Transgender Mastectomy: Incidence of High-Risk Pathologic Findings and the Need for Postoperative Cancer Surveillance.

BACKGROUND: Chest masculinization surgeries are one of the most common gender-affirming procedures performed. There is a need for better understanding of the risk of breast cancer and postsurgical screening in female to male (FtM) individuals. This study aimed to evaluate the incidence of high-risk pathologic findings in FtM transgender patients undergoing gender-affirming chest reconstructive surgery. METHODS: Medical records were reviewed from all FtM patients undergoing gender-affirming chest reconstructive surgery from January 2010 to February 2021 by 3 plastic surgeons at the University of Pittsburgh Medical Center. Relative risk of malignant progression was used to stratify pathologic data. Subsequent management of atypical, in situ, and invasive pathology were recorded. RESULTS: A total of 318 patients were included in this study; the average age at surgery was 24.6 ± 8.1 years. Eighty-six patients (27%) had a family history of breast and/or ovarian cancer. Overall, 21 patients (6.6%) had some increased risk of breast cancer: 17 (5.3%) had proliferative lesions, mean age 38.2 ± 12.4 years; 2 had atypical ductal hyperplasia, ages 33.4 and 38.3 years; and 2 had invasive ductal carcinoma, ages 35.4 and 40.6 years. CONCLUSIONS: In this study, we found that 6.6% of FtM transgender patients undergoing top surgery had an elevated risk of breast cancer, with 1.2% of patients having a greater than 2 times risk of breast cancer. These results highlight the importance of appropriate preoperative screening as well as pathological analysis of surgical specimens to help guide clinical care. The authors advocate for a thorough breast cancer risk assessment before surgery for all patients, as well as using pathologic findings to guide postoperative cancer screening and follow-up.

Steroid Use in Palatoplasty: A Single Surgeon Comparative Retrospective Cohort Study.

BACKGROUND: Fistula rates in cleft palate repair vary by technique, surgeon, and institution. Although steroids are commonly used in airway surgery, many plastic surgeons are reluctant to use steroids because of concerns with wound healing. This study aims to assess outcomes and safety of steroid use in Furlow palatoplasty and determine its impact on fistula formation. METHODS: A retrospective cohort study was done of all cleft palate surgeries performed by a single surgeon between 2010 and 2014. Data reviewed included demographics, type of cleft, steroid use, length of surgery, length of stay, and fistula formation rate. RESULTS: One hundred thirty-five patients underwent palatoplasty, of which 101 received steroids and 34 did not. The mean age was 4.6 years. A total of 42.2% of patients underwent primary palatoplasty, 48.1% underwent submucous cleft palatoplasty, and 9.7% underwent conversion palatoplasty. The overall fistula rate was 1.5% and was comparable between the 2 groups (steroids = 2.0%, no steroids = 0.0%, P = 0.558), and all occurred in primary palatoplasty patients. The average length of stay in the hospital was shorter among patients receiving steroids (steroids = 2.0 days, no steroids = 2.5 days, P < 0.05). CONCLUSIONS: Steroid use in cleft palate surgery appears to be safe and likely not associated with impaired wound healing or increased fistula formation. It may also shorten length of hospitalization.

Timing of Nerve Recovery After Nerve Grafting in Obstetrical Brachial Plexus Palsy Patients With Isolated Upper Trunk Neuromas.

