Publications by authors named "Lorelei Grunwaldt"

49 Publications

Transcriptomic evaluation of pediatric localized scleroderma skin with histological and clinical correlation.

Arthritis Rheumatol 2021 Apr 12. Epub 2021 Apr 12.

University of Pittsburgh, Pittsburgh, PA, USA.

Objective: Juvenile localized scleroderma (jLS) is an autoimmune disease of the skin in which the pathogenesis is not well understood due to its rarity. Our goal was to determine the skin transcriptome of LS tissue compared with healthy controls to identify molecular targets using RNA sequencing (RNAseq). Differentially expressed genes (DEGs) identified in jLS patients were compared with histopathological features, clinical features and used to cluster jLS patients.

Methods: RNAseq was performed on paraffin-embedded skin (n=28 jLS, n=10 pediatric healthy) using the Illumina HTS and TrueSeq Access library preparation, aligned with STAR and analyzed using DESeq2. Standardized histology scoring was developed for inflammation and collagen deposition and was completed by 2 blinded pathologists. Spearman's correlation was used to determine significance between DEGs and histology.

Results: We identified 589 significant DEGs between jLS and controls. Hierarchical clustering demonstrated three distinct jLS immunophenotype groupings. Degree of inflammatory cell infiltrates significantly correlated with HLA-DPB1, HLA-DQA2, HLA-DRA, and STAT1 (rs> 0.5, p<0.01). Collagen thickness correlated with collagen organization genes, but also with genes identified in the inflammatory infiltrate correlation, such MHC Class I and II and interferon gamma signaling.

Conclusion: The identified groupings of jLS patients showed three distinct genetic signatures, one with upregulated inflammatory-related pathways, which corresponded to inflammatory infiltrate score, a second group with upregulated fibrosis-related pathways, and a third which corresponded to healthy skin gene expression. HLA Class II gene upregulation was observed within the inflammatory group, which has also been described for morphea peripheral blood and systemic sclerosis skin.
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http://dx.doi.org/10.1002/art.41758DOI Listing
April 2021

Musculoskeletal Pain Survey Outcomes in Cleft Surgeons and Orthodontists.

Cleft Palate Craniofac J 2021 Feb 6;58(2):222-229. Epub 2020 Aug 6.

72058University of Pittsburgh Medical Center Children's Hospital, PA, USA.

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/m. 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 ( < .01), medication ( = .03), and physical therapies ( < .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.
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http://dx.doi.org/10.1177/1055665620946184DOI Listing
February 2021

Pain outcomes following mastectomy or bilateral breast reduction for transgender and nontransgender patients who received pectoralis nerve blocks.

Paediatr Anaesth 2020 09 6;30(9):1049-1050. Epub 2020 Aug 6.

Department of Anesthesiology and Perioperative Medicine, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

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http://dx.doi.org/10.1111/pan.13969DOI Listing
September 2020

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

J Plast Reconstr Aesthet Surg 2020 Dec 21;73(12):2178-2184. Epub 2020 May 21.

Division of Plastic Surgery, Section of Pediatric Plastic Surgery. Albany Medical Center and Bernard and Millie Duker Children's Hospital at Albany Medical Center. Albany, New York.

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.
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http://dx.doi.org/10.1016/j.bjps.2020.05.028DOI Listing
December 2020

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

Plast Reconstr Surg Glob Open 2019 Dec 31;7(12):e2516. Epub 2019 Dec 31.

Department of Plastic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pa.

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%, = 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%, = 0.017). The major and minor complication rates were 1.7% (Wise = 2.0%, vertical = 0%, = NS) and 23.3% (Wise = 20.4%, vertical = 36.4%, = NS), respectively. Adolescents undergoing Wise incision demonstrated statistically significant improvement in NAC contour (Wise = 61%, vertical = 47%, = 0.028) and overall aesthetic outcome (Wise = 25%, vertical = 17%, = 0.008) with scarring not being a negative factor (Wise = -16%; vertical = -35%, = 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.
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http://dx.doi.org/10.1097/GOX.0000000000002516DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6964923PMC
December 2019

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

Cleft Palate Craniofac J 2020 02 24;57(2):148-160. Epub 2019 Oct 24.

Division of Pediatric Otolaryngology, UPMC Children's Hospital of Pittsburgh, PA, USA.

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 ( < .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.
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http://dx.doi.org/10.1177/1055665619882566DOI Listing
February 2020

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

J Craniofac Surg 2019 Jan;30(1):228-230

Children's Hospital of Pittsburgh, University of Pittsburgh Pediatric Plastic and Reconstructive Surgery, Pittsburgh, PA.

