with neurologic impairment (P = 0.030) and low birth weight (P = 0.046) compared with the MDO group. Together with syndromic status, these risk factors were useful for predicting failure of MDO to avoid subsequent tracheostomy (test sensitivity and specificity were 64.2% and 100.0%, respectively). No long-term differences in speech or micrognathia were detected between the 3 groups; however, those treated conservatively or with MDO had improved long-term feeding and airway obstruction outcomes compared with the tracheostomy group. CONCLUSIONS: Surgical intervention for PRS-related tongue-based airway obstruction should be strongly considered with an OI >20. Tracheostomy should be reserved for complex patients with concomitant syndromic diagnosis, neurologic impairment, and low birth weight.

Decannulation and Airway Outcomes With Maxillomandibular Distraction in Treacher Collins and Nager Syndrome.

BACKGROUND: Treacher Collins syndrome is a rare disorder (1/50,000 live births) with features that include hypoplastic orbitozygomatic complex with downward slanting eyes, and maxillary/mandibular retrusion. Obstructive sleep apnea and tracheostomy-dependence are common. This study presents the outcomes of skeletal distraction on avoidance of tracheostomy and decannulation in this patient population. METHODS: The authors reviewed charts of all patients with Treacher Collins syndrome who underwent craniofacial reconstruction from 2003 to 2016. Primary outcome measures included decannulation of tracheostomy dependent patients and avoidance of tracheostomy. Secondary outcome measures included cephalometric parameters, polysomnography scores, and airway exposure scores on direct laryngoscopy. RESULTS: Twenty-five patients underwent mandibular and maxillary advancement to resolve upper airway obstruction. Mandibular distraction was performed in 24 of 25 patients, and maxillary distraction in 14 of 25 patients. Maxillary distraction was combined with mandibular distraction in 13 of 17 to accomplish greater advancement and counter-clockwise rotation of the entire maxillary-mandibular complex. Six of 7 patients, 85.7%, avoided a tracheostomy and 39% (7 of 18) were decannulated. Cephalometric changes in sella-nasion-A point, sella-nasion-B , occlusal plane angle, and posterior airway space were equivalent between the groups who were able to clear their obstruction and those who were not. CONCLUSIONS: Treacher Collins is a very challenging disease in which to resolve airway obstruction. Thus, thorough evaluation of the entire airway for all levels of obstruction is critical to successful outcomes. Future collaborative efforts between multiple institutions can help to increase our understanding and effective management of this rare disease.

Neonatal Mandibular Distraction Without a Consolidation Period: Is It Safe? Is it Effective?

PURPOSE: Consolidation is a standard part of most post-distraction protocols. This study aims to determine whether the deliberate omission of a consolidation period following mandibular distraction (MD) in neonates with Pierre Robin sequence (PRS) affected the airway outcomes and complication rates. PATIENTS AND METHODS: A retrospective chart review of 28 neonates with PRS who underwent MD for severe airway obstruction between 2009 and 2014 was performed. Neonates were split into 2 cohorts: those with a very short or no consolidation (no consolidation group, n = 18) and those with a traditional consolidation period (consolidation group, n = 10). RESULTS: Mean consolidation length was 25 days in the conventional consolidation group and 1.5 days in the no consolidation group (P < 0.01). Postoperatively, both groups showed equivalent improvements in their obstructive index as measured by polysomnography. Compared with the patients who underwent convention consolidation, patients in the "no consolidation" group did not require a greater number of repeat distraction, supplemental oxygen, or tracheostomy. Nor was there any significant difference in the number of complications between the 2 groups. CONCLUSION: In neonates with PRS treated with MD for severe airway obstruction, the omission of a consolidation period does not appear to significantly affect the complication rate or resolution of airway obstruction.

Enzyme replacement therapy for congenital hypophosphatasia allows for surgical treatment of related complex craniosynostosis: a case series.

