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Congenital haemophilia A presenting with subgaleal and intracranial haemorrhage following instrumental delivery

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© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION The case is of a male infant born by vacuumassisted vaginal delivery at… Click to show full abstract

© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION The case is of a male infant born by vacuumassisted vaginal delivery at 38 weeks+3 days of gestation. The mother is a primigravida and was neither diabetic nor hypertensive. Her pregnancy course was uneventful. There was no family history of coagulopathy or bleeding disorder on the maternal or paternal side. She was presented to the delivery room with labour pain and rupture of membranes more than 18 hours before the delivery. She received one dose of penicillin G. At the delivery time, she was noted to have signs of fetal distress in the cardiotocography monitoring. On vaginal examination, the fetal head was located at +1 station. Kiwi ventouse was applied at the fetal flexion points, but it failed twice so it was removed. A silastic vacuum extractor was applied at the fetal flexion point. An episiotomy was done and the baby was delivered after 40 min from the fourth attempt. The baby was delivered in a good condition with a birth weight of 3030 g. Apgar score was 8 and 10 at 1 and 5 min, respectively. After delivery, the baby was examined by the neonatologist attending the delivery, who noted a scalp erosion and mild oozing controlled by pressure. At the age of 7 hours, the baby was examined by the postnatal neonatologist who noted a soft tissue parietotemporal head swelling (figure 1). Skull Xray was ordered and showed overlapped parietal bone over occipital bone with overlying soft tissue swelling. No bony fracture was noted. Complete blood count (CBC) was done and showed a haemoglobin (Hb) level of 125 g/L and platelet count of 234 10/L. At the age of 12 hours, the swelling was noted to be progressively increasing in size, so the baby was shifted to the neonatal intensive care unit (NICU) for further management. He remained haemodynamically stable during his postnatal and NICU stay. Head ultrasound was done in the NICU at the age of 14 hours and showed an extraaxial infratentorial haematoma at the left temporal region measuring 2.0 cm×1.4 cm with no midline shift. Subgaleal haematoma was also noted (figure 2). A followup CBC was done and showed a further drop in the Hb level to 97 g/L. Other CBC parameters including platelet count were normal. An urgent head CT scan was done at the age of 18 hours and showed bilateral subgaleal haematoma involving the temporal, parietal and occipital regions with evidence of left supratentorial bleed measuring 2.9 cm×1.4 cm. Another bleed was noted measuring 1.3 mm×0.6 mm in the left infratentorial region suggestive of tentorial subdural haematoma. No hydrocephalus, midline shift or mass effect was noted (figure 3). Given the obstetric history, sex of the baby and postnatal course, the neonatal team decided to investigate the baby for coagulopathy even with the absence of an index case in the family. Activated partial thromboplastin time was measured at that time and it was prolonged up to 102.8 s. Prothrombin time was 12.3 s, and the international normalised ratio was 1.2. Coagulation factor VIII was ordered, and it came back to be 0.4%, confirming the diagnosis of severe congenital haemophilia A. The baby underwent packed red blood cells transfusion once and fresh frozen plasma three Figure 1 Subgaleal haematoma involving the parietal (transverse arrow), temporal (vertical arrow) and occipital areas.

Keywords: time; haematoma; age hours; congenital haemophilia; delivery; baby

Journal Title: BMJ Case Reports
Year Published: 2022

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