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.

Outcomes of Furlow Double-Opposing Z-Plasty Palatoplasty for the Treatment of Symptomatic Overt and Occult Submucous Cleft Palate: A Comparison Study.

BACKGROUND: The submucous cleft palate can be overt or occult and may require surgical repair. The double-opposing Z-plasty (Furlow repair) is the authors' center's preferred approach. This study evaluated complication rates, differences in outcome between overt and occult types, and patient factors associated with surgical failure. METHODS: This retrospective study reviewed documentation on all patients who underwent Furlow Z-plasty for submucous cleft palate at a single center between 2004 and 2018. Speech pathology was quantified using the Pittsburgh Weighted Speech Score. RESULTS: A total of 351 patients were included (125 overt and 226 occult cases). Furlow Z-plasty was successful (postoperative Pittsburgh Weighted Speech Score <7 without recommendation for secondary speech surgery) in 291 patients (82.1 percent). Apart from those requiring secondary surgery, there were no documented complications. Occult-type patients were 7.5 years old at palatoplasty with a speech score of 14.1; overt-type patients were 6.5 years old with a score of 15.7. Postoperative speech scores were similar for both groups. Secondary speech surgery patients had a higher preoperative score (16.9 versus 14.2). Age at time of palatoplasty and submucous cleft palate type were not predictive of the need for secondary surgery. Syndromic patients had higher preoperative and postoperative speech scores (15.6 and 7.5, respectively) than nonsyndromic patients (14.3 and 4.3) and needed secondary surgery more often (24.4 percent versus 9.2 percent). V-shaped velar vaulting on preoperative assessment was present in 92 percent of occult-type patients. CONCLUSIONS: Furlow palatoplasty is a safe and effective means of repairing submucous cleft palate. Patients with the occult type presented later with a lower Pittsburgh Weighted Speech Score. High preoperative speech score and syndromic status were associated with the need for secondary speech surgery. V-shaped velar vaulting is a reliable sign of occult submucous cleft palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Transcriptomic Evaluation of Juvenile Localized Scleroderma Skin With Histologic and Clinical Correlation.

OBJECTIVE: Juvenile localized scleroderma (LS) is an autoimmune disease of the skin whose pathogenesis is not well understood due to the rarity of the disease. This study was undertaken to determine the skin transcriptome in skin biopsy tissue from children with juvenile LS compared to pediatric healthy controls, with identification of significant molecular targets using RNA sequencing (RNA-Seq). In this study, differentially expressed genes (DEGs) were assessed for correlations with histopathologic and clinical features in children with juvenile LS, and were used to group the children into distinct genetic clusters based on immunophenotype. METHODS: RNA-Seq was performed on sections of paraffin-embedded skin tissue obtained from 28 children with juvenile LS and 10 pediatric healthy controls. RNA-Seq was carried out using an Illumina HTS TruSeq RNA Access library prep kit, with data aligned using STAR and data analysis using a DESeq2 platform. A standardized histologic scoring system was used to score skin sections for the severity of inflammation and levels of collagen deposition. Histologic scoring was completed by 2 pathologists who were blinded with regard to the status of each sample. Spearman's rank correlation coefficients were used to assess significant correlations between DEG expression profiles and skin histologic findings in patients with juvenile LS. RESULTS: We identified 589 significant DEGs in children with juvenile LS as compared to healthy controls. Hierarchical clustering was used to demonstrate 3 distinct juvenile LS immunophenotype clusters. The histologic scores of skin inflammation (based on numbers and categories of inflammatory cell infiltrates) were significantly correlated with the expression levels of HLA-DPB1, HLA-DQA2, HLA-DRA, and STAT1 genes (rs > 0.5, P < 0.01). Collagen thickness correlated with the expression levels of collagen organization genes as well as with genes found to be correlated with the severity of inflammation, including genes for major histocompatibility complex (MHC) class I, MHC class II, and interferon-γ signaling. CONCLUSION: Among children with juvenile LS, 3 distinct genetic signatures, or clusters, were identified. In one cluster, inflammation-related pathways were up-regulated, corresponding to the histologic skin inflammation score. In the second cluster, fibrosis-related pathways were up-regulated. In the third cluster, gene expression in the skin corresponded to the patterns seen in healthy controls. Up-regulation of HLA class II genes was observed within the first cluster (characterized by predominant inflammation), a feature that has also been observed in the peripheral blood of patients with morphea and in the skin of patients with systemic sclerosis.