Hypophosphatasia (HPP) is a rare inherited disorder of bone metabolism that results in the loss of function of the gene coding for tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have defective bone mineralization as well as craniosynostosis that can be seen in the infantile and childhood forms of this disease. Traditionally, HPP has had a poor prognosis, with few children surviving to exhibit the phenotype of clinical craniosynostosis that requires surgical intervention. Here, the authors report on new advancements in enzyme replacement therapy (ERT) for children affected by HPP, allowing these patients to survive and undergo surgery to address complex craniosynostosis. The authors discuss their case series of 4 HPP patients treated at their institution with ERT who have undergone successful surgical intervention for craniosynostosis. These children had no complications related to their surgeries and exhibited decreased neurological symptoms following cranial vault remodeling. This study reveals that ERT administered either pre- or post- operatively paired with cranial vault remodeling strategies can yield improved neurological outcomes in children affected by HPP.

Pediatric frontal sinus fractures: outcomes and treatment algorithm.

Pediatric frontal sinus fractures are a rare clinical entity. Owing to the large amount of force required to fracture the frontal sinus, it is often associated with severe intracranial and craniofacial injuries. The treatment of frontal sinus fractures is controversial, with many different established algorithms based mainly on the adult population. The authors present their experience with pediatric frontal sinus fractures; they also present a treatment algorithm. A retrospective review of the Cincinnati Children's Hospital Medical Center trauma database was performed. From 1998 to 2010, the authors identified patients between the ages of 0 and 18 with frontal sinus fractures and analyzed demographics, fracture pattern, associated injuries, methods of treatment, and complications. Descriptive statistics and univariate analyses were performed.A total of 39 patients were included in the study with a mean follow-up of 31.2 months. Fractures of the anterior and posterior table with displacement greater than one table width were significantly associated with higher hospital costs, higher velocity mechanism of injuries, lower Glasgow Coma Scale scores, nasofrontal outflow tract (NFOT) involvement, and cerebrospinal fluid leak. There were no differences in short- and long-term complications. Additionally, these patients were more likely to be treated surgically in the form of obliteration or cranialization.Patients without NFOT involvement can be managed with observation only. Patients with NFOT involvement or persistent cerebrospinal fluid leak should be treated with obliteration or cranialization, respectively, to reduce the risk of severe complications.

Cost analysis of mandibular distraction versus tracheostomy in neonates with Pierre Robin sequence.

OBJECTIVE: To evaluate costs associated with surgical treatment for neonates with Pierre Robin sequence (PRS). STUDY DESIGN: Retrospective cohort study. SETTING: Cincinnati Children's Hospital Medical Center. SUBJECTS AND METHODS: With Institutional Review Board approval, we retrospectively studied neonates with PRS treated from 2001 to 2009 with either tracheostomy (Trach), mandibular distraction (MD), or Trach with subsequent MD (Trach+MD). Actual charges over a 3-year period associated with operative costs, hospital stay, imaging and sleep studies, clinic visits, and related emergency room visits were collected. Home tracheostomy care charges were estimated individually for each patient. Charges were compared using regression and appropriate statistical analyses. RESULTS: Forty-seven neonates were included in the study (MD, n = 26; Trach, n = 12; Trach+MD, n = 9). Trach group patients had 2.6-fold higher charges than the MD group despite no difference in length of hospital stay. This difference increased to 7.3-fold when including home trach care-related costs. Trach+MD group patients had longer hospital lengths of stay and higher operation room (OR) fees, but no increased total charges compared with the Trach only group. CONCLUSIONS: For patients with severe PRS, mandibular distraction provides significant cost savings over tracheostomy ($300,000 per patient over 3 years). Increased costs with tracheostomy come from greater hospital-related charges, more frequent airway procedures, a higher incidence of gastrostomy tube feeds, and home trach care costs. A careful examination of long-term outcomes will be critical as mandibular distraction continues to gain acceptance for treatment of PRS.

Evolution in minimal-incision palatoplasty: surgical technique and outcomes in 67 consecutive cases.

