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Lymphangioma of the falciform ligament—a case report
Lymphangioma of the Falciform Ligament—A Case Report By Katherine Morgan and Richard R. Ricketts Atlanta, Georgia
A rare case of a lymphangioma of the falciform ligament in a child is described. He presented with abdominal pain and was found to have an unusual intraabdominal mass. Resection was curative.
J Pediatr Surg 39:1276-1279. © 2004 Elsevier Inc. All rights reserved.
L
from the falciform ligament, was found. It extended from the umbilicus to the undersurface of the liver. It had a stalk of vascularized tissue coming out of the region of the porta hepatis, just superior to the portal triad. The mass was intimately adherent to the anterior abdominal wall, and, therefore, the posterior rectus sheath was excised with the mass, which was separated easily from the liver by division of the described vascular pedicle. An intraoperative frozen section initially suggested mesenchymal hamartoma of the liver. The final pathology report, however, showed lymphangioma of the falciform ligament with acute and chronic inflammation (Fig 2). The patient’s postoperative course was uneventful, and he went home on a regular diet on the second postoperative day. He was well on follow-up examination 18 months later.
YMPHANGIOMAS are benign lesions of the lymphatic system most likely congenital in origin. They may be locally aggressive. They are most commonly located in the skin but more importantly may involve the deeper tissues, particularly of the neck, axilla, mediastinum, and retroperitoneum. Exceptionally, they may be found intraabdominally. We describe 1 patient who, on exploration, was found to have a lymphangioma of the falciform ligament. This unusual location has not been described previously.
INDEX WORDS: Lymphangioma, intraabdominal, falciform ligament.
CASE REPORT A 7-year-old white boy without significant past medical history presented with a 1-day history of constant periumbilical and right upper quadrant abdominal pain. He complained of nausea and anorexia without emesis. He maintained normal bowel function. He complained of some subjective fevers. On physical examination his temperature was 38.6°C, pulse was 120, and respiratory rate was 32. He appeared well nourished and developed but was in mild distress. His abdominal examination found normoactive bowel sounds, fullness in the right upper quadrant, and tenderness in the midepigastrum and right upper quadrant with some voluntary guarding. Results of his laboratory studies showed a white blood count of 13,200 with 85% segmented neutrophils, and normal hemoglobin level, serum chemistry results, and urinalysis. His C-reactive protein value was 1.9. He had an abdominal sonogram and abdominal and pelvic computed tomography scan. These showed a 10-⫻ 6.1-⫻ 16.5-cm complex cystic lesion within the right upper quadrant inferior to the liver margin, displacing the transverse colon and duodenum. The scans also showed free fluid in the right paracolic gutter and pelvis (Fig 1). The differential diagnosis included a mesenteric cyst, a mesenchymal hamartoma of the liver, or an intraabdominal lymphangioma. He was hydrated, given perioperative antibiotics, and taken to the operating room for abdominal exploration. A firm but cystic mass, arising
From the Division of Pediatric Surgery, Emory University, Atlanta, GA. Address reprint requests to Richard R. Ricketts, MD, Division of Pediatric Surgery, 2040 Ridgewood Dr, NE, Atlanta, GA 30322. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3908-0027$30.00/0 doi:10.1016/j.jpedsurg.2004.04.032 1276
DISCUSSION
Lymphangiomas are hamartomatous or neoplastic tumors of the lymphatic system. The etiology and pathophysiology is not completely understood, but it is likely that they result from sequestration of segments of the primordial endothelial sacs from which the mature lymphatic system is derived. These abnormally isolated sacs then may proliferate to become lymphatic malformations (such as lymphangiomas).1 Lymphangiomas are benign but characteristically infiltrate surrounding structures to cause morbidity. They are found primarily in children, with two thirds apparent at birth and most discovered before the age of 2 years.2 The mean age of patients at the time of resection of abdominal lymphangiomas is reported between 2 and 10 years.3-5 They tend to progress and grow, unlike mesenteric cysts, which remain stable in size, or hemangiomas, which often involute. Lymphangiomas are commonly located in the skin and superficial subcutaneous tissues, although they can be found in deeper tissues, in particular the neck, axilla, mediastinum, and retroperitoneum. The incidence of intraabdominal lymphangioma is low. Hancock6, in a series of 193 cases of lymphangiomas in children over a 10-year period in Montreal, found the distribution of lymphangiomas to be cervical (31.4%), craniofacial Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: pp 1276-1279
FALCIFORM LIGAMENT LYMPHANGIOMA
1277
Fig 1. Serial CT images, from rostral (A) to caudal (D), show a cystic mass arising from just above the porta hepatis and extending inferiorly along the anterior abdominal wall.