BACKGROUND: The timing of nerve recovery after nerve grafting in obstetrical brachial plexus palsy patients has not been well reported. One prior study reported a return to baseline function at 3 to 6 months postoperatively. However, there is a paucity of studies to corroborate this timing, and there have been no studies delineating the timeline to obtain clinically meaningful function. METHODS: OBPP patients with upper trunk neuromas-in-continuity who were treated with resection and sural nerve grafting at a single institution were studied. Time to return to baseline function was assessed by Active Movement Scale (AMS) scores preoperatively and postoperatively. Time to clinically meaningful function, defined as an AMS score of ≥6, was also assessed. RESULTS: Eleven patients with isolated upper trunk neuromas-in-continuity underwent excision and reversed sural nerve grafting. Three of 11 patients also underwent spinal accessory to suprascapular nerve transfers. Average age at surgery was 9.8 ± 1.9 months. One patient did not have follow-up data and was excluded. Average follow-up was 37.1 ± 16.8 months. Average return to baseline AMS score was approximately 4 to 8 months for shoulder abduction, shoulder flexion, shoulder external rotation, elbow flexion, and forearm supination. Clinically meaningful function was obtained in most patients between 9 and 15 months. The remaining patients who did not achieve clinically meaningful function had all obtained scores of 5, which reflects less than one half normal range of motion against gravity. CONCLUSIONS: Nerve recovery after surgical intervention in OBPP patients who undergo resection of an upper trunk neuroma-in-continuity and nerve grafting is more rapid than in adults but longer than previously reported in OBPP literature. This study provides an important data point in delineating the timeline of nerve recovery.

Musculoskeletal Pain Survey Outcomes in Cleft Surgeons and Orthodontists.

OBJECTIVE: Determine prevalence and characteristics of musculoskeletal pain and pathology in cleft providers. DESIGN: An IRB-exempt survey based on previously validated surveys was administered. Data collected included demographics, practice description, musculoskeletal pain history, formal diagnoses, and interventions. SETTING: Survey was sent to all cleft centers approved by the American Cleft Palate-Craniofacial Association worldwide. PATIENTS, PARTICIPANTS: All cleft surgeons and orthodontists at these centers met entry criteria. Eighty-three providers responded. Cleft center coordinators were unable to confirm the number of survey recipients. MAIN OUTCOME MEASURES: The hypothesis formulated prior to data collection was that prevalence would be comparable to general plastic surgeons and other at-risk health care providers. RESULTS: Average age of respondents was 49.8 ± 11.3 years; 33.9% of respondents were female. Average body mass index was 24.8 ± 3.5 kg/m2. Headaches were observed in 62.7% of surveyed respondents while musculoskeletal symptoms were reported in 89.8%. Of the 12 body parts addressed, most commonly affected were the neck (71.2%), shoulders (52.5%), and lower back (67.8%). Pain interfered with hobbies and home life in the majority of respondents (62.7%). Those who reported a formal diagnosis were more likely to undergo treatment including surgery (P < .01), medication (P = .03), and physical therapies (P < .01). CONCLUSIONS: Cleft surgeons and orthodontists experience a higher frequency of headaches compared to the general population, and musculoskeletal disorders are more prevalent than reported by general plastic surgeons. Pain interferes with hobbies and home life. Formal diagnosis leads to treatment. Preventative exercises and interventions are presented.

The Association Between Age at Palatoplasty and Speech and Language Outcomes in Children With Cleft Palate: An Observational Chart Review Study.

OBJECTIVE: To determine whether timing of palatoplasty (early, standard, or late) is associated with speech and language outcomes in children with cleft palate. DESIGN: Retrospective case series. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from 733 children born between 2005 and 2015 and treated at the Cleft Craniofacial Clinic of a tertiary children's hospital were retrospectively reviewed. Exclusion criteria were cleft repair at an outside hospital, intact secondary palate, absence of postpalatoplasty speech evaluation, syndromes, staged palatoplasty, and introduction to clinic after 12 months of age. Data from 232 children with cleft palate ± cleft lip were analyzed. INTERVENTIONS: Palatoplasty. MAIN OUTCOME MEASURES: Speech/language delays and disorders at 20 months and 5 years of age based on formal hospital or community-based testing or screening evaluation in the Cleft Craniofacial Clinic; additional speech surgery. RESULTS: Median age at palatoplasty was 12.6 months (range: 8.8-21.9 months). Age at palatoplasty was classified as early (<11 months, n = 28), standard (11-13 months, n = 158), or late (>13 months, n = 46). Late palatoplasty was associated with increased odds of speech/language delays and speech therapy at 20 months, and language delays at 5 years, compared with standard or early palatoplasty (P < .05 for all comparisons). However, speech sound production disorders, velopharyngeal incompetence, tube replacement, and hearing loss were not significantly associated with age at palatoplasty. CONCLUSIONS: Late palatoplasty may be associated with short- and long-term delays in speech/language development. Future studies with standardized surgical technique/timing and outcome measures are required to more definitively describe the impact of age at palatoplasty on speech/language development.