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.

Hyperbaric Oxygen Therapy for Large Composite Grafts: An Alternative in Pediatric Facial Reconstruction.

BACKGROUND: Management of pediatric facial defects can be challenging, as reattachment of large composite grafts is usually unsuccessful. Hyperbaric oxygen therapy (HBO) has been researched to augment composite graft survival, but clinical use for this application remains anecdotal. The authors present their successful experience managing select cases with large composite grafts and HBO as an adjunct. METHODS: A retrospective chart review identified children presenting with facial defects and managed operatively with large composite grafts (≥1.5 × 1.5 cm) and HBO therapy. Records were reviewed for defect characteristics, management details, and outcomes at last follow-up. RESULTS: Nine children (avg. 8.4 years, range 1.6-15.1) presented with ear or nose defects secondary to dog bites (n=7), falls (n=1), or congenital causes (n=1). Three experienced ear amputations, and six suffered nasal avulsions of varying degrees. All avulsed ears were reattached. Three cases of nose avulsions were reattached; the other three underwent secondary reconstruction with composite ear grafts. HBO was initiated immediately and continued for 8-10 days. All grafts survived at least 80% with no postoperative complications. At last follow-up (avg. 30.1 months; 0.8-63.9), all patients demonstrated good cosmetic results with minimal residual deformity. CONCLUSION: When reconstruction of pediatric facial defects warrants a large chondrocutaneous graft, immediate postoperative HBO therapy can increase survival. Particularly when reattaching amputated segments, if successful, this approach offers an anatomically ideal result without donor site morbidity. If unsuccessful, it does not "burn bridges" and decreases the extent of secondary reconstruction. The authors present their HBO protocol along with a review of available literature.

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.

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.

Reduction Mammaplasty in Adolescents: A Comparison of Wise and Vertical Incision Patterns.

BACKGROUND: Reduction mammaplasty was shown to ameliorate physical and psychological problems in adolescents suffering from macromastia. However, benefits of the Wise compared to the vertical incision pattern have not yet been established in this population. The aim of this study is to compare the outcomes of these 2 techniques in adolescents undergoing reduction mammaplasty. METHODS: A retrospective study of adolescents undergoing breast reduction by a single surgeon between 2011 and 2017 was conducted. Wise and vertical reduction techniques were compared based on demographics, surgical outcomes, patient satisfaction, and aesthetic outcomes. Patient satisfaction was determined using the validated BREAST-Q survey, and aesthetic outcomes using the validated ABNSW system. RESULTS: A total of 60 adolescents underwent reduction mammaplasty (Wise/inferior pedicle = 80.0%, Wise/superior medial pedicle = 1.7%, vertical/superior medial pedicle = 18.3%). Patients who reported preoperative pain (Wise = 95.9%, vertical = 72.7%, P = 0.039) were more likely to undergo Wise reduction. Patients with Wise reductions also were more likely to undergo bilateral reduction (Wise = 93.9%; vertical = 63.6%, P = 0.017). The major and minor complication rates were 1.7% (Wise = 2.0%, vertical = 0%, P = NS) and 23.3% (Wise = 20.4%, vertical = 36.4%, P = NS), respectively. Adolescents undergoing Wise incision demonstrated statistically significant improvement in NAC contour (Wise = 61%, vertical = 47%, P = 0.028) and overall aesthetic outcome (Wise = 25%, vertical = 17%, P = 0.008) with scarring not being a negative factor (Wise = -16%; vertical = -35%, P = 0.004). Patient satisfaction was comparable in both groups. CONCLUSIONS: Reduction mammaplasty is a safe, effective treatment for adolescent macromastia. The similarity in complication and satisfaction rates between Wise and vertical patterns suggests that both techniques can be safely performed in the adolescent population and allow for overall improvements in aesthetic outcomes.

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 Upper Extremity Replantation: Courage in the Face of a Life-altering Injury.