(18.9%), extremity (18.9%), trunk (9.2%), intraabdominal (9.2%), cervicoaxillothoracic (4.9%), multiple (3.8%), cervicomediastinal (2.2%), and intrathoracic (1.6%). The occurrence of abdominal lymphangioma has been reported to be between 2% and 9.2%.2,6 Many rare sites have been reported, including cecum (causing intussusception),7 small bowel mesentery,4,8-15 omentum,4,9 pancreas,16 retroperitoneum,4,9,11,13,17,18 spleen,19 gallbladder,9,20 liver,21 adrenal,4,22 gluteus,13 pelvis,13 and inguinoscrotal region (one reportedly a 57-kg scrotal tumor).13,23 We encountered a lymphangioma of the falciform ligament in a 7-year-old boy. This location is unusual, and, on our review of the literature, has not been described previously. In addition, this patient presented with significant abdominal pain, fever, tachycardia, and mild leukocytosis. These symptoms and laboratory findings were probably caused by the acute inflammation present within this lymphangioma. Many descriptions of abdominal lymphangioma suggest a significant incidence of asymptom-
atic presentation. This may reflect a failure to distinguish between abdominal lymphangiomas and mesenteric cysts. Review of the literature, however, finds a high incidence of associated symptoms with lymphangioma, particularly in children.3,24,25 Kosir et al3, in a review of 13 cases of abdominal lymphangioma, found that 88% (12 of 13) were symptomatic. Abdominal pain (11 of 13), vomiting (8 of 13), increased abdominal girth (8 of 13), and nausea (6 of 13) were the most common symptoms. The author called for a reconsideration of abdominal lymphangioma as a cause for the “acute abdomen” in children.3 We concur and suggest that this entity should be a part of the differential diagnosis. The treatment of choice of abdominal lymphangioma is complete surgical resection, if possible, without damage to vital structures. The recurrence rate with incomplete resection was found by Hancock to be unacceptably high at 52.9% compared with 11.8% in total resection.6 The overall benign nature of these lesions, however, precludes sacrifice of vital structures to achieve complete
1278
MORGAN AND RICKETTS
Fig 2. Lymphangioma with septae, proteinaceous debris and lymphocytes in the lumen, and acute and chronic inflammation (arrow).
radical resection in more extensive cases. Other lessinvasive adjunctive measures such as sclerotherapy or radiation therapy have been used for large cystic hygro-
mas and can be considered in the treatment of unresectable lymphangiomas, although these methods have not been evaluated sufficiently.6,26-30
REFERENCES 1. Gross RE: Cystic hygroma, in Gross RE (ed): The Surgery of Infancy and Childhood. Philadelphia, PA, Saunders, 1953, pp 960-970 2. Guemes I, Ibanez V, Barrios JE, et al: Acute abdomen: Other initial symptom of abdominal lymphangioma. Cirug Pediatr 11:73-75, 1998 3. Kosir MA, Sonnino RE, Gauderer MW: Pediatric abdominal lymphangiomas: A plea for early recognition. J Pediatr Surg 26:13091313, 1991 4. Rygl M, Snajdauf J, Pycha K, et al: Abdominal lymphangiomas in childhood. Rozhledy VChir 79:609-612, 2000 5. Steyaert H, Guitard J, Moscovici J, et al: Abdominal cystic lymphangioma in children: Benign lesions that can have a proliferative course. J PediatrSurg 31:677-680, 1996 6. JSt-Vil D, Luks FI, et al: Complications of lymphangiomas in children. Pediatr Surg 27:220-226, 1992 7. Wan YL, Lee TY, Hung CF, et al: Ultrasound and CT findings of a cecal lymphangioma presenting with intussusception. Euro J Radiol 27:77-79, 1998 8. Calderon-Garciduenas AL, Padron-Trevino M, Holguin-Aragon J, et al: Cystic lymphangioma of the ileum. A case report. Boletin Medico del Hospital Infantil de Mexico 48:267-270, 1991 9. Cjung JH, Suh YL, Park IA, et al: A pathologic study of abdominal lymphangiomas. J Korean Med Science 14:257-262, 1999 10. Garay J, Barriola M, Gutierrez-Hoyos A, et al: Mesenteric cystic lymphangiomas (report of three cases). Anales Espanoles de Pediatria 11:913-920, 1978 11. Liessi G, Sandini F, Spaliviero B: Cystic lymphangiomas of the abdomen. CT and US findings. Radiologia Medica 78:204-209, 1989 12. Melcher GA, Ruedi T, Allemann J, et al: Cystic lymphangioma of the mesenterial root as a rare cause of acute abdomen. Chirurg 66:229-231, 1995
