Airway compromise following palatoplasty in Robin sequence: improving safety and predictability.

BACKGROUND: Prior studies report a high incidence of airway complications in patients with Robin sequence following palatoplasty. The authors' institution uses polysomnography to assess risk of airway compromise before palatoplasty in Robin sequence. This study compares airway complications in Robin sequence to cleft palate only using this screening airway protocol and identifies risk factors for airway complications after palatoplasty. METHODS: A 12-year retrospective review of patients with Robin sequence undergoing palatoplasty was performed. Robin sequence patients were divided into nonoperative management and mandibular distraction osteogenesis subgroups. Preoperative variables including comorbidities were recorded. The primary outcome was postoperative airway complication, defined as reintubation, emergency room visit, or hospital admission within 3 months of palatoplasty. RESULTS: One hundred thirteen patients met inclusion criteria: polysomnography, 34.5 percent; Robin sequence, 65.5 percent; and Robin sequence treated with mandibular distraction osteogenesis, 30.1 percent. Screening polysomnography was used to indicate patients for palatoplasty or other airway interventions. The total airway complication rate was 7.1 percent; this was similar in Robin sequence (5.8 percent) and cleft palate only (7.7 percent). In isolated Robin sequence, the reintubation rate was 0 percent. Lower airway anomalies were associated with airway complications (p = 0.03). Significant variables for reintubation were cardiac (p = 0.046), gastrointestinal (p = 0.04), and lower airway anomalies (p = 0.025) and syndromic diagnosis (p = 0.05). CONCLUSION: Screening polysomnography can control airway complications following palatoplasty in Robin sequence patients to a rate that is comparable to that of patients with cleft palate only.

Robin sequence: mortality, causes of death, and clinical outcomes.

BACKGROUND: The authors report the cause of and risk factors for mortality in infants with Robin sequence and identify characteristics associated with quality-of-life outcomes. METHODS: The authors performed an 11-year retrospective review of all infants with Robin sequence treated at a neonatal intensive care unit. Patient characteristics were correlated to mortality and quality-of-life measures. Emergency room visits and hospital admissions were used to assess quality-of-life outcomes. Significant variables were identified by means of univariate analysis. RESULTS: One hundred eighty-one consecutive infants were identified. Patient characteristics included the following: isolated, 32.6 percent; syndromic, 31.5 percent; gastrointestinal, 38.1 percent; pulmonary, 32.6 percent; cardiac, 30.9 percent; central nervous system, 25.4 percent; and two or more organ system anomalies, 69.6 percent. Mortality was 16.6 percent; two deaths were related to airway obstruction problems. There were no deaths in isolated Robin sequence (p = 0.002). Mortality was statistically associated with cardiac anomalies (p < 0.001), central nervous system anomalies (p = 0.001), and two or more organ system abnormalities (p = 0.001). Variables associated with an increased rate of emergency room visits were cardiac anomalies (p = 0.04) and two or more organ system abnormalities (p = 0.04). The presence of two or more organ system abnormalities (p = 0.04) was associated with an increased hospital admission rate. CONCLUSIONS: Mortality and negative quality-of-life measures in Robin sequence are not directly related to respiratory obstruction. Isolated Robin sequence confers no increased risk of mortality. There is a high incidence of cardiac and central nervous system anomalies, which are significantly associated with mortality. Cardiac and cranial imaging should be performed during the initial evaluation of infants with Robin sequence. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Complications associated with neonatal mandibular distraction osteogenesis in the treatment of Robin sequence.

BACKGROUND: Robin sequence (RS) is defined as a triad of retrognathia, glossoptosis, and airway obstruction. Although several studies have reported on the efficacy of mandibular distraction, the risks associated with this operation remain unclear. An outcomes analysis focusing on complications is reported here. METHODS: A 7-year retrospective review of all patients with RS treated with mandibular distraction was performed. Recorded variables included associated medical comorbidities, improvement in apnea/hypopnea index, need for tracheostomy, repeat distraction, and complications. Complications associated with mandibular distraction were categorized as major, moderate, or minor. They included surgical site infection (SSI), device failure, temporomandibular joint ankylosis, facial nerve injury, hypertrophic scarring, self-extubation premature ossification, and fibrous nonunion. RESULTS: Fifty patients were identified. Four patients (8%) required tracheostomy following distraction, and 3 required repeat distraction. There were 0% major, 12% moderate, and 18% minor complications. Moderate complications were device failure (2%), SSI requiring return to the operating room (2%), and SSI requiring intravenous antibiotics (8%). Minor complications were SSI managed with oral antibiotics (12%), self-extubation (4%), and transient facial nerve palsy (2%). The most common complication was SSI (22%), of which 90.9% were successfully treated by antibiotics alone. There was a 0% rate of temporomandibular joint ankylosis. CONCLUSIONS: Mandibular distraction is a safe and effective treatment option for infants with RS and severe airway obstruction. The most common complication is infection; the majority of cases are successfully treated with antibiotics alone.