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.
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http://dx.doi.org/10.1097/SCS.0000000000004868DOI Listing
January 2019

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

Plast Reconstr Surg Glob Open 2018 Jul 13;6(7):e1766. Epub 2018 Jul 13.

Department of Plastic Surgery, University of Pittsburgh, Pittsburgh, Pa.

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.
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http://dx.doi.org/10.1097/GOX.0000000000001766DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6110672PMC
July 2018

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

Int J Pediatr Otorhinolaryngol 2018 Oct 12;113:34-37. Epub 2018 Jul 12.

Department of Otolaryngology--Head & Neck Surgery Children's Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Faculty Pavilion, 7th Floor, Pittsburgh, PA, USA, 15224. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijporl.2018.07.019DOI Listing
October 2018

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

Cleft Palate Craniofac J 2018 10 10;55(9):1282-1288. Epub 2018 Jul 10.

1 Division of Pediatric Plastic Surgery and the Cleft Craniofacial Center, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.

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.
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http://dx.doi.org/10.1177/1055665618763374DOI Listing
October 2018

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

Plast Reconstr Surg 2017 Sep;140(3):582-590

Pittsburgh, Pa.; and Madison, Wis.

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.
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http://dx.doi.org/10.1097/PRS.0000000000003602DOI Listing
September 2017

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

Pediatr Emerg Care 2019 Sep;35(9):618-623

From the Department of Pediatric Plastic Surgery.

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.
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http://dx.doi.org/10.1097/PEC.0000000000001132DOI Listing
September 2019

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

Cleft Palate Craniofac J 2017 11 12;54(6):726-733. Epub 2016 Sep 12.

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.
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http://dx.doi.org/10.1597/15-319DOI Listing
November 2017

PHACE syndrome is associated with intracranial cavernous malformations.

Childs Nerv Syst 2016 Aug 28;32(8):1463-9. Epub 2016 Apr 28.

Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, 4401 Penn Ave, Faculty Pavilion, 4th Floor, Pittsburgh, PA, 15224, USA.

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.
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http://dx.doi.org/10.1007/s00381-016-3097-zDOI Listing
August 2016

Antibiotic Use in Primary Palatoplasty: A Survey of Practice Patterns, Assessment of Efficacy, and Proposed Guidelines for Use.

Plast Reconstr Surg 2016 Feb;137(2):574-582

St. Petersburg, Fla.; Baltimore, Md.; and Pittsburgh, Pa. From the Department of Plastic and Reconstructive Surgery, University of Pittsburgh Medical School; the Divisions of Pediatric Plastic Surgery and Pediatric Infectious Diseases, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center; and the Department of Surgery, Allegheny General Hospital.

Background: The literature provides no guidelines for antibiotic use in palatoplasty. The authors sought to ascertain practice patterns; review a large, single-surgeon experience, and propose guidelines for antibiotic use in primary palatoplasty.

Methods: A six-question survey was e-mailed to all surgeons of the American Cleft Palate-Craniofacial Association. A retrospective study was also conducted of the senior author's 10-year primary palatoplasty series, and two groups were studied. Group 1 received no antibiotics. Group 2 received preoperative and/or postoperative antibiotics.

Results: Three hundred twelve of 1115 surgeons (28 percent) responded to the survey. Eighty-five percent administered prophylactic antibiotics, including 26 percent who used a single preoperative dose. A further 23 percent gave 24 hours of postoperative therapy; 12 percent used 25 to 72 hours, 16 percent used 4 to 5 days, and 12 percent used 6 to 10 days. Five percent of surgeons administered penicillin, 64 percent administered a first-generation cephalosporin, 13 percent administered ampicillin/sulbactam, and 8 percent gave clindamycin. The authors reviewed 311 patients; 173 receive antibiotics and 138 did not. Delayed healing and fistula rates did not differ between groups: 16.8 percent versus 15.2 percent (p = 0.71) and 2.9 percent versus 1.4 percent (p = 0.47), respectively. A single patient treated without antibiotics developed a postoperative bacteremia. This case did not meet the Centers for Disease Control definition of a surgical site infection, but the patient developed a palatal fistula.

Conclusions: Antibiotic use in primary palatoplasty varies widely. The authors' data support a clinician's choice to forego antibiotic use; however, given the significance of palatal fistulae and the single case of postoperative streptococcal bacteremia, the study group recommends a single preoperative dose of ampicillin/sulbactam. Current evidence cannot justify the use of protracted antibiotic regimens.

