A Rare Case of Chiai II Malformation with Uterine Rupture at a Regional Hospital in Guyana

Abstract

Background: Chiari II malformation is a congenital hindbrain anomaly that is commonly associated with neural tube defects and can present substantial prenatal and perinatal management challenges. This report describes an unusual co-occurrence of Chiari II malformation and rupture of an unscarred uterus. Case Presentation: A 26-year-old woman, G3P2001, was admitted at 40 weeks + 6 days of gestation for delivery. She had attended 10 antenatal visits and had undergone one ultrasound examination at 17+0 weeks, which reported no congenital abnormality. She presented in spontaneous labour at 5 cm cervical dilatation with spontaneous rupture of membranes and a breech presentation. Emergency caesarean delivery resulted in the birth of a live male neonate weighing 5.3 kg, with Apgar scores of 2 and 2. The neonate had hydrocephalus, clubfoot, spina bifida, and meningiomylocele. Intraoperative examination identified an 8 cm rupture of the unscarred uterus extending from the lower segment to the fundus. Hysterorrhaphy and B-Lynch compression sutures achieved haemostasis; estimated blood loss was 450 mL. The neonate died after 100 minutes. Post-mortem findings included cerebral atrophy, congenital hydrocephaly consistent with Arnold-Chiari type II malformation, and meningiomylocele. Conclusion: This case highlights the importance of accurate prenatal detection, counselling, multidisciplinary planning, and prompt obstetric management when severe fetal anomalies and unexpected maternal complications occur.