BACKGROUND: Pediatric plastic surgeons perform reconstructive surgeries for various congenital, oncologic, and traumatic injuries. METHODS: Our Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC) Plastic Surgery team was tasked to care for a young man who suffered a proximal humeral amputation of his dominant upper extremity. RESULTS: A multidisciplinary team collaborated throughout his entire acute care and postoperative course, guiding treatment and care in effort to maximize function of his replanted extremity. CONCLUSIONS: This case report details the patient's unique journey and highlights his determination and courage to return back to a normal life.

Subglottic hemangioma: Understanding the association with facial segmental hemangioma in a beard distribution.

OBJECTIVE: A subglottic hemangioma (SGH) is a benign tumor of infancy that can cause severe obstruction of the airway. Infantile hemangiomas, in general, are the most common head and neck tumor in children, affecting 4-5% of the pediatric population. This retrospective cohort study characterizes subglottic infantile hemangiomas at a single vascular anomaly center over a 5-year period (2013-2017) during the era of propranolol treatment. METHODS: Queried the Vascular Anomaly Database at Children's Hospital of Pittsburgh for all infantile hemangioma(s) and then identified case of subglottic hemangiomas. Characterized key features of presentation, natural history and management for subglottic hemangiomas. A secondary differentiation focused on differences between subglottic hemangiomas associated with Beard Distribution (BD) vs not (NBD). RESULTS: Analysis of 761 cases of infantile hemangiomas demonstrated only 13 patients with subglottic hemangiomas (1.7%). Of those 13 patients, only 4 patients (30%) had BD while 2 patients (15%) had other cutaneous hemangiomas and 7 patients (55%) had no cutaneous hemangiomas. Secondarily, a total of 31 case of beard distribution cutaneous hemangiomas with 11 patients having oropharyngeal involvement (35%) but only 4 patients with subglottic hemangiomas (13%). Interestingly, 2 of the 4 BD patients had treatment failure on propranolol and required second line treatment with steroids or surgical excision while only 1 of 9 NBD patients failed propranolol treatment. As well the same 2 BD patients which failed propranolol also had PHACES syndrome. CONCLUSION: Subglottic hemangiomas are a rare presentation of infantile hemangiomas but with significant morbidity. While the classic teaching that a segmental beard distribution hemangioma raises concern for a subglottic hemangioma, this cohort indicates subglottic hemangiomas occur in a NBD presentation (1.3%), and demonstrated only an approximate 10% incidence rate with a beard distribution. But more importantly, this study raises the question that beard distribution in setting of PHACES syndrome may herald a more recalcitrant and complicated natural history for a subglottic hemangioma. This is of significant concern as risk for CVA in setting of PHACES is highest with use of steroid treatment. None of our patients had high risk extra or intra cranial vascular arterial anomalies and no CVA were noted.

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.

A Treatment Algorithm for Patients Presenting with Sagittal Craniosynostosis after the Age of 1 Year.

BACKGROUND: Sagittal craniosynostosis typically presents shortly after birth, with a scaphocephalic head shape, and is addressed surgically for functional and aesthetic concerns. This study highlights the authors' experience with a challenging patient population: those with phenotypically mild, missed, and late-developing sagittal craniosynostosis. METHODS: A prospective cohort study was conducted for all cases of sagittal craniosynostosis presenting to the authors' institution between July of 2013 and December of 2015. Patients older than 1 year with isolated sagittal craniosynostosis were included. All children were evaluated by craniofacial surgery, neurosurgery, and ophthalmology departments. All patients had dilated fundus examinations and visual evoked potentials. RESULTS: Fifty-two patients met inclusion criteria. Only nine patients have been treated surgically (17.3 percent). Two patients underwent operative correction for obvious scaphocephaly. Four patients who presented with concerning ophthalmologic evaluations and another patient with classic intracranial hypertension-related headaches underwent cranial vault expansion. Eight patients presented with inconclusive ophthalmologic evaluations. These patients were admitted for intracranial pressure monitoring, of which two were found to have elevated levels (25 percent) and underwent operative intervention. Thirty-seven other patients presented with isolated sagittal craniosynostosis in the setting of overall normocephaly without any signs concerning for intracranial hypertension. These patients continue to undergo serial evaluation. CONCLUSIONS: The authors describe their treatment protocol for a large series of patients presenting with the delayed diagnosis of sagittal craniosynostosis. Based on the early experience of the authors' center with this protocol, the risk of intracranial hypertension appears to be low in this population. The majority of patients to date have been managed nonsurgically without invasive monitoring. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

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.