BACKGROUND: Conventional palatoplasty relies on extensive mucosal incisions and isolation of flaps on the palatine vessels to facilitate midline closure and velar reconstruction. This introduces substantial scarring, which has adverse effects on vascularity and growth. The authors have developed a minimally invasive palatoplasty technique that may have advantages over traditional techniques. The authors present their operative experience and outcomes when using the minimally invasive method paired with a novel cranial base maneuver for nasal mucosa closure. METHODS: Based on cadaveric dissections, the authors developed a modified minimal-incision approach that permits anatomical reconstruction from a midline approach. From 2003 to 2010, a retrospective review was performed on 67 consecutive minimal-incision palatoplasties. Cases requiring relaxing incision and/or conversion to other palatoplasty techniques were compared with minimal incision alone. RESULTS: Minimally invasive palatoplasty alone was able to be performed in 78 percent (n=52) of all cases. Fistula rates were 7.6 percent (n=4) in the minimally invasive palatoplasty group and 20 percent (n=3) in the relaxing/conversion group (p=0.04). Of patients requiring relaxing incisions/conversion, a higher percentage were syndromic (73 percent; p=0.01). Eighty-nine percent of all Veau class I defects were able to be successfully closed with the minimally invasive palatoplasty approach (p=0.01). CONCLUSIONS: Minimal-incision palatoplasty paired with a cranial base maneuver for nasal mucosa elevation results in adequate soft-tissue mobility and length to arrive at a tension-free closure. Fistula and velopharyngeal insufficiency rates are comparable to that of other techniques, and theoretical advantages of this technique will be borne out by longer term follow-up. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Outcomes of mandibular distraction osteogenesis in the treatment of severe micrognathia.

IMPORTANCE: Patients with severe micrognathia are predisposed to airway obstruction. Mandibular distraction osteogenesis (MDO) is an alternative to tracheotomy that lengthens the mandible in order to improve the retrolingual airway. This study presents outcomes from one of the largest cohorts reported. OBJECTIVE: To assess the rate and predictors of surgical success and complications among (1) patients who underwent MDO prior to other airway procedures (MDO first), and (2) patients who required an initial tracheotomy and were subsequently treated with MDO (tracheotomy first). DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study at a tertiary care pediatric medical center of patients diagnosed as having micrognathia resulting in symptomatic airway obstruction (Pierre Robin sequence) and who underwent MDO from September 1995 to December 2009. INTERVENTIONS: Electronic medical records were reviewed. Multivariable regression analysis was used to assess for predictors of outcome. MAIN OUTCOMES AND MEASURES: Rates of surgical success (defined as either tracheotomy avoidance or decannulation) and complications. Potential predictors included demographics, syndrome presence, follow-up time, and surgical history. RESULTS: A total of 123 patients (61 in MDO-first subgroup, 62 in tracheotomy-first subgroup) underwent MDO during the study period. Median age at time of distraction was 21 months (range, 7 days-24 years). Surgical success and complication rates were 83.6% and 14.8% in the MDO-first subgroup and 67.7% and 38.7% in the tracheotomy-first subgroup. Tracheotomy-first patients were more likely to have a syndromic diagnosis (66.0% vs 43.0%; P = .009) and were older at the time of MDO (median age, 30 months vs 5.1 months; P < .001). Poorer odds of success were associated with the need for 2 or more other airway procedures (odds ratio [OR], 0.14 [95% CI, 0.02-0.82]) in the MDO-first subgroup and craniofacial microsomia or Goldenhar syndrome (OR, 0.07 [95% CI, 0.009-0.52]) in the tracheotomy-first subgroup. CONCLUSIONS AND RELEVANCE: Mandibular distraction osteogenesis has a high rate of success in avoiding tracheotomy. Patients who required a tracheotomy before MDO had a lower success rate in achieving decannulation and a higher rate of complications. However, these patients also had a higher rate of syndromic diagnoses and associated comorbidities. Patients with Goldenhar syndrome have a decreased likelihood of surgical success.

