Perioperative risk factors in patients with 22q11.2 deletion syndrome requiring surgery for velopharyngeal dysfunction.

Objective : To determine the prevalence of cardiac, cervical spine, and carotid artery abnormalities in patients with 22q11.2 deletion syndrome (22q11.2DS) undergoing surgery for velopharyngeal dysfunction (VPD), associations between the presence of these abnormalities, and whether these abnormalities caused changes in surgical management or perioperative complications. Design : Retrospective review. Setting : Tertiary pediatric hospital. Patients : Seventy patients with 22q11.2DS with complete preoperative cervical vascular and spine imaging and cardiac evaluation between 1998 and 2011. Main Outcome Measures : Incidence of cardiac, cervical spine, and vascular abnormalities; related perioperative complications; and resulting changes in surgical, anesthetic, or perioperative management plan. Results : Cardiac abnormalities occurred in 45 patients (64.3%), and 8 patients required cardiac anesthesia. Thirty-eight patients (54.3%) had at least one vascular abnormality of the neck, and 14% had medial deviation of the internal carotid artery. Surgery was not performed in one patient, and the surgical plan was altered in three patients because of carotid anomalies. Cervical spine abnormalities were found in 24 patients (34.3%); 8 patients demonstrated radiographic evidence of cervical instability and were treated with spinal precautions during surgery. The presence of one anomaly was not predictive of any other finding, and there were no complications related to the heart, cervical spine, or carotid arteries. Conclusions : Anomalies of the heart, cervical spine, and cervical vasculature occur frequently in 22q11.2DS, vary drastically in severity, and are impossible to predict based on other features of the syndrome. Preoperative diagnosis of these comorbidities with routine imaging can minimize the risk of avoidable surgical complications.

A 35-year experience with syndromic cleft palate repair: operative outcomes and long-term speech function.

BACKGROUND: Associated comorbidities can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) vary from 8% to 64%, and need for secondary VPI surgery from 23% to 64%, with few studies providing long-term follow-up. The purpose of this study was to describe our institutional long-term experience with syndromic patients undergoing cleft palatoplasty. METHODS: A retrospective review was conducted of all patients with syndromic diagnoses undergoing primary Furlow palatoplasty from 1975 to 2011. Outcomes included postoperative oronasal fistula (ONF) and need for secondary VPI surgery. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh scale for speech assessment. Aggregate scores categorized the velopharyngeal mechanism as competent, borderline, or incompetent. Outcomes were analyzed by patient and operative factors. RESULTS: One hundred thirty-two patients were included with average age at repair of 20.7 months. Cleft type was 9% submucosal, 16% Veau class I, 50% class II, 12% class III, and 13% class IV. Forty-five syndromes were recorded, most commonly Stickler syndrome (n = 32) and 22q11.2 deletion syndrome [22q11.2DS (n = 19)]. Forty-four patients also had associated Pierre Robin sequence (PRS). The overall ONF rate was 4.5% and was highest in Veau class IV clefts (P = 0.048). Seventy-six patients were included in speech analysis, with an average age at last assessment of 10.4 years. Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent mechanism. Fifty percent of 22q11.2DS patients had borderline speech and none had competent speech, compared to 73.3% with Stickler syndrome (P = 0.01) and 71.4% of patients with associated PRS (P = 0.02). Secondary VPI surgery was performed in 11.4% of patients overall. Patients with PRS (13.6%) and with Stickler syndrome (15.6%) had secondary VPI surgery, compared to 31.6% of patients with 22q11.2DS (P = 0.01). CONCLUSIONS: This study demonstrates low rates of postoperative ONF after modified Furlow palatoplasty in syndromic patients. Speech outcomes were comparable to nonsyndromic patients at our institution, but patients with 22q11.2DS consistently had borderline-incompetent speech and a 3-fold higher incidence of secondary VPI surgery.

Do patients with Pierre Robin sequence have worse outcomes after cleft palate surgery?

