title: Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice.
abstract: OBJECTIVE
  Survivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis.
  
  
  METHODS
  A survey of follow-up practices at 16 centers within a population-based CDH network was performed. A descriptive analysis of outcomes (minimum 24 months postdischarge) of CDH survivors from the 2 largest centers was performed.
  
  
  RESULTS
  The nature of follow-up of CDH survivors was highly variable in 12 of 16 responding centers, ranging from ad hoc, community-based, and pediatrician-sponsored follow-up to a single perinatal center-based multispecialty CDH clinic. Outcomes at 24 to 36 months were reported from the 2 largest centers (n = 44). Among survivors, neurodevelopmental disability was most common (12/44; 27%) followed by gastrointestinal (9; 20.5%), pulmonary (5; 11.4%), musculoskeletal (5; 11.4%), and cardiac (2; 4.5%). Additional surgery was required in 17 patients (38.6%), including recurrent CDH repair in 7 (15.9%). Five patients (11.4%) had hearing loss. Among 41 children with available 24-month data, 32 (78%), 17 (41.5%), and 14 (34.1%) patients had weights below the 50th, 25th, and 3rd percentiles, respectively.
  
  
  CONCLUSIONS
  Congenital diaphragmatic hernia survivorship is associated with significant disability. Standardization of follow-up practices is essential to enable population-based outcomes analysis.
authors.affiliation: Division of Pediatric General Surgery, BC Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada. asafavi@cw.bc.ca
authors.forename: Arash
authors.initials: A
authors.lastname: Safavi
authors.forename: Anne R
authors.initials: AR
authors.lastname: Synnes
authors.forename: Karel
authors.initials: K
authors.lastname: O'Brien
authors.forename: Monping
authors.initials: M
authors.lastname: Chiang
authors.forename: Erik D
authors.initials: ED
authors.lastname: Skarsgard
authors.forename: Priscilla P L
authors.initials: PP
authors.lastname: Chiu
mesh_terms: D002170:Canada; D002658:Developmental Disabilities / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D005500:Follow-Up Studies; D005767:Gastrointestinal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D019538:Health Care Surveys; D034381:Hearing Loss / Q000453:epidemiology / Q000209:etiology; D006331:Heart Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D059685:Herniorrhaphy; D006801:Humans; D007231:Infant, Newborn; D008171:Lung Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D009140:Musculoskeletal Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D017063:Outcome Assessment, Health Care; D011183:Postoperative Complications / Q000175:diagnosis* / Q000453:epidemiology; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D012008:Recurrence; D012086:Reoperation / Q000706:statistics & numerical data; D011795:Surveys and Questionnaires; D017741:Survivors