Extended composite temporoparietal fascial flap: clinical implications for tissue engineering in mandibular reconstruction.

BACKGROUND: The authors have expanded upon a well-described and widely used flap in the head and neck region. The purpose of the cadaver study was to determine the feasibility, angiosome, and the potential application of this pedicled flap in bone tissue engineering of the mandible. METHODS: A total of 6 fresh human cadaver heads were dissected for a total of 12 flaps. The extended composite temporoparietal fascial flap, based on the superficial temporal artery (STA) and including cranial periosteum, was dissected and the dimensions were measured. Through a combined submandibular and preauricular incision, the mandible was exposed and the dimensions were measured from the sigmoid notch to the gonion angle and from the gonion angle to the symphysis. CT angiography and silicone injections were performed to identify the vascular anatomy of the flap. RESULTS: The combined distance from the sigmoid notch to the gonion and the gonion to the symphysis, plotted versus the cranial apex to tragus length, demonstrated adequate flap dimensions in all specimens for hemi-mandibular reconstruction. The average flap length was 16.5 ± 1.40 cm and the average flap width was 11.4 ± 0.98 cm, resulting in an average flap surface area of 94.5 ± 13.08 cm. Radiographic images and silicone injections confirmed STA perfusion of the cranial periosteum. CONCLUSIONS: The extended composite temporoparietal fascial flap with periosteum can be a viable option for providing vascularized periosteum in tissue-engineered craniofacial reconstruction.

TGFß signaling regulates lipogenesis in human sebaceous glands cells.

BACKGROUND: Sebaceous glands are components of the skin essential for its normal lubrication by the production of sebum. This contributes to skin health and more importantly is crucial for the skin barrier function. A mechanistic understanding of sebaceous gland cells growth and differentiation has lagged behind that for keratinocytes, partly because of a lack of an in vitro model that can be used for experimental manipulation. METHODS: We have developed an in vitro culture model to isolate and grow primary human sebocytes without transformation that display functional characteristics of sebocytes. We used this novel method to probe the effect of Transforming Growth Factor ß (TGFß) signaling on sebocyte differentiation, by examining the expression of genes involved in lipogenesis upon treatment with TGFß1. We also repressed TGFß signaling through knockdown of the TGFß Receptor II to address if the effect of TGFß activation is mediated via canonical Smad signal transduction. RESULTS: We find that activation of the TGFß signaling pathway is necessary and sufficient for maintaining sebocytes in an undifferentiated state. The presence of TGFß ligand triggered decreased expression in genes required for the production of characteristics sebaceous lipids and for sebocyte differentiation such as FADS2 and PPARγ, thereby decreasing lipid accumulation through the TGFß RII-Smad2 dependent pathway. CONCLUSION: TGFß signaling plays an essential role in sebaceous gland regulation by maintaining sebocytes in an undifferentiated state. This data was generated using a novel method for human sebocyte culture, which is likely to prove generally useful in investigations of sebaceous gland growth and differentiation. These findings open a new paradigm in human skin biology with important implications for skin therapies.

External fixation in a low-velocity gunshot wound to the mandible.

Low-velocity gunshot wounds to the mandible are complex injuries that can be aesthetically and functionally devastating. Despite advances in plating systems and surgical techniques, repair of such injuries remains a challenging endeavor. Traditionally, external fixation has resulted in longer treatment times and the need for revision surgery. Rigid fixation has many proponents because of shorter postoperative treatment times and fewer complications. We report a case of a low-velocity gunshot injury to the mandible with comminution and a full-thickness soft tissue wound treated definitively with maxillomandibular fixation and an external fixation device.

A Drosophila model of the neurodegenerative disease SCA17 reveals a role of RBP-J/Su(H) in modulating the pathological outcome.