BACKGROUND: Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often clefting of the soft and hard palate. The purpose of this study was to compare oronasal fistula rates, long-term speech outcomes, and rates of secondary surgery for velopharyngeal insufficiency (VPI) after primary cleft palate repair using the modified Furlow technique between nonsyndromic patients with and without PRS. This study also sought to determine if type of airway management in infancy correlated with speech outcomes in patients with PRS. METHODS: A retrospective review was performed of all nonsyndromic patients with clefts of the palate only who underwent repair between 1981 and 2006 at The Children's Hospital of Philadelphia using the modified Furlow technique. Patient outcomes were evaluated by the rate of postoperative oronasal fistula, speech scores at a minimum of age 5 years using the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI, and the need for secondary pharyngeal surgery for VPI. RESULTS: Fifty-five patients with PRS and 129 without PRS were included in this study. There was no significant difference in oronasal fistula rate between groups. Speech outcomes were worse in patients with PRS with 52.7% demonstrating a competent velopharyngeal mechanism, and 30.9% a borderline mechanism, compared to 72.1% and 19.4% in the non-PRS group, respectively (P = 0.035). In addition, only 76.3% of patients with PRS had no or mild hypernasality compared to 91.5% of patients without PRS (P = 0.01). The rates of nasal emission, articulation errors associated with VPI, and secondary surgery for VPI did not differ between groups. Of the patients with PRS, 36 were managed in infancy with positioning alone and 15 underwent surgical intervention for airway obstruction, and there were no significant differences in speech scores or rates of secondary surgery for VPI between these subgroups. CONCLUSIONS: Nonsyndromic patients with PRS had worse speech outcomes after modified Furlow cleft palate repair, but no significant differences were seen in the rates of secondary surgery for VPI or postoperative oronasal fistula. In addition, initial airway management in patients with PRS did not correlate with speech outcomes.

The Children's Hospital of Philadelphia modification of the Furlow double-opposing Z-palatoplasty: 30-year experience and long-term speech outcomes.

BACKGROUND: The purpose of this study was to evaluate the long-term outcomes following modified Furlow palatoplasty over a 30-year experience at a single institution. METHODS: A retrospective review was performed of all nonsyndromic patients undergoing primary modified Furlow palatoplasty at The Children's Hospital of Philadelphia between 1980 and 2011. Surgical success was evaluated by the rate of postoperative oronasal fistula, speech outcomes assessed at a minimum of age 5 years, and the need for secondary surgery for velopharyngeal dysfunction. Results were analyzed by surgeon, Veau cleft type, age at repair, and over time. RESULTS: Eight hundred sixty-nine patients who underwent modified Furlow repair were included in this study. The mean age at palatoplasty was 1.1 years, and the mean length of follow-up was 6.7 years. Repairs were performed by 11 surgeons, with 44.3 percent performed by a single surgeon. The overall oronasal fistula rate was 5.2 percent, and this improved significantly over the 30-year period (p = 0.003). Speech results at age 5 or older were available for 559 patients. Of these 559 patients, 72.4 percent had a competent velopharyngeal mechanism, and 21.5 percent had a borderline mechanism consistent with socially acceptable speech. Secondary pharyngeal surgery was indicated in 8.1 percent of patients. Speech results were stable (p = 0.10), and rates of secondary surgery for velopharyngeal dysfunction declined significantly over the study period (p = 0.005). CONCLUSIONS: The authors' 30-year experience with the modified Furlow palatoplasty shows stable speech results, decreasing rates of secondary surgery, and an acceptable rate of postoperative oronasal fistula.

Perioperative risk factors for adverse airway events in patients undergoing cleft palate repair.

Objective : To establish the incidence of perioperative airway complications in a large series of pediatric patients undergoing palatoplasty and to identify which specific patient, procedural, and provider factors are associated with increased risk for perioperative adverse airway events (AAEs). Design : Retrospective chart review. Setting : Tertiary pediatric hospital. Patients : Included were 300 patients who underwent primary cleft palate repair using the modified Furlow technique between 2008 and 2011. Patients were 2 years or younger at the time of the operation. Main Outcome Measure(s) : Charts were reviewed for perioperative AAEs, which were defined as postoperative airway obstruction, oxyhemoglobin saturation ≤85% for ≥45 seconds, bronchospasm, laryngospasm, reintubation, and unplanned admission to the intensive care unit. Patient-specific factors (diagnosis of a craniofacial syndrome, Veau cleft type, preoperative pulmonary and airway history), procedural factors (operative time, anesthesia time, opioid dose, administration and reversal of neuromuscular blockers), and provider factors (experience, number of providers), were documented, and associations with AAEs were investigated. Results : AAEs occurred in 23% of patients overall and were significantly more common in syndromic patients (P = .003), patients with jaw or tracheal anomalies (P = .001), and patients with a history of difficult airway (P = .001). Other significant factors included prior history of difficult intubation (P = .05), surgeon (P = .02) and anesthesiologist experience (P = .05), and operative time (P = .02). Conclusions : Diagnosis of a craniofacial syndrome, a history of preoperative airway problems, and provider inexperience correlated with increased risk for airway complications after palatoplasty. Recognizing patients at risk for AAEs may permit improved preoperative planning to optimize surgical outcomes and minimize complications.

