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

Costa, Melinda A; Tu, Michael M; Murage, Kariuki P; Tholpady, Sunil S; Engle, William A; Flores, Roberto L
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.
PMID: 25357033
ISSN: 1529-4242
CID: 1344062

Measuring surgical competency in facial trauma: the arch bar placement assessment scale

Flores, Roberto L; Havlik, Robert J; Choi, Matthew; Heidelman, Joseph F; Bennett, Jeffrey D; Tholpady, Sunil
BACKGROUND: Surgical education is in a period of significant change. Assessment of surgical competency is imprecise compared with cognitive knowledge and judgment. A surgical competency measurement tool may be useful for plastic surgery training programs and certification societies. We present a validation study of a novel measurement instrument for arch bar placement and dental wire handling. METHODS: An Arch Bar Placement Assessment Scale (ABPAS) was created via consensus by 2 craniofacial and 2 maxillofacial surgeons. Residents and faculty members of plastic and maxillofacial surgery (n = 20) then placed an arch bar on the lower jaw of a skull model. Performances were video recorded without revealing identities. Two study groups were created based on subjects experience level: group 1 (n = 10) previously placed fewer than 25 arch bars; group 2 (n = 10) previously placed more than 25 arch bars. Two craniofacial surgeons used the ABPAS to blindly grade surgical performance. RESULTS: The ABPAS consisted of a 48-point rating scale that included a 23-point task-specific work list and a 25-point global rating scale. Pearson coefficient showed limited intraobserver (P = 0.97) and interobserver (P = 0.95) variance of test scores. The ABPAS demonstrated superior performance in group 2 in the task-specific work list [12.6 (5.5) vs 17.6 (1.5), P = 0.02], global rating scale [17.4 (4.4) vs 22 (2.1), P = 0.01], and ABPAS score [30 (9.8) and 39.6 (3.2), P = 0.01]. CONCLUSIONS: The ABPAS is a novel measurement tool which assesses technical surgical skill and can identify surgical competency in arch bar placement and dental wire handling. This tool may have future use in residency training and continuing education.
PMID: 23759961
ISSN: 0148-7043
CID: 1130002

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

Tholpady, Sunil S; DeMoss, Patrick; Murage, Kariuki P; Havlik, Robert J; Flores, Roberto L
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.
PMID: 23932740
ISSN: 1010-5182
CID: 1130012

The surgical correction of Pierre Robin sequence: mandibular distraction osteogenesis versus tongue-lip adhesion

Flores, Roberto L; Tholpady, Sunil S; Sati, Shawkat; Fairbanks, Grant; Socas, Juan; Choi, Matthew; Havlik, Robert J
BACKGROUND: The authors present an outcomes analysis of mandibular distraction osteogenesis versus tongue-lip adhesion in the surgical treatment of Pierre Robin sequence. METHODS: A retrospective, 15-year, single-surgeon review was undertaken of all nonsyndromic neonates with Pierre Robin sequence treated with mandibular distraction osteogenesis (2004 to 2009; n = 24) or tongue-lip adhesion (1994 to 2004; n = 15). Outcomes included time of extubation, length of intensive care unit stay, incidence of tracheostomy, and surgical complications. Polysomnography data were collected 1 month and 1 year postoperatively. Sleep study data included changes in oxygen saturation and apnea-hypopnea index. RESULTS: There were no postprocedure tracheostomies in the mandibular distraction osteogenesis group and four tracheostomies in the tongue-lip adhesion group. The preoperative oxygen saturations were significantly lower in the mandibular distraction osteogenesis group compared with tongue-lip adhesion (76.5 percent versus 82 percent; p < 0.05). Preoperative apnea-hypopnea index was significantly higher in the mandibular distraction osteogenesis group compared with the tongue-lip adhesion group (47 versus 37.6; p < 0.05). Despite these preoperative differences, patients undergoing mandibular distraction osteogenesis demonstrated significantly higher oxygen saturation levels at 1 month (98.3 percent versus 87.5 percent; p < 0.05) and 1 year postoperatively (98.5 percent versus 89.2 percent; p < 0.05) and lower apnea-hypopnea index at 1 month (10.9 versus 21.6; p < 0.05) and 1 year postoperatively (2.5 versus 22.1; p < 0.05) compared with tongue-lip adhesion. Surgical complications were comparable between the two groups. CONCLUSIONS: In nonsyndromic patients with Pierre Robin sequence, mandibular distraction osteogenesis demonstrates superior outcome measures regarding oxygen saturation, apnea-hypopnea index, and incidence of tracheostomy compared with tongue-lip adhesion. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.
PMID: 24569425
ISSN: 1529-4242
CID: 1130032

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

Murage, Kariuki P; Costa, Melinda A; Friel, Michael T; Havlik, Robert J; Tholpady, Sunil S; Flores, Roberto L
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.
PMID: 24531254
ISSN: 1049-2275
CID: 1130042

