Solitary
Nonparasitic Hepatic Cyst Causing Abdominal Respiratory Distress in a Newborn
Distension
and
By Dimitri Merine, Anna FL Nussbaum, and Roger C. Sanders Baltimore,
Maryland
0 This is a report of e girl infant born with a protuberant abdomen and respiratory distress. A cystic mass was excised from the liver. 0 1990 by W.B. Saunders Company. INDEX WORDS: Liver cyst; neonatal abdominal cyst.
N
ONPARASITIC LIVER cysts are uncommon, and may be congenital or acquired in origin. The congenital cysts are thought to arise from obstruction of aberrant bile ducts, leading to stasis and retention.’ Unlike multiple liver cysts seen in association with polycystic kidney disease, solitary cysts represent a separate and distinct disease. We report a case of a large solitary liver cyst that caused abdominal distension and severe respiratory distress in a newborn. CASE REPORT A 3,420 g girl was born at 37 weeks’gestation to a 28-year-old woman by cesarean section. At birth, the infant was noted to have a large, protuberant abdomen, and respiratory distress that subsequently required intubation. Physical examination showed diffuse abdominal distension, but no discrete mass was palpable. Chest and abdominal radiographs demonstrated a large soft tissue mass that filled nearly the entire abdomen and displaced bowel gas to the left side; the lungs were moderately hypoinflated secondary to the abdominal mass (Fig 1). Sonography showed a huge cystic mass that filled nearly the entire abdomen and extended into the pelvis (Fig 2). The cyst was contiguous with, and flattened, the inferior margin of the right lobe of the liver, but there was no apparent hepatic parenchyma surrounding the cyst. The gallbladder and common hepatic duct were normal. The kidneys were intrinsically normal, but the right kidney was slightly compressed by the cyst. At surgery, a large soft, cystic mass measuring approximately 14 cm x 8 cm arose from the edge of the right lobe of the liver. The liver and gallbladder were otherwise normal, as were the remainder of the abdominal contents. The mass was easily excised from the liver; 400 mL of fluid was evacuated from the cyst. At the time of histologic examination, the cyst wall contained benign me-senchymal tissue with a rim of hepatic parenchyma. The infant had an uncomplicated recovery, and a repeat sonogram 1 year later showed normal hepatic parenchyma. DISCUSSION Intrahepatic cysts may be solitary or multiple. The solitary liver cyst is unassociated with cystic involvement of other viscera, whereas diffuse polycystic disease of the liver is frequently associated with cystic disease in other organs, most commonly the kidneys. The latter is considered part of the spectrum of adult polycystic renal disease.2 Solitary cysts may occur at any age, but most patients have been diagnosed in the fourth and fifth decades of life.’ Few cases have been Journal of Pediatric Surgery, Vol 25, No 3 (March), 1990: pp 349-350
Fig 1. Chestand abdominalroentgenogramrhowa a large soft tissuemass fillingnaarlythe entireabdomenand displacingbowel gas to the left side. The lungs are hypoinflatadsecondaryto the abdominalmass.
reported in the newborn perux13-’ Females are affected more often than males, with a 4:l ratio.’ The most common location is the right lobe of the liver.’ The majority of solitary liver cysts are asymptomatic and are first noted incidentally on radiologic evaluation performed for other reasons. When they occur, symptoms are generally due to compression of adjacent structures or hemorrhage into the cyst. Instances of torsion, strangulation, and rupture have also From the Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, Baltimore, MD. Address reprint requests to Anna R. Nussbaum. MD, Department of Radiology, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC 20010. Q 1990 by W.B. Saunders Company. 0022-3468/90/2503-0020$03.00/0
349
MERINE, NUSSBAUM. AND SANDERS
Fig 2. (ALLongitudinal and (B) transverse sonograms demonatrato a large, anechoic mass filling the entire right abdomen and crossing the midline. The cyst is contiguous with, end flatten% the inferior margin of the right lobe of the liver.