Clinical Question/level Of Evidence: Therapeutic, III.
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http://dx.doi.org/10.1097/01.prs.0000475784.29575.d6DOI Listing
February 2016

Obstetrical brachial plexus palsy: Can excision of upper trunk neuroma and nerve grafting improve function in babies with adequate elbow flexion at nine months of age?

J Plast Reconstr Aesthet Surg 2016 May 7;69(5):629-33. Epub 2016 Jan 7.

Department of Plastic Surgery, Children's Hospital of Pittsburgh of UPMC, USA. Electronic address:

Accepted indications for exploration in obstetrical brachial plexus palsy (OBPP) vary by center. Most agree that full elbow flexion against gravity at nine months of age implies high chance of spontaneous recovery and thus excludes a baby from surgical intervention. However, there are certain movements of the shoulder and forearm that may not be used frequently by the infant, but are extremely important functionally as they grow. These movements are difficult to assess in a baby and may lead to some clinicians to recommend conservative treatment, when this cohort of infants may in fact benefit substantially from surgery. A retrospective review was conducted on all infants managed surgically at the Brachial Plexus Center of a major children's hospital from 2009 to 2014. Further analysis identified five patients who had near-normal AMS scores for elbow flexion but who had weakness of shoulder abduction, flexion, external rotation, and/or forearm supination. In contrast to standard conservative management, this cohort underwent exploration, C5-6 neuroma excision, and sural nerve grafting. Data analysis was performed on this group to look for overall improvement in function. During an average follow-up period of 29 months, all patients made substantial gains in motor function of the shoulder and forearm, without loss of elbow flexion or extension, or worsening of overall outcome. In select infants with brachial plexus injuries but near-normal AMS scores for elbow flexion, surgical intervention may be indicated to achieve the best functional outcome.
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http://dx.doi.org/10.1016/j.bjps.2015.12.020DOI Listing
May 2016

A case of spinal epidural venous malformation with mediastinal extension: management with combined surgery and percutaneous sclerotherapy.

J Neurosurg Pediatr 2016 May 15;17(5):612-7. Epub 2016 Jan 15.

Departments of 1 Neurological Surgery.

While spinal epidural arteriovenous malformations, fistulas, and shunts are well reported, the presence of a venous malformation in the spinal epidural space is a rare phenomenon. Herein, the authors report the clinical presentation, imaging findings, pathological features, and the outcome of surgical and percutaneous interventional management of a mediastinal and spinal epidural venous malformation in a young woman who presented clinically with neurogenic claudication from presumed venous hypertension precipitating the formation of a syrinx. The patient underwent a C6-T5 osteoplastic laminectomy for decompression of the spinal canal and subtotal resection of the epidural venous malformation, followed by percutaneous sclerotherapy of the mediastinal and residual anterior spinal venous malformation. She developed transient loss of dorsal column sensation, which returned to baseline within 3 weeks of the surgery. A 6-month postoperative MRI study revealed complete resolution of the syrinx and the mediastinal venous malformation. Twelve months after the surgery, the patient has had resolution of all neurological symptoms with the exception of her premorbid migraine headaches. A multidisciplinary approach with partial resection and the use of percutaneous sclerotherapy for the residual malformation can be used to successfully treat a complex venous malformation.
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http://dx.doi.org/10.3171/2015.9.PEDS15341DOI Listing
May 2016

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

J Craniofac Surg 2016 Jan;27(1):159-62

*University of Pittsburgh Medical Center, Pittsburgh, PA †University of Wisconsin, Madison, WI.

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.
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http://dx.doi.org/10.1097/SCS.0000000000002206DOI Listing
January 2016

Speech Outcomes After Clinically Indicated Posterior Pharyngeal Flap Takedown.

Ann Plast Surg 2016 Oct;77(4):420-4

From the Division of Pediatric Plastic Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA.

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.
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http://dx.doi.org/10.1097/SAP.0000000000000632DOI Listing
October 2016

A review of eight unusual pediatric skin and soft-tissue lesions: Diagnosis, workup, and treatment.

J Plast Reconstr Aesthet Surg 2015 Dec 6;68(12):1637-46. Epub 2015 Jul 6.

