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Solitary hepatic lymphangioma in a 22-day-old infant
Journal of Pediatric Surgery (2009) 44, E9–E11
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Solitary hepatic lymphangioma in a 22-day-old infant Kedar Singh Shahi a,⁎, Bhandari Geeta b , Prashant Rajput a a
Department of Surgery, U.F.H.T. Medical College, Haldwani (Nainital), India Department of Anaesthesiology, U.F.H.T. Medical College, Haldwani (Nainital), India
b
Received 23 December 2008; revised 2 April 2009; accepted 4 April 2009
Key words: Hepatic lymphangioma; Infant
Abstract Lymphangiomas are congenital malformations of the lymphatic system composed of dilated endothelial lined spaces of varying sizes containing lymph. Hepatic lymphangioma is a rare finding, exclusively present in children and adolescents. Most reported cases occur as part of diffuse lymphangiomatosis. Solitary lymphangioma of the liver in infants is extremely rare. This report describes a case of unilocular, solitary hepatic lymphangioma in a 22-day-old infant. © 2009 Elsevier Inc. All rights reserved.
Lymphangiomas are congenital malformations of the lymphatic system composed of dilated endothelial lined spaces of varying sizes containing lymph. Hepatic lymphangioma is a rare finding, exclusively present in children and adolescents. Most reported cases occur as part of diffuse lymphangiomatosis. Solitary lymphangioma of liver in infants is extremely rare. This report describes a case of unilocular, solitary hepatic lymphangioma in a 22-day-old infant.
1. Case report A 22-day-old male baby presented with progressive abdominal distension since birth. The baby was otherwise normal. There was no associated pain, fever, refusal to feed, vomiting, urinary symptoms, or alteration in bowel motions. The antenatal period and delivery were uneventful. Physical examination revealed weight of 4.5 kg. The abdomen was markedly distended, tense, and nontender. A fluid thrill was ⁎ Corresponding author. Tel.: +91 05946 234442, 09412906925 (Mobile); fax: +91 05946 234423. E-mail address: [email protected] (K.S. Shahi). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.04.022
noted, and the bowel sounds were normal. Plain abdominal radiographs were unremarkable. Abdominal ultrasonography revealed a huge cystic mass in the peritoneal cavity. Computed tomography of the abdomen demonstrated a large abdominopelvic hypodense cystic lesion with thin walls measuring 9.6 × 8 × 13 cm within the peritoneal cavity, displacing the bowel loops to the periphery and extending superiorly abutting the inferior surface of the liver. There were no definite septations or solid elements within the cyst (Fig. 1). Liver function tests showed the serum bilirubin increased, indirect (3.3 mg/dL), and alkaline phosphatase level elevated (771 U/L). At laparotomy, a huge unilocular cyst arising from the visceral surface of the right lobe of liver extending into whole abdominal cavity and pelvis was observed. The cyst contained straw colored fluid (Figs. 2 and 3). The gallbladder, extrahepatic biliary tree, and other intraabdominal organs were normal. Complete excision of the cyst was accomplished. Histological examination showed the mass had multiple thin-walled cystic spaces lined by a single layer of endothelial cells filled with brownish granular tissue and stroma infiltrated by lymphoid cells (Fig. 4). A diagnosis of cystic lymphamgioma was made. The postoperative period
E10
Fig. 1 Computerized tomography of abdomen showing the cystic lesion in the abdomen.
was uneventful. The patient was discharged on the fifth postoperative day. A repeat abdominal ultrasound study after 1 month was normal.
2. Discussion Hepatic lymph channels are derived from the space of Disse as well as from capillary leakage from the peribiliary plexus [1]. Hepatic lymphangioma may occur as a solitary mass or more commonly as multiple masses composed of dilated lymphatic channels containing proteinaceous fluid or blood [2]. Solitary hepatic lymphangioma is an extremely rare benign neoplasm characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma. Lymphangiomas of the liver may occur in isolation or in association with lymphangiomas of other viscera [3]. They are congenital malformations of the lymphatic system, considered as areas of localized lymphatic stasis due to blockage of regional
Fig. 2 Intraoperative finding: huge cyst arising from under surface of right lobe of liver.
K.S. Shahi et al.
