Elective Caesarean Delivery in a Patient With May-Hegglin Anomaly: Its Concerns and Review of the Literature

May-Hegglin anomaly (MHA) is an autosomal dominant haematological disorder that is commonly misdiagnosed as idiopathic thrombocytopenic purpura. The presence of leukocyte inclusion bodies differentiates it from other causes of thrombocytopenia. There is a lack of agreement on the perioperative management of patients with MHA. Our patient was a 33-year-old lady with underlying MHA, gravida 2 para 1 at 37 weeks of pregnancy, who was electively admitted for lower-segment caesarean section under general anaesthesia. The caesarean delivery was uneventful, and the mother and baby were discharged without any complications from the operation. In this patient, an intra-abdominal drain was inserted to drain any postoperative intra-abdominal bleeding; however, the drain produced minimal haemoserous drainage, which was similarly observed in her first pregnancy. We performed a systematic search of the literature on MHA and discussed the concerns of the anaesthetic, obstetric, and paediatric teams. We only found 96 pregnancies by 56 mothers with MHA and 51% (n = 49) of these pregnancies were via vaginal delivery and 43.8% (n = 42) were delivered via caesarean sections. About 85.4% (n = 82) of the 96 pregnancies had an uneventful delivery. Only one patient in the postpartum haemorrhage group was considered likely to be attributed to MHA. None of the mothers who received neuraxial anaesthesia in our literature search experienced any epidural-spinal haematoma. There were 103 infants delivered, and 85.4% (n = 88) of them had no associated complications at birth. The rest of the fetal complications were not considered to be associated with MHA. The literature suggests that mothers with MHA generally have an uneventful delivery via all methods.