Department of Plastic Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA. Electronic address:

The spectrum of clinical presentations in pediatric skin and soft-tissue lesions often makes diagnosis challenging. Delays in diagnosis and subsequent treatment can be life threatening, and they can subject patients to more complicated reconstruction. This retrospective case series reviews the presentation, management, and current literature on eight pediatric skin and soft-tissue lesions. For rare lesions and those with unclear pathology, a multidisciplinary approach is strongly advocated for optimal management.
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http://dx.doi.org/10.1016/j.bjps.2015.06.017DOI Listing
December 2015

Bilateral Circum-scrotal Z-plasties for Creation of Single Scrotal Sac in Cloacal Exstrophy With Widely Divergent Scrotal Sacs.

Urology 2015 Oct 14;86(4):808-9. Epub 2015 Jul 14.

Division of Pediatric Plastic Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA.

Significant number of patients with cloacal exstrophy variants present with poorly developed scrotum. Widely divergent hypoplastic scrotal sacs pose a technical challenge for scrotal reconstruction. We describe a technique of bilateral Z-plasty for the creation of single scrotal sac in a patient with widely divergent, hypoplastic scrotal sacs.
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http://dx.doi.org/10.1016/j.urology.2015.04.053DOI Listing
October 2015

BRAF mutations are also associated with neurocutaneous melanocytosis and large/giant congenital melanocytic nevi.

Pediatr Dev Pathol 2015 Jan-Feb;18(1):1-9. Epub 2014 Dec 9.

1 Department of Pathology, Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.

NRAS and BRAF mutations occur in congenital melanocytic nevi (CMN), but results are contradictory. Sixty-six prospectively collected CMN patients were analyzed for NRAS Q61 mutations using Sanger sequencing. Negative cases were evaluated for BRAF V600E mutation. NRAS Q61 mutations affected 51 patients (77.3%), and BRAF V600E was found in 5 (7.6%). NRAS Q61 mutation affected 29 (80.6%) of 36 giant, 16 (80.0%) of 20 large, and 5 (62.5%) of 8 medium-size CMN; BRAF mutation affected 1 (5%) of 20 large and 4 (11.4%) of 36 giant CMN. Compared to NRAS, BRAF-mutated nevi show scattered/extensive dermal and subcutaneous nodules (100% BRAF+ vs 34.8% NRAS+) (P=0.002). Neurocutaneous melanocytosis (NCM) affected 16 (24.2%) of 66 patients, with NRAS Q61 mutation in 12 (75.0%), and BRAF V600E in 2 (12.5%), P=0.009. Two patients were negative for both mutations (12.5%). In conclusion, although NRAS Q61 mutations predominate, BRAF V600E mutation also affects patients with large/giant CMN (L/GCMN), and with NCM, a novel finding. BRAF V600E is also associated with increased dermal/subcutaneous nodules. These findings open the possibility of BRAF-targeted therapy in some L/GCMN and NCM cases.
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http://dx.doi.org/10.2350/14-10-1566-OA.1DOI Listing
March 2015

Neurofibromas with imaging characteristics resembling vascular anomalies.

AJR Am J Roentgenol 2014 Dec;203(6):W697-705

1 Department of Radiology, Division of Pediatric Interventional Radiology, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 4401 Penn Ave, Fl 2, Pittsburgh, PA 15224.

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.
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http://dx.doi.org/10.2214/AJR.13.12409DOI Listing
December 2014

Successful reconstruction of complex pediatric nasal lesions: improving outcomes using dermal regenerative templates.

Plast Reconstr Surg Glob Open 2014 Feb 6;2(2):e107. Epub 2014 Mar 6.

Division of Pediatric Plastic and Reconstructive Surgery, University of Pittsburgh, Cleft/Craniofacial Center, Children's Hospital of Pittsburgh, Pittsburgh, Pa.

Background: Dermal regenerate templates are currently widely used in both adult and pediatric burn reconstruction. Despite this, the safety and efficacy of regenerate templates combined with full-thickness skin grafts for the reconstruction of pediatric facial defects traditionally treated with local flaps is not widely published. The aim of this study is to report the safety and efficacy of pediatric nasal defect reconstruction using regenerative templates/full-thickness skin grafts.

Methods: A retrospective review of one institution's experience with pediatric nasal defects treated with regenerative templates was performed. All patients (n = 4) were treated with a multistage protocol. Two reviewers independently assigned Visual Analogue Cosmetic Scale (VACS) scores: 1 surgeon and 1 nonsurgical researcher not involved in patient care. Standardized photographs (anteroposterior, oblique, lateral, and worm's eye view) were assigned VACS scores according to a 100-point scale: "abhorrent," 0-24; "poor," 25-49; "moderate," 50-74; and "excellent," 75-100. Statistical analysis was performed using Mann-Whitney U and Wilcoxon paired signed-rank tests.

