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[Bilateral diaphragmatic hernia]
F Rivilla Parra1, J Murcia Zorita, A Torres Nieto
1Departamento de Cirugía Pediátrica, Facultad de Medicina, Universidad Autónoma de Madrid.
Insights
Bilateral diaphragmatic hernia is a rare condition causing severe pulmonary hypoplasia and early death in infants. This study reviews six cases, highlighting associated malformations and poor outcomes.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Congenital Malformations
Context:
- Review of six bilateral diaphragmatic hernia cases over 20 years (1966-1986) at a single institution.
- Focus on early-onset symptoms and high mortality rates in affected neonates.
- Investigation into associated congenital anomalies impacting survival.
Purpose:
- To describe the clinical presentation and outcomes of bilateral congenital diaphragmatic hernia.
- To analyze the impact of associated malformations on patient survival.
- To review obstetrical history and patient evolution in these rare cases.
Summary:
- Six cases of bilateral diaphragmatic hernia presented with early-onset symptoms.
- Five patients died shortly after birth due to severe pulmonary hypoplasia and multiple congenital anomalies.
- One patient survived for 22 days, underscoring the critical condition.
Impact:
- Highlights the extremely poor prognosis of bilateral diaphragmatic hernia.
- Emphasizes the critical role of associated anomalies in neonatal outcomes.
- Informs clinical management and genetic counseling for congenital diaphragmatic hernia.
Abstract:
Six cases of bilateral diaphragmatic hernia are described between 145 congenital diaphragmatic hernia in Department of Pediatric Surgery, "La Paz" Children's Hospital of Madrid since 1966 to 1986. All patients showed symptoms early after birth. Death occurred almost immediately in five cases, for the severe bilateral pulmonary hypoplasia and the malformations associated in the most them (cardiac, genitourinary, digestive system and metabolic). One patient was alive for 22 days. We study the obstetrical history and the evolution of our patients.