Regional variations in the presentation and surgical management of Pierre Robin sequence.
نویسندگان
چکیده
OBJECTIVES/HYPOTHESIS To estimate the current birth prevalence of isolated and syndromic Pierre Robin sequence (iPRS and sPRS), including demographic variations. To assess for regional variations in surgical airway interventions for PRS, and to determine the mean length of stay (LOS), cost of admission, complication rate, and rate of associated procedures related to tongue-lip adhesion (TLA), neonatal mandibular distraction osteogenesis (MDO), and tracheotomy. STUDY DESIGN Retrospective cross-sectional study. METHODS The 2006 and 2009 Kids Inpatient Databases were used to identify newborns and infants with PRS; analysis using cross tabulations and linear regression modeling was performed. RESULTS In 2006 and 2009, the estimated birth prevalence of iPRS was 1.8:10,000 live births and sPRS 1.4:10,000 live births. The highest rate was in whites and the lowest in non-Hispanic blacks. There were 145 TLAs (36%), 176 MDOs (43%), and 85 tracheotomies (21%). The Northeast favored a TLA strategy; the Midwest favored MDO. The mean LOS for TLA was 24.5 days, MDO 36.7 days, tracheotomy (iPRS) 44.9 days, and tracheotomy (sPRS) 53.0 days. CONCLUSIONS The birth prevalence of PRS may be higher than previously described, especially in whites. Surgical management strategies vary between regions. The overall cost of a TLA admission is lower than an MDO or tracheotomy admission, owing primarily to shorter LOS. This study was limited by not taking into account outpatient expenses (nursing care, monitoring) or need for further airway/feeding intervention over subsequent admissions. LEVEL OF EVIDENCE 2c.
منابع مشابه
Role of SOX9 in the Etiology of Pierre-Robin Syndrome
Objective(s:Cleft lip/palate are common congenital anomalies, affecting approximately 2/1000 live births. Pierre Robin Sequence is a subgroup of the cleft palate population. Chromosomal abnormalities near the SOX9 gene disrupt the regulation of this gene and prevent the SOX9 protein from properly controlling the development of facial structures, which leads to isolated PRS. The present study wa...
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Treacher Collins syndrome is characterized by the congenital hypoplasia of the zygomatic and mandible bones. It is a syndrome with a concise clinical presentation and mostly surgical treatment. Pierre Robin disorder, on the other hand, is a sequence and not a syndrome, i.e., in Pierre Robin sequence, micrognathia leads to glossoptosis. Glossoptosis, on the other hand, is responsible for the air...
متن کاملTreacher-Collins Syndrome (TCS) and Pierre Robin Sequence — Treatment and Life Expectancy
Treacher Collins syndrome is characterized by the congenital hypoplasia of the zygomatic and mandible bones. It is a syndrome with a concise clinical presentation and mostly surgical treatment. Pierre Robin disorder, on the other hand, is a sequence and not a syndrome, i.e., in Pierre Robin sequence, micrognathia leads to glossoptosis. Glossoptosis, on the other hand, is responsible for the air...
متن کاملاانجام بیهوشی عمومی برای خدمات دندانپزشکی یک بیمار مبتلا به Pierre Robin Sequence: گزارش مورد
مقدمه:Pierre Robin Sequence که قبلاً به عنوان سندروم پیر روبین نامیده میشد، شامل سه ناهنجاری مادرزادی ماندیبول کوچک، عقب افتادگی زبان و شکاف کام میباشد. به طوری که نقص اولیه عامل ایجاد نقص بعدی است. نوزادان مبتلا به این بیماری با مشکلات انسداد راه هوایی، بازگشت محتویات معده به مری وتغذیه همراهند. مداخلات صورت گرفته اغلب جهت حفظ یک راه هوایی باز است. شرح مورد: یکی از مشکلات اصلی با توجه به کو...
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ورودعنوان ژورنال:
- The Laryngoscope
دوره 124 12 شماره
صفحات -
تاریخ انتشار 2014