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1.
Cleft Palate Craniofac J ; : 10556656241272736, 2024 Sep 11.
Article in English | MEDLINE | ID: mdl-39262157

ABSTRACT

OBJECTIVE: To examine the impact of primary rhinoplasty on subsequent rhinoplasties for patients with cleft lip nasal deformity. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Individuals who underwent definitive cleft lip repair at our institution from 2000 to 2006 with a current age of 18 or older. MAIN OUTCOME MEASURES: Number and timing of subsequent rhinoplasties. RESULTS: Among 199 individuals, 94 (47.2%) underwent primary rhinoplasty. Follow-up was 15.0 ± 4.8 years in the PR cohort and 15.0 ± 5.1 years in the NPR cohort (p = 0.993). In bilateral cleft lip, interdomal suture predicted fewer subsequent rhinoplasties (ß=-0.310, p = 0.033), while history of primary rhinoplasty predicted greater age at subsequent rhinoplasty (ß=1.800, p = 0.040). Among individuals with follow-up beyond age 18, intranasal stenting predicted fewer subsequent rhinoplasties (ß=-0.609, p = 0.015). Most underwent subsequent nasal correction aside from 7 (19.4%) and 9 (20.9%) in the PR and NPR cohorts, respectively (p = 0.536). There was no difference in mean subsequent rhinoplasties between cohorts (1.1 ± 0.8 versus 1.3 ± 1.1, p = 0.284). Individuals with complete cleft lip underwent more lifetime rhinoplasties (1.9 ± 1.0 versus 1.2 ± 1.2, p = 0.007). CONCLUSIONS: Primary rhinoplasty with interdomal tip sutures in bilateral cleft lip was associated with fewer subsequent rhinoplasties. Primary rhinoplasty may delay subsequent nasal correction, though most who underwent primary rhinoplasty ultimately required nasal correction later in childhood. Postoperative nasal stenting may provide longer-term nasal benefits and should be considered at time of definitive cleft lip repair.

2.
Ann Plast Surg ; 92(4S Suppl 2): S132-S135, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38556661

ABSTRACT

INTRODUCTION: Vascular malformations (VMs) typically appear at birth and grow commensurately with patients. They can vary broadly in vessel type and tissue involvement, and upper extremity (UE) VMs can pose unique functional and aesthetic challenges in children. Given the advent of operative and nonoperative technologies like sclerotherapy and medications, a contemporary review of the surgical management of UE VMs is warranted. METHODS: We performed a retrospective review of all patients who had surgical management of VMs from 2010 to 2021 at The Children's Hospital of Philadelphia. Demographics, lesion characteristics, treatment (including preceding nonsurgical therapies), complications, and final outcomes were recorded. Operative notes were reviewed for date of operation, depth of excision, type of closure, and current procedural terminology code. RESULTS: Sixty-seven patients with 88 procedures were studied. Average patient age was 5.8 years, with 64% White and 67% male. Venous (34%) and lymphatic (19%) malformations were most common, and anatomic locations were most frequently on the hand (33%) and forearm (25%). The average lesion diameter was 4.2 cm, although this varied by location (eg, 2.9 cm, hand; 11.1 cm, chest wall). Fifty-eight patients (87%) underwent surgical excision as their index procedure, and 9 had sclerotherapy before surgery. Thirty-nine patients (60%) had subcutaneous excisions, and the remainder required subfascial or intramuscular excisions. Nearly all excisions were closed primarily (97%). Of the 53 patients with documented follow-up, 32 patients (60%) had complete resolution of their lesion as of their final visit. Thirty of these 32 patients with no clinical evidence of residual VM had only 1 surgery for excision. CONCLUSION: Upper extremity VMs were composed of diverse conditions with varying vessel types, size, depth, and anatomic sites. Surgical excision of VMs of the UE was safe and effective. A majority of VMs were fully excised after 1 procedure and frequently closed primarily with relatively low complication rates. Future work should investigate decision-making and outcomes of all treatment options of VMs of the UE for optimal functionality and aesthetics.


