Journal of Perinatology (2012) 32, 304–305 r 2012 Nature America, Inc. All rights reserved. 0743-8346/12 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION Subgaleal hemorrhage in a neonate with factor X deficiency following a non-traumatic cesarean section

EA Wetzel and PS Kingma The Perinatal Institute, Section of Neonatology, Perinatal and Pulmonary Biology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

deliveries.6 The incidence drops markedly to 4:10 000 in This case report describes a term infant born by a non-traumatic, non- spontaneous vaginal deliveries and is rarely reported in non- instrumented cesarean section that presented with respiratory failure and traumatic cesarean sections. There is literature reporting an severe metabolic acidosis secondary to subgaleal hemorrhage (SGH). association between intracranial hemorrhage and Further evaluation revealed a functional factor X deficiency that was disorders, but the association between SGH and bleeding disorders initially treated with fresh frozen plasma infusions. This report is significant is difficult to measure, because reports on this topic are rare and for the occurrence of a SGH in a non-traumatic delivery and emphasizes the most literature combines SGH and cephalhematoma into a single importance of obtaining a evaluation in patients with similar category of extracranial hemorrhage.7,8 Here we report a case of a presentations. In addition, this case suggests that the mechanism of injury term infant born by non-traumatic cesarean section that developed that causes SGH may occur more frequently than previously thought, but a SGH and was found to have a bleeding disorder associated with a does not become clinically significant in patients without an underlying mutation in factor X. coagulopathy. Journal of Perinatology (2012) 32, 304–305; doi:10.1038/jp.2011.122 Case Keywords: factor X; subgaleal hemorrhage; subgaleal neonate; hemophilia and intracranial hematoma; hemophilia and The patient is a 39 þ 1 week gestation male, born to a 30-year-old subgaleal hematoma gravida 2 para 2. Maternal prenatal labs were normal, and maternal medications included only a prenatal vitamin. The delivery was a non-traumatic cesarean section for failure to Introduction progress after 2 h of active stage 1 labor. No vacuum or forceps A subgaleal hemorrhage (SGH) occurs when shearing forces are were used. Apgars were 7 at 1 min and 9 at 5 min. Birth weight was applied to the scalp during delivery, and large emissary veins in the 3200 g (40th percentile), length 53 cm (90th percentile) and subgaleal space sever or rupture resulting in the accumulation of head circumference 35 cm (50th percentile). The infant blood between the epicranial aponeurosis and the periosteum. The received vitamin K and was doing well until 16 h of life, when loose connective tissue of the subgaleal space can accommodate up the physician was called due to an infant temperature of 1 to 260 ml of blood in a typical term infant.1 Symptoms can include 94.6 F, pallor and increased occipital swelling. Physical respiratory distress, anemia, hypovolemic shock, pallor, exam at that time was significant for a boggy occiput without hypotension, acidosis and . SGH is much more common fluctuation and a soft fontanel. in vacuum and forceps deliveries, but may occur spontaneously, On admission to the special care nursery, he was noted to be and mortality is reported as high as 12 to 25%.2–5 As blood loss grunting and pale, with a prolonged capillary refill. The capillary can be massive before is recognized, SGH can be a blood gas 17.5 h after birth was as follows: pH 7.3, pCO2 18 mm Hg devastating and potentially fatal result of . The risk and base deficit À14, with a hematocrit of 20. An umbilical venous factors of SGH include macrosomia, prolonged second stage of catheter was placed. He was intubated and given sodium labor, fetal distress and use of vacuum or forceps with instrument bicarbonate and a packed red blood cell transfusion. Follow-up gas assisted deliveries, contributing to an incidence of 40 to 60/10 000 was pH 6.7, pC02 46 mm Hg and a base deficit À30, at which time he received additional sodium bicarbonate, a normal saline bolus, Correspondence: Dr PS Kingma, The Perinatal Institute, Section of Neonatology, Perinatal and was transferred to a level III neonatal intensive care unit at and Pulmonary Biology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave. 18 h of life. Following transfer, he received additional packed red ML7009, Cincinnati, OH 45229-3039, USA. E-mail: [email protected] blood cells, fresh frozen plasma, sodium bicarbonate, calcium Received 19 April 2011; revised 28 July 2011; accepted 28 July 2011 gluconate and a stat head ultrasound. Head ultrasound revealed a Subgaleal hemorrhage after non-traumatic delivery EA Wetzel and PS Kingma 305