been reported. 6;1 Our case is unusual, in that the patient presented in the immediate postnatal period with respiratory distress secondary to marked abdominal distension. Cesarean section, performed for unrelated reasons, proved fortuitous as vaginal delivery may have been difficult. There has been such a case, in which a large hepatic cyst caused dystocia and interfered with delivery.’ In the newborn period, the most common considerations for a nonrenal cystic abdominal mass include ovarian cyst, mesenteric cyst, intestinal duplication cyst, and choledochal cyst. Although uncommon, our case indicatesthat a solitary liver cyst should be added to the above list of differential possibilities. An analysis of the pathologic and surgical features of 4itary liver cysts indicates that the sonographic findings displayed in this case should prove to be fairly typical. The solitary hepatic cyst usually arises from a broad base along the inferior surface of the liver, most commonly
in the right lobe.3 Occasionally, the cyst is attached by a narrow pedicle of liver tissue.3-5 In both circumstances, liver tissue does not encircle the cyst, making preoperative determination of a hepatic origin more difficult. Flattening of the inferior surface of the liver, as in our case, may prove to be of benefit in suggesting the hepatic origin of a cystic structure. It should be noted that a clearly intrahepatic location of a solitary cyst or replacement of ihe right lobe of the liver by the cyst has been reported in infants.3 The cyst is multilocular in approximately 10% of cases, and a septated cyst has been seen sonographically in an infant.4’5 Management of symptomatic hepatic cysts is variable. Aspiration or drainage, injection of the cyst with sclerosing agents, marsupialization, and excision have all been used.* Recurrences are less likely to occur with the latter two methods. Emergent surgery and excision of the large cyst, in the present case, resulted in prompt resolution of respiratory compromise.
REFERENCES 1. Geist DC: Solitary nonparasitic cyst of the liver. Arch Surg 71:867-880, 1955 2. Jones WL, Mountain JC, Warren KW: Symptomatic nonparasiticcystsof theliver. Br JSuq61:118-123, 1974 3. Johnston PW: Congenital cysts of the liver in infancy and childhood. Am J Surg 116:184-191,1968 4. Athey PA, Lauderman JA, King VE: Massive congenital solitary nonparasitic cyst of the liver in infancy. J Ultrasound Med 5:585-587,1986
5. Sabuo RM, Belsare RK, Narang R, et al: Giant congenital cyst of the liver. J Pediatr Surg 9561-562, 1974 6. Sood SC, Watson A: Solitary cyst of the liver presenting as an abdominal emergency. Postgrad Med J 50:48-49.1974 7. Flagg RS, Robinson DW: Solitary nonparasitic hepatic cysts. Arch Surg 95:964-973,1967 8. Lin TY, Chen CC, Wang SM: Treatment of non-parasitic cystic disease of the liver: A new approach to therapy with polycystic liver. Ann Surg 168:921-927,1968
Nonparasitic Hepatic Cyst Causing Abdominal Respiratory Distress in a Newborn
Distension
and
By Dimitri Merine, Anna FL Nussbaum, and Roger C. Sanders Baltimore,
Maryland
0 This is a report of e girl infant born with a protuberant abdomen and respiratory distress. A cystic mass was excised from the liver. 0 1990 by W.B. Saunders Company. INDEX WORDS: Liver cyst; neonatal abdominal cyst.
N
ONPARASITIC LIVER cysts are uncommon, and may be congenital or acquired in origin. The congenital cysts are thought to arise from obstruction of aberrant bile ducts, leading to stasis and retention.’ Unlike multiple liver cysts seen in association with polycystic kidney disease, solitary cysts represent a separate and distinct disease. We report a case of a large solitary liver cyst that caused abdominal distension and severe respiratory distress in a newborn. CASE REPORT A 3,420 g girl was born at 37 weeks’gestation to a 28-year-old woman by cesarean section. At birth, the infant was noted to have a large, protuberant abdomen, and respiratory distress that subsequently required intubation. Physical examination showed diffuse abdominal distension, but no discrete mass was palpable. Chest and abdominal radiographs demonstrated a large soft tissue mass that filled nearly the entire abdomen and displaced bowel gas to the left side; the lungs were moderately hypoinflated secondary to the abdominal mass (Fig 1). Sonography showed a huge cystic mass that filled nearly the entire abdomen and extended into the pelvis (Fig 2). The cyst was contiguous with, and flattened, the inferior margin of the right lobe of the liver, but there was no apparent hepatic parenchyma surrounding the cyst. The gallbladder and common hepatic duct were normal. The kidneys were intrinsically normal, but the right kidney was slightly compressed by the cyst. At surgery, a large soft, cystic mass measuring approximately 14 cm x 8 cm arose from the edge of the right lobe of the liver. The liver and gallbladder were otherwise normal, as were the remainder of the abdominal contents. The mass was easily excised from the liver; 400 mL of fluid was evacuated from the cyst. At the time of histologic examination, the cyst wall contained benign me-senchymal tissue with a rim of hepatic parenchyma. The infant had an uncomplicated recovery, and a repeat sonogram 1 year later showed normal hepatic parenchyma. DISCUSSION Intrahepatic cysts may be solitary or multiple. The solitary liver cyst is unassociated with cystic involvement of other viscera, whereas diffuse polycystic disease of the liver is frequently associated with cystic disease in other organs, most commonly the kidneys. The latter is considered part of the spectrum of adult polycystic renal disease.2 Solitary cysts may occur at any age, but most patients have been diagnosed in the fourth and fifth decades of life.’ Few cases have been Journal of Pediatric Surgery, Vol 25, No 3 (March), 1990: pp 349-350
Fig 1. Chestand abdominalroentgenogramrhowa a large soft tissuemass fillingnaarlythe entireabdomenand displacingbowel gas to the left side. The lungs are hypoinflatadsecondaryto the abdominalmass.
