Rhabdomyosarcoma and rhabdomyoma associated with nevoid basal cell carcinoma syndrome: Local treatment strategy.

This article presents the case of a child presenting with a rhabdomyosarcoma associated with a fetal rhabdomyoma in the setting of nevoid basal cell carcinoma syndrome. Oncologic strategy is discussed.

Criteria for early and late velopharyngoplasty in 61 children with cleft palate.

BACKGROUND: Velopharyngeal insufficiency persists in 15 to 30% of children with cleft palate, despite early velar surgery. Pharyngoplasty using a superior pedicle flap is the most common secondary surgery to treat velopharyngeal insufficiency. This study aims to identify the criteria leading to indicate velopharyngoplasty in 3 groups of age. MATERIALS AND METHODS: we conducted a retrospective single center study in the reference center for cleft palate in Paris from 2013 to 2016. We included 61 children with non-syndromic cleft operated on with a velopharyngoplasty for velopharyngeal insufficiency. Pre-operative speech and surgical assessments, as well as the operative reports of the children, were analyzed retrospectively using multivariate models. RESULTS: We included 61 patients. The only criteria factor for an early velopharyngoplasty was the Pittsburgh Weighted Speech Scale (PWSS) score (OR 1.20, CI 95% 1.07 to 1.4 ; P=.006). Criteria for a late velopharyngoplasty were a degradation of the velopharyngeal function (OR 16.07, CI 95% 1.7 to 518.7 ; P=.041) and lost of follow-up (OR 5.78, CI 95% 3.9 to 4320 ; P=.017). CONCLUSION: Criteria for early and late velopharyngoplasty were identified, and we demonstrated the insufficiency of Borel-Maisonny classification for scientific clinical study.

Evaluation of prenatal breastfeeding workshop to inform and support mother with antenatal diagnosis of cleft lip/palate.

INTRODUCTION: To support mother with antenatal diagnosis of cleft lip/palate (CL/P), we implement an antenatal breastfeeding workshop to promote breastfeeding and its continuation. The aim of this study was to evaluate patient satisfaction after this workshop and efficiency of this workshop on breastfeeding rates and duration. MATERIALS AND METHODS: We conducted a prospective study from February 2018 to April 2022. Patients received two surveys after the workshop and after birth, to evaluate their satisfaction. A prospective breastfeeding follow-up were pursued by email, at 1-3-6 and 12 months to determine if the breastfeeding had been continued or stopped. RESULTS: We included 124 pregnant women who participated to the workshop. After the workshop, most participants (114/124) answered to the first survey. Amongst the 114 participants who answered to the first survey, 45 participated to a face-to-face workshop; and 69 to a teleworkshop. Participants were globally satisfied or very satisfied from the workshop; and felt more capable to breastfeed their child. 95/124 participants answered to the second survey. Most of the participants considered that the workshop had help them carry on a maternal milk feeding to their child Breastfeeding was pursued 1 month at 90%, 3 months at 62.5%, 6 months at 41.9%, after 6 months at 18.4%. Duration of breastfeeding were not statistically different, depending on the cleft type(p = 0.022). CONCLUSION: The prenatal information helped the future mothers and met their requests and needs. The satisfaction rate reflected the importance of informing and supporting mothers in their feeding choices for their infants. The rate of breastfeeding at birth of a cleft-infant, whatever its type, for mothers who participated in the breastfeeding workshop, was over 90%.

Commissuroplasty for macrostomia: surgical technique and long-term aesthetic and functional results assessment.

BACKGROUND: Macrostomia or lateral cleft lip is a rare congenital deformity. In this article we describe a surgical technique of macrostomia repair developed. The objective of this article is to assess the results of our surgical technique and to validate a method for macrostomia surgical result evaluation. METHODS: We included retrospectively patients with unilateral and bilateral macrostomia, operated from 1995 to 2014 in our department. First part of the study was a satisfaction questionnaire completed by patients. The second part was subjective evaluation of frontal photography (closed mouth, wide open and smiling) by surgeons and lay people with a questionnaire. Both group completed a second questionnaire within one to six months. RESULTS: Eighteen patients answered the questionnaire. The satisfaction for all patients were considered as very good for 38.9% (n = 7) of patients and good for 44.4% (n = 8). 21 patients were photographed, 5 isolated macrostomia, 13 macrostomia with minor facial asymmetry and 3 with a major asymmetry. Surgeons evaluated the result as very good for isolated macrostomia and good for syndromic macrostomia. Layperson evaluated the result as good in isolated macrostomia and macrostomia with minor facial asymmetry and average with major facial asymmetry. P < 0.0001. The evolution of the results between medical and non-medical assessors in our two questionnaires, were non-significant. CONCLUSION: In this study, we propose a new methodology to assess commissuroplasty surgical results, with a 3 type of evaluator: patients, surgeons and laypeople. We present a simple surgical technique, that allows good results in syndromic and isolated macrostomia.