Subject(s)
Vascular Malformations , Veins , Child , Infant, Newborn , Humans , Male , Child, Preschool , Female , Retrospective Studies , Veins/surgery , Vascular Malformations/surgery , Sclerotherapy/methods , Hand , Treatment Outcome
3.
Ann Surg ; 2024 Apr 22.
Article in English | MEDLINE | ID: mdl-38647147

ABSTRACT

OBJECTIVE/ SUMMARY BACKGROUND DATA: We propose the first classification scheme for macroglossia in patients with Beckwith-Wiedemann Syndrome (BWS), the BWS Index of macroGlossia (BIG). METHODS: Patients with molecularly confirmed BWS seen from 2004-2023 were included to develop this system. Relationships among BIG scores, tongue reduction surgery, BWS clinical score, percent mosaicism, and polysomnography findings were examined. RESULTS: Patients were classified from BIG0 to BIG3. BIG0 includes those without macroglossia; BIG1 includes those with macroglossia not protruding beyond the teeth/alveolus; BIG2 includes those with tongue protrusion past the teeth/alveolus to the lips but that can be contained within the mouth; and BIG3 includes those with tongues that protrude beyond the teeth/alveolus and lips but that cannot be closed within the mouth. Of the 459 patients with molecularly confirmed BWS, 266 (58.0%) patients were scored. One hundred and eleven (41.7%) were BIG0, 44 (16.5%) were BIG1, 90 (33.8%) were BIG2, and 21 (7.9%) were BIG3. As scores increased, patients had an increased incidence of tongue reduction surgery (BIG0: 0% versus BIG1: 20.5% versus BIG2: 51.1% versus BIG3: 100%; r=0.66, P <0.01). Higher BIG scores were associated with elevated BWS clinical scores (r=0.68, P <0.01) and increased tissue mosaicism (r=0.50, P <0.01) as well as trends towards worse obstructive apnea-hypopnea indices (r=0.29, P =0.02) and lower SpO 2 nadirs (r=-0.29, P =0.02). CONCLUSION: In this large series of patients with Beckwith-Wiedemann Syndrome, increased BIG score correlates with undergoing tongue reduction surgery and increased phenotypic severity. Adoption of the BIG scoring system may facilitate communication and risk stratification across institutions.

4.
JMIR Dermatol ; 6: e49068, 2023 Nov 02.
Article in English | MEDLINE | ID: mdl-37917151

ABSTRACT

BACKGROUND: Despite their potential for adverse health effects, skin-lightening products remain popular among South Asian Americans. OBJECTIVE: This study investigates attitudes toward skin tone and the prevalence and adverse effects of skin-lightening product use among South Asian Americans. METHODS: We conducted a cross-sectional study, recruiting and surveying 175 women or nonbinary individuals meeting the following inclusion criteria: (1) lived in the United States, (2) identified as South Asian, and (3) were raised by parents born in South Asian countries. RESULTS: Of the 175 participants, 55 (31%) respondents used a skin-lightening product before. Parental pressure to use skin-lightening products and decreased time spent in the United States were significantly associated with skin-lightening product use (odds ratio [OR] 8.51, 95% CI 3.33-21.78, P<.001, and OR 0.70, 95% CI 0.52-0.96, P=.03, respectively). Although only 6 of the 55 (11%) users reported being aware of the potential side effects of skin-lightening products, 33 (60%) reported adverse effects, with acne, skin sensitivity, and dry skin being the most common. Users and nonusers equally endorsed statements associating lighter skin with increased attractiveness (P=.31), marriageability (P=.94), social status (P=.98), self-esteem (P=.73), and respect received from others (P=.74). CONCLUSIONS: The use of skin-lightening products among South Asian Americans is common and linked to social and psychological factors. Parental pressure and cultural beauty standards may play a significant role in perpetuating this practice. This study highlights the need for educational campaigns about the potential health risks associated with skin-lightening and increased efforts to challenge harmful beauty standards.

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