reported in the newborn perux13-’ Females are affected more often than males, with a 4:l ratio.’ The most common location is the right lobe of the liver.’ The majority of solitary liver cysts are asymptomatic and are first noted incidentally on radiologic evaluation performed for other reasons. When they occur, symptoms are generally due to compression of adjacent structures or hemorrhage into the cyst. Instances of torsion, strangulation, and rupture have also From the Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, Baltimore, MD. Address reprint requests to Anna R. Nussbaum. MD, Department of Radiology, Children’s National Medical Center, 111 Michigan Ave NW, Washington, DC 20010. Q 1990 by W.B. Saunders Company. 0022-3468/90/2503-0020$03.00/0
349
MERINE, NUSSBAUM. AND SANDERS
Fig 2. (ALLongitudinal and (B) transverse sonograms demonatrato a large, anechoic mass filling the entire right abdomen and crossing the midline. The cyst is contiguous with, end flatten% the inferior margin of the right lobe of the liver.
been reported. 6;1 Our case is unusual, in that the patient presented in the immediate postnatal period with respiratory distress secondary to marked abdominal distension. Cesarean section, performed for unrelated reasons, proved fortuitous as vaginal delivery may have been difficult. There has been such a case, in which a large hepatic cyst caused dystocia and interfered with delivery.’ In the newborn period, the most common considerations for a nonrenal cystic abdominal mass include ovarian cyst, mesenteric cyst, intestinal duplication cyst, and choledochal cyst. Although uncommon, our case indicatesthat a solitary liver cyst should be added to the above list of differential possibilities. An analysis of the pathologic and surgical features of 4itary liver cysts indicates that the sonographic findings displayed in this case should prove to be fairly typical. The solitary hepatic cyst usually arises from a broad base along the inferior surface of the liver, most commonly
in the right lobe.3 Occasionally, the cyst is attached by a narrow pedicle of liver tissue.3-5 In both circumstances, liver tissue does not encircle the cyst, making preoperative determination of a hepatic origin more difficult. Flattening of the inferior surface of the liver, as in our case, may prove to be of benefit in suggesting the hepatic origin of a cystic structure. It should be noted that a clearly intrahepatic location of a solitary cyst or replacement of ihe right lobe of the liver by the cyst has been reported in infants.3 The cyst is multilocular in approximately 10% of cases, and a septated cyst has been seen sonographically in an infant.4’5 Management of symptomatic hepatic cysts is variable. Aspiration or drainage, injection of the cyst with sclerosing agents, marsupialization, and excision have all been used.* Recurrences are less likely to occur with the latter two methods. Emergent surgery and excision of the large cyst, in the present case, resulted in prompt resolution of respiratory compromise.
REFERENCES 1. Geist DC: Solitary nonparasitic cyst of the liver. Arch Surg 71:867-880, 1955 2. Jones WL, Mountain JC, Warren KW: Symptomatic nonparasiticcystsof theliver. Br JSuq61:118-123, 1974 3. Johnston PW: Congenital cysts of the liver in infancy and childhood. Am J Surg 116:184-191,1968 4. Athey PA, Lauderman JA, King VE: Massive congenital solitary nonparasitic cyst of the liver in infancy. J Ultrasound Med 5:585-587,1986
5. Sabuo RM, Belsare RK, Narang R, et al: Giant congenital cyst of the liver. J Pediatr Surg 9561-562, 1974 6. Sood SC, Watson A: Solitary cyst of the liver presenting as an abdominal emergency. Postgrad Med J 50:48-49.1974 7. Flagg RS, Robinson DW: Solitary nonparasitic hepatic cysts. Arch Surg 95:964-973,1967 8. Lin TY, Chen CC, Wang SM: Treatment of non-parasitic cystic disease of the liver: A new approach to therapy with polycystic liver. Ann Surg 168:921-927,1968