Expanded polyglutamine (polyQ) tract in the human TATA-box-binding protein (hTBP) causes the neurodegenerative disease spinocerebellar ataxia 17 (SCA17). To investigate the pathological effects of polyQ expansion, we established a SCA17 model in Drosophila. Similar to SCA17 patients, transgenic flies expressing a mutant hTBP protein with an expanded polyQ tract (hTBP80Q) exhibit progressive neurodegeneration, late-onset locomotor impairment and shortened lifespan. Microarray analysis reveals that hTBP80Q causes widespread and time-dependent transcriptional dysregulation in Drosophila. In a candidate screen for genetic modifiers, we identified RBP-J/Su(H), a transcription factor that contains Q/N-rich domains and participates in Notch signaling. Knockdown of Su(H) by RNAi further enhances hTBP80Q-induced eye defects, whereas overexpression of Su(H) suppresses such defects. While the Su(H) transcript level is not significantly altered in hTBP80Q-expressing flies, genes that contain Su(H)-binding sites are among those that are dysregulated. We further show that hTBP80Q interacts more efficiently with Su(H) than wild-type hTBP, suggesting that a reduction in the fraction of Su(H) available for its normal cellular functions contributes to hTBP80Q-induced phenotypes. While the Notch signaling pathway has been implicated in several neurological disorders, our study suggests a possibility that the activity of its nuclear component RBP-J/Su(H) may modulate the pathological progression in SCA17 patients.

Endoscopic monobloc advancement with ultrasonic osteotomy: a feasibility study.

BACKGROUND: The monobloc procedure has been criticized owing to its tendency for cerebrospinal fluid leak, relapse, infection, and incomplete ossification. Such risks have been decreased through gradual advancement of the monobloc via distraction osteogenesis. This cadaver study was undertaken to develop an endoscopic, ultrasonic monobloc osteotomy to further minimize risks and potentially improve outcomes. METHODS: Three fresh, adult human cadavers were used in this study. Endoscopic ultrasonic monobloc osteotomy was completed in all cadavers with 3 incisions hidden in the hair-bearing scalp. The incisions afforded access for small craniotomies through which the dura was dissected from the frontal bones. An ultrasonic scalpel and endoscope then traveled extradurally to osteotomize the frontal bones, temporal bones, sphenoid wings, and superior aspects of the orbits intracranially. Pterygomaxillary dysjunction was performed with conventional osteotomes intraorally. RESULTS: The endoscopic ultrasonic monobloc osteotomy was completed as a single fragment in all 3 cadavers. No additional incisions were required. Completeness of the osteotomy and integrity of the single fragment were evaluated by manual examination and endoscopic visualization of free movement at osteotomy sites. Osteotomy completion took less than 2.5 hours. Dura and periosteum surrounding all osteotomies remained intact, eliminating concern for injury to adjacent soft tissue. Careful placement of temporal incisions and craniotomies was critical to facilitate completion of osteotomies in a clinically safe manner. CONCLUSIONS: We have demonstrated the feasibility of an endoscopic ultrasonic monobloc advancement technique in cadavers. The technique can be completed without a bicoronal incision while completely protecting all vital structures. The preservation of vascularity and periosteum afforded by this technique may provide improved outcomes and reduced complications.

Endoscopically assisted Le Fort III osteotomy using an ultrasonic scalpel: a feasibility study in cadavers.

Drawbacks to conventional Le Fort III osteotomy include bleeding, infection, relapse, and scar at the coronal incision. We performed an endoscopically assisted Le Fort III osteotomy with an ultrasonic scalpel in cadavers to develop a new technique that minimizes such complications. Endoscopically assisted Le Fort III osteotomy was performed in 3 fresh, adult human cadavers. Access incisions included the transconjunctival lower lid with lateral canthotomy, the lateral upper gingivobuccal sulcus, and a stab incision in the medial aspect of the upper eyelid. Osteotomies at the zygomaticofrontal suture, the lateral orbital wall, the orbital floor, and the medial orbital wall were carried out with an ultrasonic scalpel under direct and endoscopic visualization from the trasconjunctival incision. The zygomatic arch and the pterygomaxillary region were osteotomized via the upper gingivobuccal sulcus incisions. The nasofrontal junction and the septum were accessed and cut via a stab incision in the medial upper eyelids. Disimpaction was completed with minor digital pressure inferiorly.Each of the 3 Le Fort III osteotomies was complete, and mobility was checked by manual examination. There was a steep learning curve to the operation, but the final cadaver dissection took 99 minutes to complete. The ultrasonic scalpel provided for maximal ease in cutting bone and minimal disruption to adjacent soft tissues as judged by postoperative direct examination. This cadaver study demonstrates the feasibility of a minimally invasive, endoscopically assisted Le Fort III osteotomy using an ultrasonic scalpel. Further experimental work combined with refinements in technique and equipment will help bring this advancement into clinical application.