Fig. 3
Opened cyst: unilocular cyst containing straw colored fluid.
lymphatic drainage [4]. Lymphangiomas most frequently involve the head and neck, followed by the extremities, trunk, and intraabdominal areas in decreasing order of frequency [5]. Less than 5% of lymphangiomas involve the abdomen, mediastinum, scrotum, bone, and extremities [5,6]. Reports of lymphangiomas solely involving the liver are extremely rare [7,8]. Most of the reported cases are in older children and adults [3,7,9]. To our knowledge, the previous youngest patient and only infant with a solitary hepatic lymphangioma reported in literature was aged 4 months [10]. Clinical presentation of hepatic lymphangiomas is nonspecific and is mainly because of hepatomegaly with vague abdominal pain. Intraabdominal lymphangiomas may grow slowly and present with compression symptoms [11]. Our patient presented with rapidly progressive abdominal distension. Operation was planned to avoid the possible complications such as rupture, hemorrhage, and compressive symptoms. Elevation in serum alkaline phosphatase and bilirubin levels were nonspecific and were not helpful in making the diagnosis.
Fig. 4 Dilated cystic lymphatic channels lined by a single layer of endothelial cells (hematoxylin-eosin stain ×40).
Solitary hepatic lymphangioma Preoperative diagnosis of hepatic lymphangiomas is difficult. Most cases are diagnosed at laparotomy as in our case [9,10]. The typical ultrasonography, computed tomography, and magnetic resonance imaging appearance of hepatic lymphangioma is that of a cystic or multicystic hepatic mass with internal septations [12-14]. Our case was different because it consisted of a huge unilocular cyst with no septations. This finding was confirmed at the time of laparotomy. Solitary hepatic lymphangioma is a benign curable disease in infants and children. It should be considered in the differential diagnosis of cystic intraabdominal mass in infants. Accurate preoperative diagnosis is extremely difficult, and surgical excision is the recommended treatment when diagnosis is in doubt and in symptomatic patients [3,9]. Recurrence has not been reported, and long-term prognosis is excellent.
E11
[3] [4] [5] [6]
[7] [8] [9]
[10] [11] [12]
References [1] Kanel GC, Korula J. General aspect of liver and liver diseases. Atlas of liver pathology. 4th ed. Philadelphia: Elsevier/Saunders; 2005. p. 5. [2] Anthony PP. Tumors; aetiology, epidemiology and pathology. In: McSween RNM, Butt AD, Portman BC, et al, editors. Pathology of
[13]
[14]
liver. 4th ed. Philadelphia: Churchill Livingstone/Elsevier; 2002. p. 799-850. Chan SC, Huang SF, Lee WC, et al. Solitary hepatic lymphangioma— a case report. Int J Clin Pract Suppl 2005;147:100-2. Godart S. Embryological significance of lymphangiomas. Arch Dis Child 1966;41:204-6. Hancock BJ, Dickens St-Vil D. Complications of lymphangiomas in children. J Pediatr Surg 1992;27:220-4. Wan YL, Lee TY, Hung CF, et al. Ultrasound and CT findings of a cecal lymphangioma presenting with intussusception. Eur J Radiol 1998;2:77-9. Koh CC, Sheu JC. Hepatic lymphangioma—a case report. Pediatr Surg Int 2000;16:515-6. Barnes PA, Thomas JL, Bernardino ME. Pitfalls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981;141:129-33. Stavropoulos M, Vagianos C, Scopa CD, et al. Solitary hepatic lymphangioma—a rare benign tumour: a case report HPB. Surgery 1994;8:33-6. Martin A, Nzegwu O, et al. Solitary hepatic lymphangioma in an infant. J Perinat Med 35 2007;35(2):164-5. Wan YL, Lee TY, Gau JS, et al. A rare abdominal cystic mass with unusual presentation. Postgrad Med J 1998;74:175-6. Barnes PA, Thomas JL, Bernardino ME. Pit-falls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981;141:129-33. Blumhagen JD, Wood BJ, Rosenbaum DM. Sonographic evaluation of abdominal lymphangiomas in children. J Ultrasound Med 1987; 6:487-95. Cutillo DP, Swatbe KC, Cucco J, et al. Case report—CT and MR imaging in cystic abdominal lymphangiomatosis. J Comput Assist Tomogr 1989;13:534-6.
