A retrospective study of patients with Robin sequence: Patient characteristics and their impact on clinical outcomes.
- 作者列表："Hamilton S","Dzioba A","Husein M
INTRODUCTION:Robin sequence (RS) is a congenital set of abnormalities of the head and neck, consisting of a hypoplastic mandible (micrognathia), a tongue that is displaced posteriorly (glossoptosis), and obstruction of the airway. A clear set of diagnostic criteria for this complex condition has recently been established, but there is still no consensus in the literature with respect to managing the associated airway, feeding, and hearing difficulties. The objectives of the study are: 1) to describe the management of airway and feeding issues in children with RS; and 2) to evaluate the impact of airway and feeding strategies on important clinical outcomes. METHODS:A retrospective chart review was conducted of all pediatric patients diagnosed with RS at London Health Sciences Centre from January 1995 until September 2017. The frequencies of all airway and feeding interventions were collected. Data were collected on several clinical outcomes including initial admission length, enteral feeding duration, tympanostomy tube insertion frequency, and hearing thresholds. Statistical analyses to evaluate the impact of airway and feeding strategies on clinical outcomes were carried out using independent samples t-tests and Chi-square tests, where appropriate. RESULTS:Twenty-four patients were identified. Five patients (20.8%) required airway surgery and 18 patients (75.0%) required enteral feeding. Airway surgery was significantly associated with a longer ICU admission (15.8 vs. 4.3 days, p < 0.05), a longer overall hospital admission (73.0 vs. 25.2 days, p < 0.05), a delay in introducing oral feeds (222.8 vs. 11.5 days, p < 0.05), and a higher frequency of tympanostomy tube insertions (80% vs. 23.5% requiring ≥2 insertions, p < 0.05). Enteral feeding was significantly associated with a longer ICU admission (8.8 vs. 0 days, p < 0.05) and a longer overall hospital admission (43.9 vs. 5.6 days, p < 0.05). CONCLUSIONS:This study demonstrates the substantial impact that airway and feeding difficulties have on RS patient morbidity, particularly with respect to length of hospital and ICU admission. This information has prognostic value and may be helpful in generating a management algorithm for this complex patient population.
简介: Robin序列 (RS) 是一组先天性头颈部异常，由发育不良的下颌骨 (小颌畸形) 、向后移位的舌头 (舌下垂) 、和气道阻塞。最近已经建立了一套明确的这种复杂疾病的诊断标准，但是在管理相关的气道、进食和听力困难方面，文献中仍然没有达成共识。研究的目的是: 1) 描述RS患儿气道和喂养问题的管理; 2) 评估气道和喂养策略对重要临床结局的影响。 方法: 对 1995 年 1 月至 2017 年 9 月在伦敦健康科学中心诊断为RS的所有儿童患者进行回顾性图表回顾。收集所有气道和喂养干预的频率。收集了几种临床结局的数据，包括初始入院长度、肠内喂养持续时间、鼓膜置管频率和听阈。使用独立样本t检验和卡方检验 (如适用) 进行统计分析，以评价气道和喂养策略对临床结局的影响。 结果: 确定了 24 例患者。5 例 (20.8%) 患者需要气道手术，18 例 (75.0%) 患者需要肠内喂养。气道手术与入住ICU时间延长显著相关 (15.8 vs. 4.3 天，p <0.05)，住院时间较长 (73.0 vs. 25.2 天，p <0.05)，延迟引入口服饲料 (222.8 vs. 11.5 天，p <0.05)，且鼓膜置管插入频率较高(80% vs. 23.5% 需要 ≥ 2 次插入，p <0.05)。肠内喂养与入住ICU时间延长显著相关 (8.8 vs. 0 天，p <0.05) 和更长的总住院时间 (43.9 vs. 5.6 天，p <0.05)。 结论: 本研究证明了气道和喂养困难对RS患者发病率的实质性影响，特别是在住院时间和入住ICU方面。该信息具有预后价值，可能有助于为该复杂患者群体生成管理算法。
METHODS:BACKGROUND:The anterior oronasal fistulae neighboring the alveolar cleft could persist or reappear after the alveolar reconstruction with cancellous bone grafting. The persistent symptomatic anterior oronasal fistulae need to be repaired, but surgery remains a challenge in cleft care. Surprisingly, this issue has rarely been reported in the literature. The purpose of this long-term study was to report a single surgeon experience with a therapeutic protocol for persistent symptomatic anterior oronasal fistula repair. METHODS:This is a retrospective study of consecutive patients with Veau type III and IV clefts and persistent symptomatic anterior oronasal fistulae managed according to a therapeutic protocol from 1997 to 2018. Depending on fistula size, patients were treated with local flaps associated with an interpositional graft or two-stage tongue flaps (small/medium or large fistulae, respectively). The surgical outcomes were classified as "good" (complete fistula closure with no symptoms), "fair" (asymptomatic narrow fistula remained), or "poor" (failure with persistent symptoms). RESULTS:Forty-four patients with persistent symptomatic anterior oronasal fistulae were reconstructed with local flaps associated with interpositional fascia or dermal fat grafting (52.3%) or two-stage tongue flaps (47.7%). Most of patients (93.2%) presented "good" outcomes, ranging from 87% to 100% (local and tongue flaps, respectively). Three (6.8%) patients presented symptomatic residual fistula ("poor" outcomes). CONCLUSIONS:For the repair of persistent symptomatic anterior oronasal fistulae, this therapeutic protocol provided satisfactory outcome with low fistula recurrence rate.