Conjoined twin separation: lessons learned.

BACKGROUND: The separation of conjoined twins presents a unique challenge to many pediatric surgical specialties. A multidisciplinary approach is essential for successful twin separation, and plastic surgeons are critical members of this surgical team when soft-tissue requirements preclude simple closure. METHODS: In this article, the authors review multiple techniques available to plastic surgeons for providing adequate soft-tissue coverage in this unique clinical situation and present lessons learned from their experience at The Children's Hospital of Philadelphia with seven conjoined twin separations performed between the years 1980 and 2007. RESULTS: Successful separation was accomplished in six of the seven conjoined twin pairs, expander complications requiring reoperation occurred in three twin pairs, and skin grafting was required because of insufficient expansion in two twin pairs. The techniques found to be critical to successful separation include maximal extracavitary tissue expansion with smooth-wall remote port tissue expanders, protection of expanded skin through pressure-reducing strategies, fluorescein vascular territory mapping, the use of frozen banked autogenous skin for salvage, and the use of supplemental nutritional support. CONCLUSIONS: The overall success of conjoined twin separation depends on the experience and preparedness of the treating team and the resources available at the pediatric specialty center. Multiple techniques are available to plastic surgeons to optimize success in this challenging clinical situation. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Two-year results of a prospective, multi-site investigation of patient satisfaction and psychosocial status following cosmetic surgery.

BACKGROUND: The number of cosmetic surgical and nonsurgical procedures performed in the United States has increased by 500% over the past 10 years. Most studies of psychosocial functioning following aesthetic procedures have reported high levels of patient satisfaction and improved functioning; however, nearly all these studies focused only on changes during the first posttreatment year. OBJECTIVE: This paper reports on the 2-year results of a prospective, multi-site investigation of postoperative satisfaction and changes in psychosocial status following cosmetic surgery. METHODS: One hundred patients from 8 surgical practices completed psychometric measures of body image, depressive symptoms, and self-esteem before surgery. Patients completed the same measures again at 3, 6, 12, and 24 months postoperatively. In addition, they reported their postoperative satisfaction as well as self-rated attractiveness at the 4 postoperative assessment points. RESULTS: Patients reported improvements in their overall appearance and body image, the appearance of and their degree of dissatisfaction with the feature altered by surgery, and the frequency of negative body image emotions in specific social situations through 24 months after surgery. These improvements were first evident at 3 months postoperatively and were maintained, without deterioration, through 2 years following surgery. CONCLUSIONS: Patients reported high rates of satisfaction and improvements in body image within the first 3 months of cosmetic surgery. These improvements were well maintained through the first 2 postoperative years.

Midface growth in patients with ectrodactyly-ectodermal dysplasia-clefting syndrome.

BACKGROUND: Ectrodactyly-ectodermal dysplasia-clefting syndrome is an autosomal dominant disorder involving abnormalities of the hands, feet, skin, and teeth and clefts of the lip and palate. Patients with this syndrome have abnormal facial features--namely, clefts of the lip and palate--and abnormal dentition, which could affect the growth of their midface region. The purpose of this study was to describe characteristics of midface growth in patients with this syndrome. METHODS: A retrospective chart review was conducted for eight postoperative patients seen at the cleft clinic at The Children's Hospital of Philadelphia between 1987 and 2004. Clinical notes and operative reports were reviewed. The surgeon's assessment and cephalometric measurements were analyzed. The cephalometric values were compared with Bolton standards for normal facial growth to determine deficiency. RESULTS: All eight patients displayed midface disproportion. Five of the eight were found to have some degree of midface retrusion. The upper anterior facial height was found to be deficient for half of the patients. Six patients had deficient lower facial height and four had deficient total face height. Two of these patients have undergone midface distraction osteogenesis. CONCLUSIONS: The authors' study identified a subset of patients within the cleft clinic who carry the diagnosis of ectrodactyly-ectodermal dysplasia-clefting syndrome. The patients tend to have poor dentition that, coupled with clefts of the lip and palate, inhibits normal midface growth and can require surgical correction. The need for orthognathic surgery seems to be greater than that seen in patients who have clefts of the lip and palate but do not carry the syndrome.

