February 2013

A premature female infant delivered at 25 weeks gestation

David Howell M.D. and Jimmie SmithM.D.

Clinical History

The patient was a three day old female infant who was delivered at 25 weeks gestational age to a 33 year-old G1P0 woman. The mother presented to clinic with a clear vaginal discharge. Examination was significant for a dilated cervix and a bulging amnionic sac. Steroids and antibiotics were administered. Magnesium sulfate was also given to delay progression of labor. Despite these efforts, labor progressed. A female infant was delivered vaginally in the breech presentation. The birth weight was less than 1,000 grams. Due to respiratory distress, the infant was intubated and surfactant was administered. Vascular access was obtained by catheters placed into the umbilical vessels. Initial pertinent labs included: hemoglobin, 14 g/dL; platelet count, 232/uL; and a total bilirubin, 3.2 mg/dL. The infant was O positive and had a negative direct antiglobulin test (DAT). The infant had multiple episodes of bradycardia, hypotension and decreased oxygen saturations which responded to volume resuscitation. On the third day of life, her condition worsened and the hypotension no longer responded to resuscitative efforts. The abdomen was noted to be firm and distended. An abdominal radiograph was negative for free air. She developed metabolic acidosis, was noted to be pale and required vasopressors to maintain her blood pressure. Subsequent laboratory values noted included: hemoglobin, 4.4 g/dL; platelet count, 81/uL; PT, 12.0 seconds; INR, 1.2; PTT, 75.3 seconds; fibrinogen, < 70 mg/dL; AST, 239 U/L; LDH, 1213 U/L; total bilirubin, 2.6 mg/dL; and pH, 6.8. A significant drop in hemoglobin was noted along with evidence of coagulopathy. Red blood cells, platelets, cryoprecipitate and sodium bicarbonate were administered.

Gross Description

Representative image from ultrasounds of the abdomen (Figure 1) and the head are shown (Figure 2). The patient’s condition continued to decline despite therapy. Given the overall very poor prognosis and after discussions with her parents, supportive therapies were withdrawn. The infant expired and subsequently an autopsy was performed.

The autopsy results revealed a large for gestational age, premature female infant. Grossly, there was a lesion in the anterior aspect of the liver (Figures 3A and 3B). Hematoxylin and eosin stained sections of the liver lesion are shown (Figures 3C and 3D). An additional finding was cerebral intraventricular hemorrhage (Figure 4). Hyaline membrane disease of the lungs was also present.

fig1.jpg (3)
Figure 1. Longitudinal abdominal ultrasound showing the right lobe of the liver, right kidney (Kid) and right adrenal (Ad). Anterior is toward the left and rostral is toward the top of the image. A large and nearly anechoic lesion is seen on the anterior surface of the right lobe of the liver.
fig2.jpg (3)
Figure 2. Ultrasound of the head through the central aspect of the cerebral hemispheres at the level of the lateral ventricles (LV).
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Figure 3. A. Intact anterior surface of the liver. B. Cut surface of the liver lesion. The anterior surface is toward the top of the image. Hematoxylin and eosin stained sections of the liver lesion are shown in images C and D. The capsular surface is toward the left of the image (panel C) and toward the top of the image (panel D).
fig4.jpg (3)
Figure 4. Coronal section through the central aspect of the cerebral hemispheres at the level of the hippocampus (arrows). The lateral ventricles are designated with an asterisk (*).

Diagnosis

Which of the following is most likely true regarding the liver lesion ?

Answer

Please select an answer above.

Discussion

The case history describes a very typical history for the majority of pediatric subcapsular hepatic hematomas. A subcapsular hepatic hematoma is a collection of blood between the liver parenchyma and Glisson’s capsule. Hepatic subcapsular hematomas are rare beyond the perinatal period but are relatively common lesions in fetuses and neonates [1]. Birth trauma is the major association with the development of this lesion in the neonatal period; however, coagulopathy, sepsis, umbilical vein catheterization, and hypoxia are all associated with subcapsular hepatic hematomas in the neonatal period [1, 2, 3]. The incidence increases in very low birth weight infants and in babies delivered in breech presentation [4]. Their development is rare in term, healthy infants. The development of subcapsular hepatic hematomas in older individuals is most frequently associated with blunt trauma and has been rarely diagnosed as a complication of endoscopic retrograde cholangiopancreatography, cholecystectomy, and extracorporeal shock wave lithotripsy [5, 6, 7]. The case presented in the current study has numerous findings that are associated with subcapsular hepatic hematomas.