Epidemiology, demographics, and outcomes of craniomaxillofacial gunshot wounds in a level I trauma center.

BACKGROUND: Gunshot injuries to the craniomaxillofacial region are a challenge to the trauma and reconstructive surgeon. Although management of these injuries has been standardized and early rather than late intervention is advocated, the patient characteristics before, during, and after have been poorly elucidated. METHODS: A prospectively maintained Level I trauma center database was queried as to gunshot wounds of the craniomaxillofacial skeleton. Over a five-year period (2007-2011), 168 patients were identified with these injuries. Charts were reviewed as to demographics, presentations, and outcomes and these were tested for significant relationships with hospital length of stay, numbers and types of procedures, morbidity, and mortality. RESULTS: Gunshot wounds to the craniofacial skeleton resulted in 71 deaths in this patient population. Those that died were significantly older, presented with a lower GCS, had a shorter LOS, and a higher INR than those that lived. Subgroup analysis of mechanism demonstrated mortality was more likely to occur as a result of self-inflicted injury in whites and due to assault in the African-American population. CONCLUSIONS: Data gathered from this study disputes some commonly held beliefs regarding the epidemiology of gunshot injuries and should allow for better characterization of which outcomes are consistent with which presentations.

Outcomes analysis of mandibular distraction osteogenesis for the treatment of Pierre Robin sequence.

UNLABELLED: Mandibular distraction osteogenesis is an established technique used to treat infants with Pierre Robin sequence associated with severe airway obstruction. The authors present a 7-year retrospective review of all patients with Pierre Robin sequence treated with mandibular distraction osteogenesis. Recorded variables included improvements in apnea/hypopnea index and postintervention tracheostomy. Multiple preoperative variables were assessed for association with successful mandibular distraction osteogenesis or tracheostomy. Fifty patients were identified for this study. Four patients (8 percent) required tracheostomy after distraction. A Fisher's exact test demonstrated no statistical association of tracheostomy with prematurity, low birth weight, preoperative intubation, late intervention, genetic syndromes, cardiac abnormalities, pulmonary abnormalities, or gastrostomy tube. The absence of a cleft palate, gastroesophageal reflux disease, and need for Nissen fundoplication were associated with failure of distraction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Clinical framework to guide operative decision making in disconnected left pancreatic remnant (DLPR) following acute or chronic pancreatitis.

BACKGROUND: Disconnected left pancreatic remnant (DLPR) presents clinically as a pancreatic fistula, pseudocyst, or obstructive pancreatitis. Optimal operative treatment, either distal pancreatectomy (DP) or internal drainage (ID), remains unknown. This paper critically evaluates our operative experience in patients with DLPR. METHODS: A retrospective analysis of a consecutive case series from a single, high-volume institution was carried out. A total of 76 patients with radiographic-confirmed DLPR (computed tomography + endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography) who had operations between November 1995 and September 2008 were included. Pancreas preservation (the use of ID) was our default unless anatomic, physiologic, or technical factors precluded it. Follow-up to July 2009 was done (median follow-up, 22 months). Standard statistical methodology was used (P < .05 = statistical significance). RESULTS: The mean age of this cohort was 52 years (range, 18-85); 57% of the patients were male. A total of 59 (73%) had acute pancreatitis, whereas 17 (22%) had chronic pancreatitis. Presentation was pseudocyst in 53%, pancreatic fistula in 34%, and obstructive pancreatitis in 13%. Resection (DP) and drainage (ID) options were utilized equally for each clinical presentation as follows: pseudocyst, 60/40; pancreatic fistula, 50/50; or obstructive pancreatitis, 50/50. The strongest driver for DP (92%) was a small pancreatic remnant and splenic vein thrombosis. In contrast, large pancreatic remnants had ID 70% of the time. No differences in short- or long-term outcomes between DP or ID options were identified. CONCLUSION: Using anatomic, physiologic, and technical factors to guide operative choice in DLPR, we report a 74% success rate with DP and an 82% success rate with ID at a median follow-up of 22 months. A pancreatic remnant size >6 cm favored ID options over resection.