Repair of oronasal fistulae with acellular dermal matrices.

BACKGROUND: The authors examined the efficacy of a novel technique for oronasal fistula repair using acellular dermal matrix grafts. In part I, an animal model was used to demonstrate proof-of-concept; in part II, the method was applied to oronasal fistula repair in the clinical setting. METHODS: In part I, oronasal fistulas were created in Yorkshire piglets (n = 6) and allowed to mature for 2 weeks. In three animals, acellular dermal grafts were interposed between the oral and nasal mucosa traversing the palatal fistulas. Mucosal edges were not closed. Three weeks postoperatively, the palates were examined histologically. The fistulas of control piglets (n = 3) remained unrepaired and were examined 5 weeks after their creation. In part II, acellular dermal grafts were interposed between the oral and nasal mucosa in nine consecutive patients undergoing oronasal fistula repair. Complete closure of the oral and nasal mucosa was achieved in two patients. In the remainder, nasal closure was affected by interposition of the dermal matrices beneath a complete oral mucosal closure. RESULTS: All animals that underwent fistula repair demonstrated successful healing with revascularization, complete reepithelialization, and cellular infiltration into the grafts. All control fistulas remained patent. Successful fistula closure was observed in all patients. In two patients, early oral mucosal dehiscence and exposure of the dermal graft was followed by complete healing. CONCLUSIONS: This study demonstrates successful closure of palatal fistulas in an animal model and in cleft palate patients using interposition grafts of acellular dermis. This novel method offers promise as a simple and effective technique for tension-free closure of oronasal fistulas.

Evaluation of potential modifiers of the palatal phenotype in the 22q11.2 deletion syndrome.

OBJECTIVE: To evaluate potential modifiers of the palatal phenotype in individuals with the 22q11.2 deletion syndrome. DESIGN: Data from 356 subjects enrolled in a study of the 22q11.2 deletion syndrome were used to evaluate potential modifiers of the palatal phenotype. Specifically, subjects with and without velopharyngeal inadequacy and/or structural malformations of the palate were compared with respect to gender, race, and genotype for variants of seven genes that may influence palatal development. METHODS: The chi-square test or Fisher exact test was used to evaluate the association between palatal phenotype and each potential modifier. Odds ratios and their associated 95% confidence intervals were used to measure the magnitude of the association between palatal phenotype, subject gender and race, and each of the bi-allelic variants. RESULTS: The palatal phenotype observed in individuals with the 22q11.2 deletion syndrome was significantly associated with both gender and race. In addition, there was tentative evidence that the palatal phenotype may be influenced by variation within the gene that encodes methionine synthase. CONCLUSIONS: Variation in the palatal phenotype observed between individuals with the 22q11.2 deletion syndrome may be related to personal characteristics such as gender and race as well as variation within genes that reside outside of the 22q11.2 region.

Velopharyngeal anatomy in 22q11.2 deletion syndrome: a three-dimensional cephalometric analysis.