METHODS:OBJECTIVE:Methadone is a vital treatment for women with opioid use disorder in pregnancy. Previous reports suggested an association between methadone exposure and Pierre Robin sequence (PRS), a rare craniofacial anomaly. We assessed the association between gestational methadone exposure and PRS. DESIGN/SETTING:This case-malformed control study used European Surveillance of Congenital Anomalies population-based registries in Ireland, the Netherlands, Italy, Switzerland, Croatia, Malta, Portugal, Germany, Wales, Norway and Spain, 1995-2011. PATIENTS:Cases included PRS based on International Classification of Disease (ICD), Ninth Edition-British Paediatric Association (BPA) code 75 603 or ICD, Tenth Edition-BPA code Q8708. Malformed controls were all non-PRS anomalies, excluding genetic conditions, among live births, fetal deaths from 20 weeks' gestation and terminations of pregnancy for fetal anomalies. An exploratory analysis assessed the association between methadone exposure and other congenital anomalies (CAs) excluding PRS. Methadone exposure was ascertained from medical records and maternal interview. RESULTS:Among 87 979 CA registrations, there were 127 methadone-exposed pregnancies and 336 PRS cases. There was an association between methadone exposure and PRS (OR adjusted for registry 12.3, 95% CI 5.7 to 26.8). In absolute terms, this association reflects a risk increase from approximately 1-12 cases per 10 000 births. A raised OR was found for cleft palate (adjusted OR 5.0, 95% CI 2.7 to 9.2). CONCLUSIONS:These findings suggest that gestational methadone exposure is associated with PRS. The association may be explained by unmeasured confounding factors. The small increased risk of PRS in itself does not alter the risk-benefit balance for gestational methadone use. The association with cleft palate, a more common CA, should be assessed with independent data.
METHODS::Orthopedic treatment to improve deficient maxillary growth of cleft lip and palate patients is an important part of treatment. The success of this treatment is strongly dependent on the time of initiation of therapy. There has been a large controversy in the available literature regarding the skeletal age of these patients. The aim of the present study was to compare the skeletal age of cleft lip and palate patients with normal individuals.37 unilateral and 14 bilateral cleft lip and palate patients and 47 healthy individuals participated in this cross-sectional study. The patients were classified into 8 to 10 and 11 to 14-year-old individuals. Cervical vertebral maturational stage of participants was evaluated in the lateral cephalometry. The skeletal age of cleft lip and palate patients was compared with normal controls. Chi-square was used for statistical analysis. There was not a significant difference in the skeletal developmental stage of unilateral and bilateral cleft compared to their normal peers according to their age and sex. Also, significant difference in skeletal maturational stage of cleft lip and palate patients was not found between boys and girls (P = 0.8). Similarly, no significant difference was found in the skeletal age of the 3 studied groups without considering the age and sex of participants (P = 0.5). Regarding the similar skeletal maturational stage of cleft lip and palate patients with normal controls in our study, their maxillofacial orthopedic treatment can be initiated at similar time to normal peers.