www.elsevier.com/locate/jpedsurg
Solitary hepatic lymphangioma in a 22-day-old infant Kedar Singh Shahi a,⁎, Bhandari Geeta b , Prashant Rajput a a
Department of Surgery, U.F.H.T. Medical College, Haldwani (Nainital), India Department of Anaesthesiology, U.F.H.T. Medical College, Haldwani (Nainital), India
b
Received 23 December 2008; revised 2 April 2009; accepted 4 April 2009
Key words: Hepatic lymphangioma; Infant
Abstract Lymphangiomas are congenital malformations of the lymphatic system composed of dilated endothelial lined spaces of varying sizes containing lymph. Hepatic lymphangioma is a rare finding, exclusively present in children and adolescents. Most reported cases occur as part of diffuse lymphangiomatosis. Solitary lymphangioma of the liver in infants is extremely rare. This report describes a case of unilocular, solitary hepatic lymphangioma in a 22-day-old infant. © 2009 Elsevier Inc. All rights reserved.
Lymphangiomas are congenital malformations of the lymphatic system composed of dilated endothelial lined spaces of varying sizes containing lymph. Hepatic lymphangioma is a rare finding, exclusively present in children and adolescents. Most reported cases occur as part of diffuse lymphangiomatosis. Solitary lymphangioma of liver in infants is extremely rare. This report describes a case of unilocular, solitary hepatic lymphangioma in a 22-day-old infant.
1. Case report A 22-day-old male baby presented with progressive abdominal distension since birth. The baby was otherwise normal. There was no associated pain, fever, refusal to feed, vomiting, urinary symptoms, or alteration in bowel motions. The antenatal period and delivery were uneventful. Physical examination revealed weight of 4.5 kg. The abdomen was markedly distended, tense, and nontender. A fluid thrill was ⁎ Corresponding author. Tel.: +91 05946 234442, 09412906925 (Mobile); fax: +91 05946 234423. E-mail address: [email protected] (K.S. Shahi). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.04.022
noted, and the bowel sounds were normal. Plain abdominal radiographs were unremarkable. Abdominal ultrasonography revealed a huge cystic mass in the peritoneal cavity. Computed tomography of the abdomen demonstrated a large abdominopelvic hypodense cystic lesion with thin walls measuring 9.6 × 8 × 13 cm within the peritoneal cavity, displacing the bowel loops to the periphery and extending superiorly abutting the inferior surface of the liver. There were no definite septations or solid elements within the cyst (Fig. 1). Liver function tests showed the serum bilirubin increased, indirect (3.3 mg/dL), and alkaline phosphatase level elevated (771 U/L). At laparotomy, a huge unilocular cyst arising from the visceral surface of the right lobe of liver extending into whole abdominal cavity and pelvis was observed. The cyst contained straw colored fluid (Figs. 2 and 3). The gallbladder, extrahepatic biliary tree, and other intraabdominal organs were normal. Complete excision of the cyst was accomplished. Histological examination showed the mass had multiple thin-walled cystic spaces lined by a single layer of endothelial cells filled with brownish granular tissue and stroma infiltrated by lymphoid cells (Fig. 4). A diagnosis of cystic lymphamgioma was made. The postoperative period
E10
Fig. 1 Computerized tomography of abdomen showing the cystic lesion in the abdomen.
was uneventful. The patient was discharged on the fifth postoperative day. A repeat abdominal ultrasound study after 1 month was normal.
2. Discussion Hepatic lymph channels are derived from the space of Disse as well as from capillary leakage from the peribiliary plexus [1]. Hepatic lymphangioma may occur as a solitary mass or more commonly as multiple masses composed of dilated lymphatic channels containing proteinaceous fluid or blood [2]. Solitary hepatic lymphangioma is an extremely rare benign neoplasm characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma. Lymphangiomas of the liver may occur in isolation or in association with lymphangiomas of other viscera [3]. They are congenital malformations of the lymphatic system, considered as areas of localized lymphatic stasis due to blockage of regional
Fig. 2 Intraoperative finding: huge cyst arising from under surface of right lobe of liver.
K.S. Shahi et al.