Treatment of the Prominent Ear: A Standardized Approach Without Intraoperative Measurements.

BACKGROUND: Candidates for otoplasty have variable anatomy that may result in an aesthetically suboptimal appearance. The authors describe an algorithm to obtain ideal aesthetic position of the ear using a standardized suturing only technique. METHODS: A retrospective review was conducted at a major children's hospital. Pre- and postoperative distances from the mastoid to the posterior lateral helical rim were measured at 3 consistent points (upper helix, mid helix, and lobule). Cosmetic outcomes were determined by visual analog cosmetic score (VACS), assigned by 3 independent reviewers. RESULTS: A total of 26 patients underwent otoplasty for prominent ear (average age 8.2 years). Duration of follow-up was an average of 32 weeks postoperatively. Pre- and postoperative VACSs were determined for all patients: overall-appearance, 25.8 versus 71.3; overall-ear appearance, 25.7 versus 70.0; shape, 24.4 versus 72.6; and projection, 23.7 versus 73.9 (P < 0.05 for all). There was no inter-rater difference between scores. There was greater symmetry between ears postoperatively (P < 0.05). Measurements were significantly improved pre- versus postoperatively: upper-helix 2.04 versus 1.20 cm, mid-helix 2.22 versus 1.18 cm, and lobule 1.85 versus 1.49 cm (P < 0.05.) Postoperative measurements are in accordance with established norms for ideal ear position (1.0-1.2 cm upper third of the ear). Two patients recurred, and 1 experienced a spitting suture (11.5%). CONCLUSION: Aesthetic ideal was established on the operating table based on the appearance of the ear. Postoperative measurements fell within aesthetic ideal for a normal ear, suggesting that the use of intraoperative measurements are not needed to obtain an aesthetically acceptable outcome.

Pediatric Dog Bite Prevention: Are We Barking Up the Wrong Tree or Just Not Barking Loud Enough?

OBJECTIVES: The objectives of this study were (1) to evaluate dog bite-related injuries and associated medical documentation and (2) to compare these results with a study of dog bites from the same institution 10 years prior. METHODS: Data were retrospectively collected from a pediatric emergency department from July 2007 to July 2011 for patients treated for dog bites. These data were then compared with data from the same institution from 10 years prior. RESULTS: A total of 1017 bite injuries were treated (average, 254.25 bites/year), which represents a 25% increase compared with 10 years prior. Comparing the 1997 and 2007 to 2011 cohorts, patient demographics, bite rate among children less than 5 years old, rate of dog breed documentation, and setting of injury were similar. Dog breed was reported in 47% (95% confidence interval [CI], 40.2-53.9) and 41% (95% CI, 38.0-44.0) of cases, respectively, in the 2 cohorts. Bites to the craniofacial region were most common (face only reported for 1997: 43.2%; 95% CI, 36.4-50 versus 2007-2011: 66.1%; 95% CI, 63.2-69.0). In both cohorts, the child's home was the most frequent setting, accounting for 43% of bites (1997: 95% CI, 30.2-55.9 and 2007-2011: 95% CI, 39.3-46.7). CONCLUSIONS: Pediatric dog bites continue to occur frequently, and the associated factors did not change over the 10-year period: young age of child, bites to the craniofacial region, and dogs familiar to the child. Although accurate medical documentation of dog bites is a prerequisite to develop effective prevention strategies, current medical documentation of dog bites may be misguided.

Description of Mandibular Improvements in a Series of Infants With Congenital Muscular Torticollis and Deformational Plagiocephaly Treated With Physical Therapy.