Endoscopically assisted Le Fort I osteotomy using an ultrasonic scalpel: a feasibility study in cadavers.

PURPOSE: Drawbacks to conventional Le Fort I osteotomy include bleeding, infection, and relapse. The open approach predisposes itself to each of these complications through various means. We performed an endoscopically assisted Le Fort I osteotomy with an ultrasonic scalpel in cadavers to develop a new technique that minimizes such complications. MATERIALS AND METHODS: Endoscopically assisted Le Fort I osteotomy was performed in 4 fresh adult human cadavers. Two 1-cm gingivobuccal sulcus incisions were used to approach the maxilla. Osteotomies were carried out with an ultrasonic scalpel from within the maxillary sinus under endoscopic visualization after a small antrostomy was made in its anterior wall. The external periosteal sleeve to the maxilla remained intact, eliminating the risk of massive hemorrhage and preserving bony vascularity. A stab incision was made with a 2-mm osteotome above the anterior nasal spine to separate the caudal septum from the Le Fort I segment. Pterygomaxillary disjunction was also performed with classic osteotomes. Disimpaction was completed with minor digital pressure inferiorly. RESULTS: Each of the 4 Le Fort I osteotomies was complete, and mobility was checked by manual examination. There was a steep learning curve to the operation, but the final cadaveric dissection took 37 minutes to complete. At no time did the ultrasonic scalpel violate the maxillary periosteum as judged by postoperative direct examination. DISCUSSION: This cadaveric study shows the feasibility of an endoscopically assisted Le Fort I osteotomy by use of an ultrasonic scalpel. Further experimental work combined with refinements in technique and equipment will help bring this advancement into clinical application.

Mandibular distraction for micrognathia and severe upper airway obstruction.

OBJECTIVE: To determine whether the use of mandibular distraction osteogenesis (DOG) can help to avoid tracheotomy or achieve decannulation in patients with mandibular hypoplasia and severe upper airway obstruction. DESIGN: Retrospective medical record review (spanning a 27-month period). SETTING: Tertiary care children's hospital. SUBJECTS: Group A (n=8) was composed of infants with Pierre Robin sequence and no tracheotomy (mean age, 2.5 months); group B (n=6), older nontracheotomized micrognathic children with obstructive sleep apnea (OSA) (mean age, 69 months); and group C (n=12), tracheotomized children with complex congenital syndromes (mean age, 33 months). INTERVENTION: Bilateral mandibular DOG with endoscopic (n=24) and/or radiographic (n=17) airway evaluation (mean follow-up, 16 months [range, 2-42 months]). OUTCOME MEASURES: Group A, tracheotomy avoidance; group B, resolution of OSA (clinically or on polysomnography); and group C, decannulation. RESULTS: Group A, 7 patients (88%) successfully avoided tracheotomy; group B, 5 patients (83%) had resolution of OSA; and group C, 2 patients (17%) underwent decannulation. CONCLUSIONS: Mandibular DOG (1) allows tracheotomy avoidance in infants with isolated Pierre Robin sequence and (2) relieves OSA in older micrognathic children without tracheotomy. However, mandibular DOG does not frequently lead to decannulation in tracheotomized patients with complex congenital syndromes.