Fig. 3
Opened cyst: unilocular cyst containing straw colored fluid.
lymphatic drainage [4]. Lymphangiomas most frequently involve the head and neck, followed by the extremities, trunk, and intraabdominal areas in decreasing order of frequency [5]. Less than 5% of lymphangiomas involve the abdomen, mediastinum, scrotum, bone, and extremities [5,6]. Reports of lymphangiomas solely involving the liver are extremely rare [7,8]. Most of the reported cases are in older children and adults [3,7,9]. To our knowledge, the previous youngest patient and only infant with a solitary hepatic lymphangioma reported in literature was aged 4 months [10]. Clinical presentation of hepatic lymphangiomas is nonspecific and is mainly because of hepatomegaly with vague abdominal pain. Intraabdominal lymphangiomas may grow slowly and present with compression symptoms [11]. Our patient presented with rapidly progressive abdominal distension. Operation was planned to avoid the possible complications such as rupture, hemorrhage, and compressive symptoms. Elevation in serum alkaline phosphatase and bilirubin levels were nonspecific and were not helpful in making the diagnosis.
Fig. 4 Dilated cystic lymphatic channels lined by a single layer of endothelial cells (hematoxylin-eosin stain ×40).
Solitary hepatic lymphangioma Preoperative diagnosis of hepatic lymphangiomas is difficult. Most cases are diagnosed at laparotomy as in our case [9,10]. The typical ultrasonography, computed tomography, and magnetic resonance imaging appearance of hepatic lymphangioma is that of a cystic or multicystic hepatic mass with internal septations [12-14]. Our case was different because it consisted of a huge unilocular cyst with no septations. This finding was confirmed at the time of laparotomy. Solitary hepatic lymphangioma is a benign curable disease in infants and children. It should be considered in the differential diagnosis of cystic intraabdominal mass in infants. Accurate preoperative diagnosis is extremely difficult, and surgical excision is the recommended treatment when diagnosis is in doubt and in symptomatic patients [3,9]. Recurrence has not been reported, and long-term prognosis is excellent.
E11
[3] [4] [5] [6]
[7] [8] [9]
[10] [11] [12]
References [1] Kanel GC, Korula J. General aspect of liver and liver diseases. Atlas of liver pathology. 4th ed. Philadelphia: Elsevier/Saunders; 2005. p. 5. [2] Anthony PP. Tumors; aetiology, epidemiology and pathology. In: McSween RNM, Butt AD, Portman BC, et al, editors. Pathology of
[13]
[14]
liver. 4th ed. Philadelphia: Churchill Livingstone/Elsevier; 2002. p. 799-850. Chan SC, Huang SF, Lee WC, et al. Solitary hepatic lymphangioma— a case report. Int J Clin Pract Suppl 2005;147:100-2. Godart S. Embryological significance of lymphangiomas. Arch Dis Child 1966;41:204-6. Hancock BJ, Dickens St-Vil D. Complications of lymphangiomas in children. J Pediatr Surg 1992;27:220-4. Wan YL, Lee TY, Hung CF, et al. Ultrasound and CT findings of a cecal lymphangioma presenting with intussusception. Eur J Radiol 1998;2:77-9. Koh CC, Sheu JC. Hepatic lymphangioma—a case report. Pediatr Surg Int 2000;16:515-6. Barnes PA, Thomas JL, Bernardino ME. Pitfalls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981;141:129-33. Stavropoulos M, Vagianos C, Scopa CD, et al. Solitary hepatic lymphangioma—a rare benign tumour: a case report HPB. Surgery 1994;8:33-6. Martin A, Nzegwu O, et al. Solitary hepatic lymphangioma in an infant. J Perinat Med 35 2007;35(2):164-5. Wan YL, Lee TY, Gau JS, et al. A rare abdominal cystic mass with unusual presentation. Postgrad Med J 1998;74:175-6. Barnes PA, Thomas JL, Bernardino ME. Pit-falls in the diagnosis of hepatic cysts by computed tomography. Radiology 1981;141:129-33. Blumhagen JD, Wood BJ, Rosenbaum DM. Sonographic evaluation of abdominal lymphangiomas in children. J Ultrasound Med 1987; 6:487-95. Cutillo DP, Swatbe KC, Cucco J, et al. Case report—CT and MR imaging in cystic abdominal lymphangiomatosis. J Comput Assist Tomogr 1989;13:534-6.