BACKGROUND: Many infants with congenital muscular torticollis (CMT) have deformational plagiocephaly (DP), and a small cohort also demonstrate mandibular asymmetry (MA). The aim of this retrospective study was to evaluate mandibular changes in these infants with previous computed tomography (CT) scans who underwent physical therapy (PT) to treat CMT. METHODS: A retrospective study included patients presenting to a pediatric plastic surgery clinic from December 2010 to June 2012 with CMT, DP, and MA. A small subset of these patients initially received a 3D CT scan due to concern for craniosynostosis. An even smaller subset of these patients subsequently received a second 3D CT scan to evaluate for late-onset craniosynostosis. Patients were treated with PT for at least 4 months for CMT. Initial CT scans were retrospectively compared to subsequent CT scans to determine ramal height asymmetry changes. Clinical documentation was reviewed for evidence of MA changes, CMT improvement, and duration of PT. RESULTS: Ten patients met inclusion criteria. Ramal height ratio (affected/unaffected) on initial CT was 0.87, which significantly improved on subsequent CT to 0.93 ( P < .05). None of the patients were diagnosed with craniosynostosis on initial CT. One patient was diagnosed with late-onset coronal craniosynostosis on subsequent CT. CONCLUSIONS: We identified a small cohort of infants with MA, CMT, and DP. These patients uniformly demonstrated decreased ramal height ipsilateral to the affected sternocleidomastoid muscle. Ramal asymmetry measured by ramal height ratios improved in all infants undergoing PT.

Simonart's Band: Its Effect on Cleft Classification and Recommendations for Standardized Nomenclature.

OBJECTIVE: Accurate classification of cleft lip plays an important role in communication, treatment planning, and comparison of outcomes across centers. Although there is reasonable consensus in defining cleft types, the presence of Simonart's band can make classification challenging. Our objective was to survey cleft care providers to determine what all consider to be Simonart's band, how its presence effects cleft lip classification, and to provide recommendations for standardized nomenclature. DESIGN: A multiple-choice survey was e-mailed to 1815 members of the American Cleft Palate-Craniofacial Association, assessing each respondent's definition of Simonart's band and its effect on cleft classification. Cleft classification was drawn from the ICD system diagnosis billing codes. Descriptive analysis was performed. RESULTS: Three hundred seventy-three providers completed the survey (20.5% response), the majority of whom were surgeons (61.5%); 87.1% agreed with the definition that a Simonart's band is "any soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges." However, only 41.8% felt that the presence of a Simonart's band rendered a cleft lip incomplete; 54.4% felt that an alveolar cleft was the defining difference between a complete and an incomplete cleft lip. When asked to define the child with a cleft involving the upper lip that extends into the naris but interrupted by a soft tissue bridge located only at the base of the nostril or more internally, without a cleft of the alveolar ridge and palate, 61.4% classified this as an incomplete cleft lip, 32.7% as a complete cleft lip, and 5.9% as an unspecified cleft lip. CONCLUSIONS: Responses revealed wide discrepancy in the classification of cleft phenotypes and in the interpretation of the significance of anatomical components in the classification of a cleft lip. We discuss the difficulty in aligning classification based on unclear definition of terms and variable anatomic parameters. We highlight this issue in the face of a need for comparability in clinical evidence-based practices. To ensure precision and uniformity in cleft classification, we recommend that use of the term "Simonart's band" be abandoned while incorporating a notation of the integrity of the nasal sill into the LAHSHAL system. We propose a uniform definition of incomplete versus complete cleft lip, wherein a cleft lip will be classified as complete in the presence or absence of narrow bands of tissue present at the base of the nasal sill or more internally.

PHACE syndrome is associated with intracranial cavernous malformations.