despite a non-traumatic delivery. In addition, this case demonstrates that SGH in a non-instrumented delivery should prompt an evaluation for bleeding disorders. Although there are rare case reports of SGH in patients with hemophilia, our patient was unique in that his coagulopathy was due to a rare autosomal recessive deficiency in factor X that occurs in 1:1 000 000 people.9,10 As the current patient had no known risk factors for SGH (other than the brief period of failure to progress labor), it is likely that his SGH only became clinically significant due to his bleeding disorder. Traditionally, the mechanism of injury in SGH (i.e. the rupture or shearing of an emissary vein during delivery) was thought to be initiated primarily by vacuum- or forceps- assisted delivery, and typically resulted in devastating extracranial hemorrhage. The current case suggests that the mechanism of SGH may occur more frequently and without inciting factors. In Figure 1 The response in the international normalized ratio (INR) was followed addition, the degree of venous tearing in SGH may be potentially serially during the first 11 days of life. Arrows indicate when fresh frozen plasma variable such that the smaller tears and subsequent smaller (FFP) was administered. hemorrhages do not become clinically significant in patients without an underlying coagulopathy. SGH. He was also found to have bilateral pneumothoracies requiring a right chest tube. Labs were significant for a coagulopathy (prothrombin time 50.8 partial thromboplastin time Conflict of interest 63.6 international normalized ratio (INR) 5.02), which persisted The authors declare no conflict of interest. throughout his initial hospital course and required multiple fresh frozen plasma infusions. Family history was negative for bleeding disorders. Hematology was consulted to evaluate, and References factor levels were obtained. His factor X level was 4%, indicating a moderate quantitative deficiency in factor X. He was continued on 1 Davis DJ. Neonatal subgaleal hemorrhage: diagnosis and management. CMAJ 2001; 164(10): 1452–1453. multiple fresh frozen plasma infusions and his coagulopathy 2 Chang HY, Peng CC, Kao HA, Hsu CH, Hung HY, Chang JH. Neonatal subgaleal improved (Figure 1). He was subsequently transitioned to factor IX hemorrhage: clinical presentation, treatment, and predictors of poor prognosis. Pediatr complex infusions for long-term therapy. His SGH was shown to be Int 2007; 49(6): 903–907. resolving on repeated ultrasounds, and by discharge, his factor X 3 Doumouchtsis SK, Arulkumaran S. Head trauma after instrumental births. levels were greater than 60% post factor IX complex infusions, with Clin Perinatol 2008; 35(1): 69–83, viii. 4 Gebremariam A. Subgaleal haemorrhage: risk factors and neurological and factor 2 levels of 145%. developmental outcome in survivors. Ann Trop Paediatr 1999; 19(1): 45–50. 5 Christensen RD, Baer VL, Henry E. Neonatal subgaleal hemorrhage in a multihospital healthcare system: prevalence, associations, and outcomes. e-J Neonatol Discussion Res 2011; 1(1): 1–8. SGHs can be significant and life threatening, with mortality as 6 Plauche WC. Subgaleal hematoma. A complication of instrumental delivery. JAMA high as 25%.2–6 SGH is more common in vacuum and forceps 1980; 244(14): 1597–1598. 7 Kulkarni R, Lusher J. Perinatal management of newborns with haemophilia. assisted deliveries, although there are reports of SGH in Br J Haematol 2001; 112(2): 264–274. spontaneous vaginal deliveries associated with macrosomia, fetal 8 Kulkarni R, Lusher JM. Intracranial and extracranial hemorrhages in newborns distress or prolonged second stage of labor.4,6 In the current case, with hemophilia: a review of the literature. J Pediatr Hematol Oncol 1999; 21(4): SGH occurred in an infant with a non-traumatic cesarean delivery 289–295. with no known associated-risk factors. Although SGH is often 9 Rohyans JA, Miser AW, Miser JS. Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature. 1982; 70(2): considered in traumatic or instrumented deliveries, our patient 306–307. illustrates the important lesson that SGH should also be part of the 10 Menegatti M, Peyvandi F. Factor X deficiency. Semin Thromb Hemost 2009; 35(4): differential diagnosis in infants with severe acidosis and shock, 407–415.

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