OBJECTIVE: 22q11.2 deletion syndrome is the most common genetic cause of velopharyngeal dysfunction (VPD). Magnetic resonance imaging (MRI) is a promising method for noninvasive, three-dimensional (3D) assessment of velopharyngeal (VP) anatomy. The purpose of this study was to assess VP structure in patients with 22q11.2 deletion syndrome by using 3D MRI analysis. DESIGN: This was a retrospective analysis of magnetic resonance images obtained in patients with VPD associated with a 22q11.2 deletion compared with a normal control group. SETTING: This study was conducted at The Children's Hospital of Philadelphia, a pediatric tertiary care center. PATIENTS, PARTICIPANTS: The study group consisted of 5 children between the ages of 2.9 and 7.9 years, with 22q11.2 deletion syndrome confirmed by fluorescence in situ hybridization analysis. All had VPD confirmed by nasendoscopy or videofluoroscopy. The control population consisted of 123 unaffected patients who underwent MRI for reasons other than VP assessment. INTERVENTIONS: Axial and sagittal T1- and T2-weighted magnetic resonance images with 3-mm slice thickness were obtained from the orbit to the larynx in all patients by using a 1.5T Siemens Visions system. OUTCOME MEASURES: Linear, angular, and volumetric measurements of VP structures were obtained from the magnetic resonance images with VIDA image-processing software. RESULTS: The study group demonstrated greater anterior and posterior cranial base and atlanto-dental angles. They also demonstrated greater pharyngeal cavity volume and width and lesser tonsillar and adenoid volumes. CONCLUSION: Patients with a 22q11.2 deletion demonstrate significant alterations in VP anatomy that may contribute to VPD.

Craniosynostosis: another feature of the 22q11.2 deletion syndrome.

We report on the presence of craniosynostosis in four patients with the 22q11.2 deletion. In light of previous reports of the association, we propose that the occurrence is higher than the general population incidence. Therefore, we suggest that craniosynostosis should be considered a manifestation of the 22q11.2 deletion and conversely that the 22q11.2 deletion should be considered in the differential diagnosis of craniosynostosis.

The 22q11.2 deletion in African-American patients: an underdiagnosed population?

Findings associated with the 22q11.2 deletion often include congenital heart malformations, palatal anomalies, immunodeficiency, hypocalcemia, and developmental delay or learning disabilities. Often the clinical suspicion of the diagnosis in a patient with one or more of these findings is heightened based on the presence of a characteristic facial appearance. In our large cohort of 370 patients with the 22q11.2 deletion, we report the under-representation of African-Americans in our group, as well as, the paucity of craniofacial dysmorphism in these patients. We note that the absence of the typical facial features may result in decreased ascertainment in this population and, furthermore, may delay the implementation of palliative care, cognitive remediation, and recurrence risk counseling. We, therefore, suggest that the clinician's threshold of suspicion should be lower in African-American patients.

A prospective, multi-site investigation of patient satisfaction and psychosocial status following cosmetic surgery.

BACKGROUND: Although several studies have investigated patient satisfaction and changes in body image following aesthetic plastic surgery, few have investigated more specific dimensions of body image, including dysphoric emotions that occur in specific social situations or body image quality of life. In addition, few studies have investigated changes in body image that may occur in concert with changes in more general areas of psychosocial functioning, such as depressive symptoms and self-esteem. OBJECTIVE: This prospective, multi-site study investigated postoperative satisfaction and changes in psychosocial status following cosmetic surgery. METHODS: One hundred patients recruited from 8 geographically diverse surgical practices completed psychometric measures of body image, depressive symptoms, and self-esteem prior to surgery. Seventy-two patients completed the 3-month postoperative assessment, 67 completed the 6-month assessment, and 63 completed the 12-month assessment. All statistical tests on changes after surgery were conducted using the sample of 72 patients who completed the 3-month assessment. A Last Observation Carried Forward analysis was used to account for patients who did not complete the subsequent follow-up assessments. In addition, they reported their postoperative satisfaction as well as self-rated attractiveness at the 3 postoperative assessment points. RESULTS: Eighty-seven percent of patients reported satisfaction with their postoperative outcomes. Patients also reported significant improvements in their overall appearance, as well as the appearance of the feature altered by surgery, at each of the postoperative assessment points. Patients experienced significant improvements in their overall body image, their degree of dissatisfaction with the feature altered by surgery, and the frequency of negative body image emotions in specific social situations. All of these improvements were maintained 12 months after surgery. CONCLUSIONS: These results add to a growing body of literature documenting improvements in body image following cosmetic surgery.

Mental health histories and psychiatric medication usage among persons who sought cosmetic Surgery.