Low-cost, high-definition video documentation of corrective cleft surgeries using a fixed laparoscope [Letter]

Demoss, Patrick; Murage, Kariuki P; Tholpady, Sunil; Friel, Michael; Havlik, Robert J; Flores, Roberto L
PMID: 24090725
ISSN: 1748-6815
CID: 1130062

Repair of a pediatric bilateral condylar and symphyseal fracture using a transfacial Steinman pin

Grow, Jacob N; Flores, Roberto L; Tholpady, Sunil S
The proper management of complex pediatric mandibular fractures remains a topic of debate because of the relatively uncommon presentation of these fractures, combined with concerns related with deciduous teeth, tooth buds, and growth inhibition. In this current study, we present a novel approach to the repair of bilateral condylar fractures with concomitant symphyseal fracture in a 4-year-old girl. Manual closed reduction was first obtained, followed by placement of a transfacial Steinman pin through the angles of the mandible and placement of circummandibular wires. Maxillomandibular fixation remained for 2 weeks and the Steinman pin was removed after 11 weeks. At 3 months of postsurgical follow-up, the patient displayed class I occlusion, facial symmetry, full range of jaw motion, and absence of deviation or pain on full oral excursion. Radiographic findings also revealed complete resolution of the symphyseal fracture with appropriate condylar healing and alignment.
PMID: 24448533
ISSN: 1049-2275
CID: 1130052

Incidence of concomitant airway anomalies when using the university of California, Los Angeles, protocol for neonatal mandibular distraction [Letter]

Flores, Roberto L; Murage, Kariuki; Tholpady, Sunil S
PMID: 24281617
ISSN: 1529-4242
CID: 1130072

The cost of intraoperative plastic surgery education

Sasor, Sarah E; Flores, Roberto L; Wooden, William A; Tholpady, Sunil
PURPOSE: Within the surgical community, it is commonly accepted that the length and cost of a surgical case increase when a resident physician participates. Many accountable care organizations, however, believe the opposite, that is, resident assistance enhances efficiency and diminishes operative time. The purpose of this study is to determine the opportunity cost to the attending surgeon for intraoperative teaching during index plastic surgery cases. METHODS: A single senior surgeon's experience over a 7-year period was evaluated retrospectively for Current Procedural Terminology codes 40700 (repair of primary, unilateral cleft lip) and 42200 (palatoplasty). Variables collected include operative time, the presence or absence of a physician learner, and postgraduate year level. Statistical analysis was performed with the Kruskal-Wallis test using the S+ programming language. A cost analysis was performed to quantify the effect of longer operative times in terms of relative value units (RVUs) lost. RESULTS: During the study period, a total of 45 patients had primary, unilateral cleft lip repair; 70 patients had cleft palate repair. Of those cases, 39 (87%) cleft lip repairs and 60 (86%) cleft palate repairs were performed with a resident or fellow present. There was a statistically significant difference in the amount of time required to perform either surgery with a physician learner than without, with operative times being 60% (p = 0.020) longer for cleft lip repair and 65% (p = 0.0016) longer for cleft palate repair. The results were further stratified based on level of training, with craniofacial fellows and plastic surgery residents (independent and integrated) compared separately. Cases where a craniofacial fellow was present required the longest operative times: 103% (p = 0.0012) longer for cleft lip repairs and 104% (p < 0.0001) longer for cleft palate repairs when compared with the senior surgeon operating alone. Using the 2011 physician work RVUs for these surgeries and the 2011 Medicare conversion factor for RVUs to dollars, the opportunity cost is over $275 per case per trainee for any physician learner. When craniofacial fellows are analyzed separately, over $440 is invested in intraoperative teaching per case per fellow. CONCLUSIONS: Resident involvement in the operating room is crucial to the education of independent surgeons. This involvement, however, comes at a significant opportunity cost to the attending surgeon. As an incentive to retain academic surgeons and uphold a quality academic environment in the OR, compensation should be offered for intraoperative teaching.
PMID: 24016378
ISSN: 1878-7452
CID: 1130092

Congenital lower lip pits (van der Woude syndrome): what pathologists need to know

Fan, Rong; Flores, Roberto L; Faught, Philip R; Lin, Jingmei
Congenital lower lip pits are cardinal findings of van der Woude syndrome [OMIM 119300]. The nosologic context of how lower lip pits are catalogued is easily lost because of insufficient clinical history, subtle findings misidentified as artifacts, lack of awareness by the pathologist, the perception that these are identify/confirm descriptive-diagnosis only, not necessarily an element of an actionable report, and/or the rarity with which these specimens are accessioned (in the authors' experience, less than 1 case per year). We present the salient findings on 19 lower lip pits specimens from the files of a single institution collected over the last 25 years.
PMID: 23947719
ISSN: 1093-5266
CID: 1130102