INTRODUCTION: PHACE syndrome is a neurocutaneous disorder involving large facial hemangiomas in association with posterior fossa abnormalities, cerebral arterial anomalies, cardiac defects, and eye abnormalities. A recent consensus statement has delineated criteria necessary for the diagnosis of PHACE syndrome. Extracutaneous manifestations of PHACE syndrome predominately affect the cerebrovascular system. To date, there are no reports of cerebral cavernous malformations (CCMs) in children with PHACE syndrome. METHODS: We reviewed the charts of children admitted to the Children''s Hospital of Pittsburgh who met criteria for PHACE syndrome, and evaluated neuroimaging for cerebrovascular abnormalities, including the finding of CCMs. RESULTS: Six children met criteria for PHACE syndrome at our institution over a 10-year period. All children were female. All children had cerebrovascular abnormalities sufficient to meet major criteria for diagnosis. Four children (66.7 %) were found incidentally to have CCMs; all lesions measured less than 5 mm at the time of diagnosis and were asymptomatic. CONCLUSION: At present, CCMs are not listed among the diagnostic criteria for PHACE syndrome, and they have not previously been reported in association with PHACE syndrome. Hypoxic injury in utero may be the common denominator in the pathogenesis of many of the abnormalities already accepted in the criteria for PHACE syndrome and the formation of CCMs. In the setting of PHACE syndrome, we encourage clinicians to evaluate children for CCMs, which are readily apparent on the already-recommended screening MRIs.

Speech Outcomes After Clinically Indicated Posterior Pharyngeal Flap Takedown.

BACKGROUND: Velopharyngeal insufficiency affects as many as one in three patients after cleft palate repair. Correction using a posterior pharyngeal flap (PPF) has been shown to improve clinical speech symptomatology; however, PPFs can be complicated by hyponasality and obstructive sleep apnea. The goal of this study was to assess if speech outcomes revert after clinically indicated PPF takedown. METHODS: The cleft-craniofacial database of the Children's Hospital of Pittsburgh at the University of Pittsburgh Medical Center was retrospectively queried to identify patients with a diagnosis of velopharyngeal insufficiency treated with PPF who ultimately required takedown. Using the Pittsburgh Weighted Speech Score (PWSS), preoperative scores were compared to those after PPF takedown. Outcomes after 2 different methods of PPF takedown (PPF takedown alone or PPF takedown with conversion to Furlow palatoplasty) were stratified and cross-compared. RESULTS: A total of 64 patients underwent takedown of their PPF. Of these, 18 patients underwent PPF takedown alone, and 46 patients underwent PPF takedown with conversion to Furlow Palatoplasty. Patients averaged 12.43 (range, 3.0-22.0)(SD: 3.93) years of age at the time of PPF takedown, and 58% were men. Demographics between groups were not statistically different. The mean duration of follow-up after surgery was 38.09 (range, 1-104) (SD, 27.81) months. For patients undergoing PPF takedown alone, the mean preoperative and postoperative PWSS was 3.83 (range, 0.0-23.0) (SD, 6.13) and 4.11 (range, 0.0-23.0) (SD, 5.31), respectively (P = 0.89). The mean change in PWSS was 0.28 (range, -9.0 to 7.0) (SD, 4.3). For patients undergoing takedown of PPF with conversion to Furlow palatoplasty, the mean preoperative and postoperative PWSS was 6.37 (range, 0-26) (SD, 6.70) and 3.11 (range, 0.0-27.0) (SD, 4.14), respectively (P < 0.01). The mean change in PWSS was -3.26 (range, -23.0 to 4.0) (SD, 4.3). For all patients, the mean preoperative PWSS was 5.66 (range, 0.0-26) (SD, 6.60) and 3.39 (range, 0.0-27) (SD, 4.48), respectively (P < 0.05). The mean change in PWSS was -2.26 (range, -23.0 to 7) (SD, 5.7). There was no statistically significant regression in PWSS for either surgical intervention. Two patients in the PPF takedown alone cohort demonstrated deterioration in PWSS that warranted delayed conversion to Furlow palatoplasty. Approximately 90% of patients, who undergo clinically indicated PPF takedown alone, without conversion to Furlow Palatoplasty, will show no clinically significant reduction in speech. CONCLUSIONS: Although there is concern that PPF takedown may degrade speech, this study finds that surgical takedown of PPF, when clinically indicated, does not result in a clinically significant regression of speech.

