Hepatic Subcapsular Hematoma: Two Neonates With Disparate Presentations

Subcapsular hematoma of the liver rarely occurs in neonates and the diagnosis is often missedor delayed. We report two babies who had this uncommon condition in the early neonatalperiod. In the first baby, the hematoma was associated with ventouse delivery and presentedwith abdominal distension and worsening jaundice. In contrast, the other baby was relativelywell, with progressive pallor as the only clinical finding. The former had no other identifiablerisk factors, whereas the latter was confirmed as having classical hemophilia. The literature isbriefly reviewed with regards to incidence, etiology, diagnosis and management. Awareness ofthis unusual entity coupled with a high index of suspicion is essential for early identificationand stabilization of such babies.

Copyright©2012, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rightsreserved.

1. Introduction

Collection of blood between the liver and its capsule isa rare manifestation of birth trauma. The diagnosis isfrequently made only at autopsy. In an analysis of 755perinatal autopsies by Singer et al. in 1999, hepaticsubcapsular hematomas were encountered in 21 neonates(2.8%).

We report two neonates who had this unusual condition,with varying etiologies and presentations.

2. Case Reports

2.1. Case 1

A 4-day-old male infant presented with poor feeding,lethargy and jaundice. He had been delivered by ventouseat term, following a prolonged second stage of labor. Heweighed 3600 g at birth and was well during the first 3 days.

Jaundice with rapid progression was noted from thethird day. Subsequently, he developed poor feeding, fastbreathing and abdominal distension. Examination revealeda sick, tachypnoeic baby with a heart rate of 180/minuteand a systolic blood pressure of 60 mmHg. He was pale anddeeply jaundiced, with abdominal distension and firmhepatomegaly. No other abdominal masses were palpableand bowel sounds were heard normally.

The hemoglobin level was 8 g/dL (80 g/L) with a normalplatelet count and coagulation profile. He had indirecthyperbilirubinemia (total bilirubin 17.5 mg/dL, direct1.6 mg/dL), with no evidence of hemolysis. Sepsis screenwas negative. Ultrasonogram of abdomen revealeda moderate-sized anechoic area separating the anterolateral edge of the right lobe of liver from its capsule, witha few internal echoes towards the dependent part, suggesting a subcapsular hematoma (Figure 1).

There was no history of bleeding diathesis in the family.The baby was managed with blood transfusion andsupportive care. The clinical condition improved gradually.The maximum measured dimensions of the hematoma were1.9
4.6 cm and serial ultrasonograms demonstratedresolution of the lesion. He recovered uneventfully and iscurrently under follow-up.

2.2. Case 2

A term neonate was born following uncomplicated pregnancy and delivery. He was noted to be lethargic and notfeeding well at 8 hours of life. Clinical examination showedmild pallor and otherwise normal systemic examination.Complete blood count done as a part of partial septicscreen showed hemoglobin of 13.1 g/dL (131 g/L) withnormal white cell count, platelets and blood film.

The baby continued to refuse feeds and had increasingpallor. Cranial ultrasonogram ruled out intracranialhemorrhage. He was started on intravenous 10% dextrose inaddition to first-line antibiotics. A repeat full blood countshowed a significant drop in hemoglobin to 9.2 g/dL.Coombs’ test was negative, serum bilirubin was withinnormal limits and blood film examination was unremarkable. An ultrasonogram of abdomen showed a curvilinearfluid collection with some echogenecity between the liver and its capsule, consistent with a subcapsular hematoma.The maximum recorded dimensions were 1.3×2.9 cm.

A subsequent coagulation screen showed a prolongedactivated partial thromboplastin time of 129 seconds(normal 35-90 seconds), normal prothrombin time of 16seconds (normal 13-17 seconds) and fibrinogen level of3.3 g/L (normal 1.5e3.5 g/L). Further investigations wereperformed to establish the cause for the bleeding diathesis. The factor VIII level was noted to be strikingly low at0.03 U/mL (3%; normal 1.00 U/mL±0.39), thus confirminga diagnosis of severe classical hemophilia. There wasno family history of bleeding disorder, which is the casein approximately one-third of patients with classicalhemophilia.

The baby was managed conservatively with cryoprecipitate, transfusion of factor VIII and packed red cells.Serial ultrasonograms demonstrated resolution of thehematoma and he is on hematological follow-up.

3. Discussion

The process of labor is a combination of tractional forces,torsion, compression and contractions. Injuries to theinfant resulting from mechanical forces during delivery arecategorized as birth trauma. Although there has beena considerable decline in their incidence followingadvances in obstetric management and reduced use ofinstrumentation, birth injuries continue to complicatemany deliveries. Subcapsular hematoma of liver isa collection of blood under the Glisson capsule. Thoughknown to occur in adults following blunt abdominal trauma,it is a very unusual manifestation of birth injury. This shouldbe distinguished from an intrahepatic hematoma, wherebleeding is usually less extensive and occurs within the liverparenchyma. Proposed predisposing factors for a subcapsular hematoma include traumatic labour, coagulopathies,prematurity, very low birth weight, hypoxia, sepsis, pneumothorax and umbilical venous catheterization.2Mostreported cases in the literature are in babies born earlywith very low birth weight and the condition is extremelyrare in term healthy neonates.

Acute massive bleeds can present in the immediateneonatal period with signs of hypovolemia and shock.Slowly progressing hematomas manifest with pallor, jaundice, irritability or respiratory distress. They can rarelypresent as abdominal masses without clinical signs ofbleeding and may mimic tumors.3Ultrasonography is theinvestigation of choice. It can delineate the lesion well,differentiate it from neoplasms, rule out rupture and aid inserial follow-up.

The neonatal liver has limited ability to achieve spontaneous hemostasis, because of the weaker parenchymalconnective tissue framework and poor contractility ofhepatic veins. In addition, compression of the thoracic cageduring delivery may lead to stretching and damage of thecoronary ligament of the liver, which is attached to theinferior surface of the diaphragm. In Case 1, presence ofbirth trauma may have augmented these inherent riskseven in the absence of other risk factors. We hypothesizethat the considerable negative pressure exerted by theventouse may lead to significant pressure changes in the body cavities and great veins. This, when transmitted tosolid organs like liver, can potentially cause hemorrhage.

Severe hemophilia is well known to present as bleedingin the neonatal period, most commonly as intracranialbleeds. Even in the absence of a positive family history,hemophilia should be suspected in neonates with unusualmajor bleeding manifestaions as new mutations account for30% of patients, as in Case 2. Although other forms ofbleeding like muscular hematoma following injections andbleeding from venepuncture sites have been reported,subcapsular hematoma of liver has seldom been identified asa manifestation of hemophilia in neonates.5,6Early diagnosisis essential as the subcapsular hematoma may progressand rupture and result in catastrophic hemorrhagic shock inthe absence of prompt factor VIII replacement therapy.

It is interesting to note that the two cases discussed herehad strikingly dissimilar clinical features. The presentationin Case 1 was as a sick neonate with jaundice and abdominal distension with hepatomegaly. In contrast, Case 2 wasa relatively stable baby with unexplained worsening palloralone as the clinical manifestation. Awareness of thevarying spectrum of severity and the diverse etiology willhelp in clinical suspicion of a hepatic subcapsular hematoma at birth.

Management is mainly conservative, including bloodtransfusion, correction of coagulopathies and avoidingexcessive handling of the baby. Surgery is reserved forrupture of the hematoma into the peritoneum. In view ofthe potential for life-threatening complications, timelysuspicion and use of appropriate investigative modalitiesare essential to minimize morbidity and mortality from thisrare entity.