LB00.0

ICD-11 MMS 2026-01

Congenital diaphragmatic hernia

Description

Congenital diaphragmatic hernia is a posterolateral defect of the diaphragm that allows passage of abdominal viscera into the thorax, leading to respiratory insufficiency and persistent pulmonary hypertension with high mortality.

Index terms

  • Congenital diaphragmatic hernia
  • congenital diaphragm defect with hernia
  • congenital diaphragm hernia
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  • gross congenital diaphragm defect
  • Bochdalek hernia

Excludes

  • LB13.1 – Congenital hiatus hernia

Exclusions from above levels

  • LD2F.10 – Prune belly syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LB00.1 – Absence of diaphragm
  • LB00.Y – Other specified structural developmental anomalies of diaphragm
  • LB00.Z – Structural developmental anomalies of diaphragm, unspecified

Foundation entity

Congenital diaphragmatic hernia – http://id.who.int/icd/entity/1414428936

Part of: LB00: Structural developmental anomalies of diaphragm › 20: Developmental anomalies