Treatment for Infantile Hemangiomas: Selection Criteria, Safety, and Outcomes Using Oral Propranolol During the Early Phase of Propranolol Use for Hemangiomas.

OBJECTIVE: Despite the increasing popularity of propranolol for treatment of infantile hemangioma (IH), there is need for further evidence of efficacy and safety. This study is a retrospective review of one institution's experience treating IH with propranolol using a standard protocol. METHODS: Between 2009 and 2014, patients with IH were evaluated for treatment with propranolol. Exclusion criteria included a history of hypoglycemia, respiratory disorders, and cardiovascular disorders. Propranolol, 2 mg/kg/d, was initiated during 48-hour inpatient stay. Weight and complications were monitored. Appearance was assessed by Visual Analog Cosmetic Scale (VACS) via serial photography. RESULTS: Twenty-three patients were treated with propranolol. Average age at initiation of therapy was 14.9 weeks. Twenty-two lesions were on the head and neck, and 1 was on the trunk. Average treatment duration was 54.3 weeks (range 24-148 wk). Treatment was confirmed to be complete in 23 patients at the time of review (91.3%). Two patients were lost to follow-up. Posttreatment color, size, and VACS improved significantly (P < 0.05). There was no significant difference between first and most recent weight. Two patients experienced hypoglycemia, 1 during a diarrheal illness and 1 during inpatient treatment initiation. CONCLUSION: The authors present a series of patients with IH safely treated with 2 mg/kg/d of propranolol. Using a strict protocol, few complications were observed. Patients achieved significant reduction in size and improvement of the overall appearance of IH.

A comparison of speech outcomes using radical intravelar veloplasty or furlow palatoplasty for the treatment of velopharyngeal insufficiency associated with occult submucous cleft palate.

BACKGROUND: The safety, efficacy, and direct comparison of various surgical treatments for velopharyngeal insufficiency (VPI) associated with occult submucous cleft palate (OSMCP) are poorly characterized. The aim of this study was to report and analyze the safety and efficacy of Furlow palatoplasty (FP) versus radical intravelar veloplasty (IVV) for treatment of VPI associated with OSMCP. METHODS: A retrospective review of one institution's experience treating VPI associated with OSMCP using IVV (group 1) or FP (group 2) during 24 months was performed. Statistical significance was determined by Wilcoxon matched-pair, Independent-Samples Mann-Whitney U, and analysis of variance (SPSS 20.0.0). RESULTS: In group 1 (IVV), 18 patients were identified from August 2010 to 2011 (12 male and 6 female patients; average age, 5.39 years). Seven patients were syndromic and 11 were nonsyndromic. In group 2 (FP), 17 patients were identified from August 2009 to 2011 (8 male and 9 female patients; average age, 8.37 years). Three patients were syndromic and 14 patients were nonsyndromic. There was statistical significance between the average pretreatment Pittsburgh Weighted Speech Score (PWSS) of the 2 groups (group 1 and 2 averages 19.06 and 11.05, respectively, P=0.002), but there was no statistical significance postoperatively (group 1 and 2 averages 4.50 and 4.69, respectively, P=0.405). One patient from each group required secondary speech surgery. Average operative time was greater for FP (140 minutes; range, 93-177 minutes) compared to IVV (95 minutes; range, 58-135 minutes), P<0.001. Average hospital stay was 3.9 days for IVV (range, 2-9 days) and 3.2 days for FP (range, 2-6 days), with no significant difference (P=0.116). There were no postsurgical wound infections, oral-nasal fistulas, postoperative bleeding complications, or mortalities. CONCLUSIONS: Nonsyndromic patients with hypernasal speech are treated effectively and safely with either IVV or FP. Intravelar veloplasty trended toward lower speech scores than FP (76% IVV, 58% FP PWSS absolute reduction). Syndromic patients with OSMCP may be more effectively treated with FP (72% IVV vs 79% FP PWSS absolute reduction). Intravelar veloplasty is associated with shorter operative times. Both techniques are associated with low morbidity, improved speech scores, and low reoperative rates.

