Buonpane Christie, Lautz Timothy B, Hu Yue-Yung
Division of Pediatric Surgery, Ann & Robert H. Lurie Children's Hospital, Northwestern University Feinberg School of Medicine.
Division of Pediatric Surgery, Ann & Robert H. Lurie Children's Hospital, Northwestern University Feinberg School of Medicine; Surgical Outcomes and Quality Improvement Center, Department of Surgery, Northwestern University Feinberg School of Medicine.
J Pediatr Surg. 2019 Jun;54(6):1164-1167. doi: 10.1016/j.jpedsurg.2019.02.036. Epub 2019 Mar 1.
Meconium plug syndrome (MPS) is associated with Hirschsprung disease (HD) in 13-38% of cases. This study sought to assess institutional variation in utilization of rectal biopsy in children with MPS and the likelihood of diagnosing HD in this population.
Patients with MPS on contrast enema in the first 30 days of life from the Pediatric Health Information System database in 2016-2017 were included. Institutional rates of rectal biopsies performed during the initial admission were calculated and then used to predict institutional rates of early HD diagnoses using Poisson regression.
Of 373 newborns with MPS, 106 (28.4%) underwent early rectal biopsy, of whom 43 (40.5%) had HD. Fifty-seven (15.3%) were ultimately diagnosed with HD. Eight (14%) of these patients had a delayed diagnosis. HD rates between institutions did not differ significantly (range 0-50%, p=0.52), but usage of early rectal biopsy did (range 0-80%, p=0.03). Each additional early biopsy increased the early HD diagnosis rate by 35% (β=0.30, 95% CI 0.15-0.45, p<0.0001).
The incidence of HD is increased in children with MPS. There is significant hospital variability in the utilization of early rectal biopsy, and opportunity exists to standardize practice.
Study of Diagnostic test Level of Evidence: Level III.
胎粪栓综合征（MPS）在13% - 38%的病例中与先天性巨结肠（HD）相关。本研究旨在评估MPS患儿直肠活检应用的机构差异以及该人群中HD的诊断可能性。
纳入2016 - 2017年儿科健康信息系统数据库中出生后30天内进行造影灌肠的MPS患者。计算初次住院期间进行直肠活检的机构率，然后使用泊松回归预测早期HD诊断的机构率。
373例MPS新生儿中，106例（28.4%）接受了早期直肠活检，其中43例（40.5%）患有HD。57例（15.3%）最终被诊断为HD。这些患者中有8例（14%）诊断延迟。各机构之间的HD发生率无显著差异（范围0 - 50%，p = 0.52），但早期直肠活检的应用情况有差异（范围0 - 80%，p = 0.03）。每增加一次早期活检，早期HD诊断率提高35%（β = 0.30，95%CI 0.15 - 0.45，p < 0.0001）。
MPS患儿HD的发生率增加。早期直肠活检的应用在医院之间存在显著差异，有机会实现实践标准化。
诊断试验研究 证据水平：III级