Results: Four patients (2 boys and 2 girls, average age 6.8 yr) who met the inclusion criteria were identified. A total of 5 nasal lesions (2 spitz nevi, 1 vascular lesion, and 2 congenital nevi) were removed. The preoperative VACS score was 45.2 (range, 5-70), compared with 84.5 (range, 45-100) postoperatively (P <0.000). There was no significant difference between raters (preoperative, P = 0.346; postoperative, P = 0.678).

Conclusions: The reconstruction of complex pediatric nasal lesions using dermal regenerative templates and full-thickness postauricular skin grafts is safe and effective, and associated with low morbidity and significant improvement in VACS scores.
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http://dx.doi.org/10.1097/GOX.0000000000000033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4173827PMC
February 2014

Pierre Robin Sequence: a perioperative review.

Anesth Analg 2014 Aug;119(2):400-12

From the Departments of *Anesthesiology and †Plastic Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania; and ‡Pediatric Otolaryngology, UH Rainbow Babies and Children's Hospital CWRU School of Medicine, Cleveland, Ohio.

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.
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http://dx.doi.org/10.1213/ANE.0000000000000301DOI Listing
August 2014

Complete penile amputation during ritual neonatal circumcision and successful replantation using postoperative leech therapy.

Urology 2014 Aug 11;84(2):472-4. Epub 2014 Jun 11.

Division of Pediatric Urology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.

Circumcision is the most common surgical procedure in males in the United States, and minor complications are not uncommon. Major complications like partial penile amputations have been reported with successful replantation. Complete penile amputations in adult males have been described, and successful replantation has been reported with increasing success. We report a case of complete penile amputation at the penopubic junction using a Mogen clamp in a 7-day-old neonate with replantation using postoperative leech therapy. To our knowledge this is the first time leech therapy has been used postoperatively for neonatal penile amputation.
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http://dx.doi.org/10.1016/j.urology.2014.04.021DOI Listing
August 2014

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

J Craniofac Surg 2014 Mar;25(2):343-7

From the *Division of Pediatric Plastic Surgery, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; and †Departments of Oral Biology and Surgery/Plastic Surgery, Georgia Health Sciences University, Augusta, Georgia.

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.
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http://dx.doi.org/10.1097/SCS.0000000000000375DOI Listing
March 2014

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

Ann Plast Surg 2015 Feb;74(2):182-6

From the Department of Pediatric Plastic and Reconstructive Surgery, University of Pittsburgh, Cleft/Craniofacial Center, Children's Hospital of Pittsburgh, Pittsburgh, PA.

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.
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http://dx.doi.org/10.1097/SAP.0b013e3182956632DOI Listing
February 2015

Bilateral congenital amazia: a case report and systematic review of the literature.

J Plast Reconstr Aesthet Surg 2014 Jan 26;67(1):27-33. Epub 2013 Jul 26.

Department of Plastic Surgery, University of Pittsburgh, 3550 Terrace Street, 6B Scaife Hall, Pittsburgh, PA 15261, USA. Electronic address:

Background: Congenital breast anomalies present challenging management decisions to the plastic surgeon. One must consider the optimal age of reconstruction as well as the ideal surgical technique. Amazia, a very rare condition characterised by a complete lack of breast tissue in the presence of a nipple areolar complex (NAC), is one such congenital breast anomaly.

Methods: A comprehensive systematic review of the literature was performed to examine the various approaches to reconstruction of congenital breast anomalies. From this review, the data compiled included patient demographics and operative details, including type of reconstruction, treatment of the contralateral breast and treatment of the NAC. A case of bilateral amazia is also reported.

Results: Of 178 articles, 13 ultimately met the inclusion criteria and 54 individual patient reconstructions were identified from these papers. At the time of reconstruction, the patients were in the range of 13-54 years, with an average age of 27.6 years. Prosthetic and autologous reconstructions were equally represented (19 patients each, 35.2%; Table 2). Autologous reconstruction with prosthesis was slightly less common (15 patients, 27.8%). One patient was reconstructed using autologous lipo-augmentation only. Of the 36 cases in which the approach to the NAC was addressed, most (66.7%) were not reconstructed.

Conclusions: Amazia is a very rare congenital anomaly of the breast. This systematic review of the literature highlights the need for better reporting and examination of this type of data to allow for future study and to better advise on decision making regarding the timing of reconstruction, surgical technique and the approach to the NAC.
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http://dx.doi.org/10.1016/j.bjps.2013.06.048DOI Listing
January 2014