Neurofibromas with imaging characteristics resembling vascular anomalies.

OBJECTIVE: Although neurofibromas are rare, their initial clinical and imaging presentation can mimic those of vascular anomalies, particularly if the characteristic clinical features of neurofibromatosis are not present. The diagnostic challenges encountered in five cases of histologically proven neurofibromas, initially diagnosed as vascular anomalies, are reviewed and discussed. CONCLUSION: The clinical and imaging differences between neurofibromas and vascular anomalies are detailed with the histopathologic features to better understand why some neurofibromas are diagnosed as vascular anomalies.

Pierre Robin Sequence: a perioperative review.

The clinical triad of micrognathia (small mandible), glossoptosis (backward, downward displacement of the tongue), and airway obstruction defines the Pierre Robin sequence (PRS). Airway obstruction and respiratory distress are clinical hallmarks. Patients may present with stridor, retractions, and cyanosis. Severe obstruction results in feeding difficulty, reflux, and failure to thrive. Treatment options depend on the severity of airway obstruction and include prone positioning, nasopharyngeal airways, tongue lip adhesion, mandibular distraction osteogenesis, and tracheostomy. The neonate and infant with PRS require care from multiple specialists including anesthesiology, plastic surgery, otolaryngology, speech pathology, gastroenterology, radiology, and neonatology. The anesthesiologist involved in the care of patients with PRS will interface with a multidisciplinary team in a variety of clinical settings. This perioperative review is a collaborative effort from multiple specialties including anesthesiology, plastic surgery, otolaryngology, and speech pathology. We will discuss the background and clinical presentation of patients with PRS, as well as some of the controversies regarding their care.

Improving speech outcomes after failed palate repair: evaluating the safety and efficacy of conversion Furlow palatoplasty.

BACKGROUND: Velopharyngeal insufficiency occurs in a nontrivial number of cases following cleft palate repair. We hypothesize that a conversion Furlow palatoplasty allows for long-term correction of VPI resulting from a failed primary palate repair, obviating the need for pharyngoplasty and its attendant comorbidities. METHODS: A retrospective review of patients undergoing a conversion Furlow palatoplasty between 2003 and 2010 was performed. Patients were grouped according to the type of preceding palatal repair. Velopharyngeal insufficiency was assessed using Pittsburgh Weighted Speech Scale (PWSS). Scores were recorded and compared preoperatively and postoperatively at 3 sequential visits. RESULTS: Sixty-two patients met inclusion criteria and were grouped by preceding repair (straight-line repair (n = 37), straight-line repair with subsequent oronasal fistula (n = 14), or pharyngeal flap (n = 11). Median PWSS scores at individual visits were as follows: preoperative = 11, first postoperative = 3 (mean, 114.0 ± 6.7 days), second postoperative = 1 (mean, 529.0 ± 29.1 days), and most recent postoperative = 3 (mean, 1368.6 ± 76.9 days). There was a significant difference between preoperative and postoperative PWSS scores in the entire cohort (P < 0.001) with overall improvement, and post hoc analysis showed improvement between each postoperative visit (P < 0.05) with the exception of the second to the most recent visit. There were no differences between postoperative PWSS scores in the operative subgroupings (P > 0.05). Eight patients failed to improve and showed no differences in PWSS scores over time (P > 0.05). Patients with a PWSS score of 7 or greater (n = 8) at the first postoperative visit (0-6 months) displayed improvement at the most recent visit (P< 0.05). CONCLUSIONS: Conversion Furlow palatoplasty is an effective means for salvaging speech. Future studies should elucidate which factors predict the success of this technique following failed palate repair.

Exserohilum infection in an immunocompromised neonate.