Neonatal subcapsular hepatic hematomas are associated with significant morbidity and mortality because they are frequently not identified clinically and are instead recognized at autopsy [1]. Rapid bleeding results in hypovolemia and shock while slow expansion of the hematoma can cause abdominal discoloration, anemia, jaundice, and even an abdominal mass. Subcapsular hematomas are not associated with primary neonatal hepatic tumors; however, the lesion can mimic these tumors [3]. Ultrasonography is the imaging modality of choice for the diagnosis of subcapsular hepatic hematomas. Hemangiomas are the most common tumor of the liver in the neonate; however, most hemangiomas have a distinct appearance on ultrasound that is distinct from that of subcapsular hematoma [8]. Hepatic hemangiomas can have a hemorrhagic component, although the hemorrhage usually consists of an intraparenchymal hemorrhage and is rarely limited to the subcapsular region. Hepatoblastomas, the most common malignant primary liver tumor in the neonate, can be more difficult to differentiate from a subcapsular hematoma [3, 9]. A hepatoblastoma can rupture during delivery, resulting in a hematoma. Like hemangiomas, the bleeding is usually located in the hepatic parenchyma and is rarely limited to the subcapsular region. Hepatoblastomas are more frequently encountered in premature infants of low birth weight [10]. Hepatoblastomas can be identified on prenatal ultrasounds. Virtually all hepatoblastomas in older children have an elevation of alpha fetoprotein (AFP). However, only 50% of neonates with a hepatoblastoma will have an elevation of AFP [8]. Additionally, elevation of AFP has been reported in two cases of subcapsular hepatic hematomas through an unknown mechanism [11]. A potentially fatal complication of both lesions is capsule rupture resulting in intra-abdominal hemorrhage. Therefore, cesarean delivery is recommended when a prenatal ultrasound identifies a hepatic tumor to decrease the likelihood of tumor rupture. The liver lesion in this cases consisted only of clotted blood that was confined to the anterior subcapsular region on the liver (Figure 3).

The autopsy findings in the current study also identified a grade III germinal matrix hemorrhage in which blood fills and dilates the ventricles of the brain (Figures 2 and 4). Bleeding in grade I hemorrhages is limited to the germinal matrix. Grade II hemorrhage involves, but does not dilate the ventricular system. Grade IV hemorrhage involves the brain parenchyma. Like subcapsular hepatic hematomas, germinal matrix hemorrhages are associated with preterm infants and are frequently encountered in very low birth weight infants [12]. Geminal matrix hemorrhages arise due to the inherent fragility of the blood vessels in the germinal matrix, changes in cerebral blood flow, and platelet or coagulation disorders [12]. A germinal matrix hemorrhage is also identified in approximately 35% of infants with subcapsular hepatic hematomas [13]. Both of these lesions are a primary complication of prematurity and may result in significant morbidity and mortality.

The mainstay of treatment for both subcapsular hepatic hematomas and cerebral intraventricular hemorrhages is supportive medical therapy. These measures include blood transfusions, correction of coagulopathy if present, volume resuscitation and ventilatory support. Surgery is not recommended as a first line treatment of subcapsular hepatic hematomas and should be limited to cases where supportive measures are not successful or in cases of capsule rupture. A major complication of intraventricular hemorrhages is hydrocephalus. Decompression with serial lumbar punctures is no longer recommended. Acetazolamide may offer some benefit through decreased cerebrospinal fluid production. The definitive treatment of hydrocephalus in the setting of intraventricular hemorrhage involves the surgical creation of a shunt. Significant morbidity and mortality remain problematic even with awareness of these entities.

References

  1. Singer DB, et al. Hepatic subcapsular hematomas in fetuses and neonatal infants. Pediatr Dev Pathol. 1999. 2:215-20.
  2. Anjay MA, Sasidharan CK, Anoop P. Hepatic subcapsular hematoma: two neonates with disparate presentations. Pediatr Neonatol. 2012. 53:144-6.
  3. Mouratidis B, Antonio G. Sonographic diagnosis of subcapsular liver hematoma mimicking tumor in a neonate. J Clin Ultrasound. 2000. 28:53-7.
  4. Ahn HS, Chang YW, Lee DW, Kwon KH, Yang SB. An incidentally detected hepatic subcapsular hematoma in a very low birth weight newborn: a case report. Cases J. 2010. 3:32-6.
  5. Orellana F, et al. Subcapsular hepatic hematoma post ERCP: a rare or an underdiagnosed complication? Endoscopy. 2012. 44:108-9.
  6. Hansen AJ, et al. Large subcapsular liver hematoma following single-incision laparoscopic cholecystectomy. JSLS. 2011. 15:114-6.
  7. Ng CF, et al. Hepatic hematoma after shockwave lithotripsy for renal stones. Urol Res. 2012. 40:785-9.
  8. Isaacs H. Fetal and neonatal hepatic tumors. J Pediatr Surg. 2007. 42:1797-803.
  9. Lam CY, Chan KF, Fan TW, Kwok CH, Chan CH, Tsang TK. Intrahepatic hematoma: hepatic lesion in a newborn with high alpha-fetoprotein level. Pediatr Radiol. 2005. 35:1139-41.
  10. Kapfer SA, Petruzzi MJ, Caty MG. Hepatoblastoma in low birth weight infants: an institutional review. Pediatr Surg Int. 2004. 20:753-6.
  11. Costa S, De Carolis MP, Savarese I, Lacerenza S, Romagnoli C. Hepatic hematoma in a neonate with a high level of alpha-fetoprotein. Eur J Pediatr. 2008. 167:591-3.
  12. Ballabh P. Intraventricular hemorrhage in premature infants: mechanism of disease. Pediatr Res. 2010. 67:1-8.
  13. Amodio J, Fefferman N, Rivera R, Pinkney L, Strubel N. Idiopathic intraparenchymal hematoma of the liver in a neonate. Pediatr Radiol. 2004. 34:358-61.