This retrospective chart review study examined the mental health histories and psychiatric medication usage of persons who sought cosmetic surgery. The medical records of 232 patients who had undergone a consultation for a cosmetic procedure were reviewed. The rates of self-reported psychiatric disorders and of psychiatric medication usage of these patients were compared with those of 200 patients who underwent noncosmetic procedures. Nineteen percent of cosmetic surgery patients reported a mental health history, significantly greater than the 4 percent of the noncosmetic patients who reported a psychiatric history. Eighteen percent of the cosmetic patients reported using a psychiatric medication at the time of their consultation, also significantly greater than the 5 percent of the noncosmetic patients who reported psychiatric medication usage. These results underscore the importance of collecting a psychiatric history from prospective cosmetic surgery patients. Recommendations for assessing patients' psychiatric history and status are provided.

The Children's Hospital of Philadelphia modification of the Furlow double-opposing z-palatoplasty: long-term speech and growth results.

Of the 261 nonsyndromic patients we studied, over 90% had minimal or absent hypernasality, almost 86% had inconsistent or no nasal emission, and 95% had no articulation errors related to velar function. The patients with a Pittsburgh score indicating an incompetent velopharyngeal mechanism comprised only about 6% of the group. Ninety-four percent had a socially functional speech quality. Secondary surgery was done in 6.5% of patients and was done or was recommended in about 8% of patients. Patients with isolated cleft palate seemed to do less well, although their outcomes were not statistically different from those with complete unilateral and bilateral clefts. Relaxing incisions have kept our fistula rate to an acceptably low rate of 6.8%. No major soft palate dehiscences or hard palate flap losses have occurred. The speech outcomes we are achieving are improved over our historical results and compared with published reports using nondouble reversing z-palatoplasty techniques. Similar outcomes with the Furlow repair have been confirmed. Maxillary growth, occlusion, and the need for orthognathic surgery do not seem to be influenced by the CHOP modification of the Furlow double-opposing z-palatoplasty. These modifications facilitate a tension free-closure and a low fistula rate.

Body image concerns of breast augmentation patients.

This study investigated the body image concerns of women who sought cosmetic breast augmentation. Thirty breast augmentation candidates completed several measures of body image before their initial surgical consultation. Thirty physically similar women who were not interested in breast augmentation were recruited from the medical center and university community and also completed the measures. Breast augmentation candidates, as compared with women not seeking augmentation, reported greater dissatisfaction with their breasts. Augmentation candidates rated their ideal breast size, as well as the breast size preferred by women, as significantly larger than did controls. In addition, women interested in breast augmentation reported greater investment in their appearance, greater distress about their appearance in a variety of situations, and more frequent teasing about their appearance. Finally, breast augmentation candidates also reported more frequent use of psychotherapy in the year before the operation as compared with women not seeking augmentation. These results replicate and extend previous studies of body image in cosmetic surgery patients.

Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?

OBJECTIVE: The purpose of this study was to examine the efficacy of tongue-lip adhesion (TLA) in the management of clinically significant airway obstruction associated with Pierre Robin sequence. DESIGN: The records of all children admitted to The Children's Hospital of Philadelphia with a diagnosis of Pierre Robin sequence were reviewed. Charts were reviewed for birth data, diagnosis, preoperative airway management methods, and surgical intervention. Records of infants undergoing TLA were analyzed for timing of surgery, operative technique, postoperative complications, length of hospital stay, and treatment outcome. RESULTS: Over the 28-year period 1971 to 1999, 107 patients (47 boys, 60 girls) meeting the criteria for Pierre Robin sequence were admitted for treatment. Of these, 74 (69.2%) were successfully managed by positioning alone. Surgical management of the airway was performed in the remaining 33 (30.8%) patients, 29 of whom underwent TLA and 4 of whom underwent tracheostomy. Dehiscence of the adhesion occurred in five patients (17.2%), two of whom subsequently required tracheostomy. Within the group of patients who underwent mucosal adhesion alone, the dehiscence rate was 41.6%. When the adhesion included muscular sutures, however, dehiscence was not observed in any patient. Of the 24 patients in whom primary TLA healed uneventfully, airway obstruction was successfully relieved in 20 (83.3%). Failure of a healed TLA to relieve the airway obstruction resulted in conversion to a tracheostomy in four patients. Six patients who underwent TLA (20.7%) ultimately required a tracheostomy; five of these patients (83.3%) were syndromic. Of patients requiring preoperative intubation, 42.9% ultimately required tracheostomy. CONCLUSION: TLA successfully relieves airway obstruction that is unresponsive to positioning alone in the majority of patients with Pierre Robin sequence and should therefore play an important role in the management of these infants.