Invasive fungal infections in children have increased in recent years, coinciding with greater survival of preterm neonates and children with immunodeficiencies, more intense chemotherapy regimens, and greater use of stem cell and solid organ transplantation. We describe a case of an immunosuppressed neonate who developed cutaneous Exserohilum rostratum infection.

Magnetic resonance imaging as a predictor of submucous cleft palate severity and guide for surgical intervention.

BACKGROUND: Diagnosis of submucous cleft palate (SMCP) is frequently delayed, adversely affecting speech outcomes. Previous studies show that MRI reliably identifies structural abnormalities in velopharyngeal musculature. This information has potential to assist with diagnosis and treatment decisions. AIMS: The objectives of this study were to (1) develop a clinician-friendly MRI grading scale of SMCP anatomy, (2) identify correlations between radiographic cleft severity and clinical severity using Pittsburgh Weighted Speech Scores (PWSS), and (3) determine if MRI is a predictor of surgical efficacy in improving PWSS. DESIGN: Thirty patients presenting to our Cleft Palate-Craniofacial Clinic for evaluation of velopharyngeal insufficiency (VPI) and suspected SMCP were reviewed. VPI severity was clinically graded using PWSS. All patients underwent MRI to grade palatal abnormalities, using a novel MRI grading scale. PWSS and cleft severity on MRI were compared. A subgroup of patients (n = 19) underwent palatoplasty. Preoperative and postoperative PWSS were compared. Degree of PWSS improvement was then correlated with the preoperative MRI grade. RESULTS: Twenty-nine out of 30 MRIs demonstrated abnormal palate anatomy. Of the 30 patients evaluated, 5 clinically improved with speech therapy alone. In this subgroup, MRI severity did not correlate with PWSS (P = 0.06-0.6). Nineteen patients underwent palatoplasty. Of these, 14 demonstrated improved postoperative PWSS. There were no significant correlations between severity of cleft on imaging and preoperative PWSS or score improvement (P = 0.056-0.65). CONCLUSION: While MRI accurately identifies structural abnormalities of the soft palate, these abnormalities do not reliably correspond to clinical severity. Clinical examination including speech scores and dynamic speech testing, rather than static MRI, should guide treatment decisions and surgical indications.

Custom porous polyethylene implants for large-scale pediatric skull reconstruction: early outcomes.

BACKGROUND AND PURPOSE: Some of the most problematic craniofacial injuries in pediatric plastic surgery are large calvarial defects in children who have passed the age of maximal dural osteogenic potential and yet are too young to yield split calvarial grafts. Porous polyethylene (Medpor; Porex) is an alloplastic material that can be customized to precisely match a cranial defect. We present a clinical series that demonstrates successful use of porous polyethylene cranioplasties in large pediatric cranial defects. METHODS: From 2007 to 2009, 9 pediatric patients underwent custom-made porous polyethylene cranioplasties for large calvarial defects. Descriptive statistical analyses were performed on the cause of the defects, time to cranioplasty, size of defect, reconstruction technique, and postoperative healing. RESULTS: A total of 5 boys and 4 girls, with a mean age of 6.8 years, underwent 9 cranioplasties incorporating custom porous polyethylene implants. Initial pathologic findings included 7 patients with traumatic brain injuries, 1 patient with intractable seizures, and 1 patient with brain cancer. Initially, each patient had a craniectomy followed by replacement of the frozen bone "flap." All patients experienced either infection or resorption of the bone leading to a permanent defect. The mean defect size was 152 cm. The mean delay between the removal of failed bone "flap" and the final implant cranioplasty was 6.8 months. At the last follow-up, which averaged 3.6 months, all patients had stable wounds with acceptable cranial contour. CONCLUSIONS: For pediatric large-scale calvarial defects, custom-made porous polyethylene implants can be safely used for cranioplasty. Tissue expansion and acellular dermal matrix were useful tools to help augment the soft tissues of the scalp before cranioplasty to prevent complications of implant extrusion and wound breakdown.