13. Pandit SK, Rattan KN, Budhiraja S, et al: Cystic lymphangioma with special reference to rare sites. Indian J Pediatrics 67:339-341, 2000 14. Scheye T, Aufauvre B, Vanneuville G, et al: Abdominal cystic lymphangiomas in children. Apropos of 6 cases. J de Chirurg 131:2733, 1994 15. Seki J, Ueda T, Kasuya T, et al: Lymphangioma of the jejunum and mesentary presenting with acute abdomen in an adult. J Gastroenterol 33:107-111, 1998 16. Itterbeek P, Vanclooster P, deGheldere C: Cystic lymphangioma of the pancreas: An unusual cause of the acute surgical abdomen. Acta Chir Belgica 97:297-298, 1997 17. Chang PF, Teng RJ, Chang MH: Retroperitoneal cystic lymphangioma: Report of one case. Chung-Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 38:306-308, 1997 18. Waisberg J, Pezzolo S, Henrique AC, et al: Retroperitoneal cystic lymphangioma. Arquivos de Gastroenterologia 36:37-41, 1999 19. Komatsuda T, Ishida H, Konno K, et al: Splenic lymphangioma: US and CT diagnosis and clinical manifestations. Abdom Imaging 24:414-417, 1999 20. Ohba K, Sugauchi F, Orito E, et al: Cystic lymphangioma of the gallbladder: A case report. J Gastroenterol Hepatol 10:693-696, 1995 21. Koh CC, Sheu JC: Hepatic lymphangioma—A case report. Pediatr Surg Int 16:515-516, 2000 22. Longo JM, Jafri SZ, Bis KB: Adrenal lymphangioma: A case report. Clin Imaging 24:104-106, 2000 23. Moreno J, Wingartz H, Gonzalez J, et al: Linfangioma gigante de escroto: Un tumor de 57 kg. Gaceta Medica de Mexico 136:369-371, 2000 24. DePerrot M, Rostan O, Morel P, et al: Abdominal lymphangioma in adults and children. Br J Surg 85:395-397, 1998
FALCIFORM LIGAMENT LYMPHANGIOMA
25. Takiff J, Calabria R, Yin L, et al: Mesenteric cysts and intraabdominal cystic lymphangiomas. Arch Surg 120:1266-1269, 1985 26. Mabrut JY, Grandjean JP, Henry L, et al: Mesenteric and mesocoloc cystic lymphangiomas. Diagnostic and therapeutic management. Annales de Chirurgie 127:343-349, 2002 27. Ng E, Shah V, Armstron D, et al: Cavernous lymphangioma. J Pediatr 138:146, 2001 28. Ogita S, Tsuto T, Tokiwa K, et al: Intracystic injection of
1279
OK-432: A new sclerosing therapy for cystic hygroma in children. Br J Surg 74:690-691, 1987 29. Ogita S, Tsuto T, Nakamura K, et al: OK-432 therapy for lymphangioma in children: Why and how does it work? J Pediatr Surg 31:477-480, 1996 30. Samuel M, McCarthy L, Boddy SA: Efficacy and safety of OK-432 sclerotherapy for giant cystic hygroma in a newborn. Fetal Diagn Ther 15:93-96, 2000
A rare case of a lymphangioma of the falciform ligament in a child is described. He presented with abdominal pain and was found to have an unusual intraabdominal mass. Resection was curative.
J Pediatr Surg 39:1276-1279. © 2004 Elsevier Inc. All rights reserved.
L
from the falciform ligament, was found. It extended from the umbilicus to the undersurface of the liver. It had a stalk of vascularized tissue coming out of the region of the porta hepatis, just superior to the portal triad. The mass was intimately adherent to the anterior abdominal wall, and, therefore, the posterior rectus sheath was excised with the mass, which was separated easily from the liver by division of the described vascular pedicle. An intraoperative frozen section initially suggested mesenchymal hamartoma of the liver. The final pathology report, however, showed lymphangioma of the falciform ligament with acute and chronic inflammation (Fig 2). The patient’s postoperative course was uneventful, and he went home on a regular diet on the second postoperative day. He was well on follow-up examination 18 months later.
YMPHANGIOMAS are benign lesions of the lymphatic system most likely congenital in origin. They may be locally aggressive. They are most commonly located in the skin but more importantly may involve the deeper tissues, particularly of the neck, axilla, mediastinum, and retroperitoneum. Exceptionally, they may be found intraabdominally. We describe 1 patient who, on exploration, was found to have a lymphangioma of the falciform ligament. This unusual location has not been described previously.
INDEX WORDS: Lymphangioma, intraabdominal, falciform ligament.
CASE REPORT A 7-year-old white boy without significant past medical history presented with a 1-day history of constant periumbilical and right upper quadrant abdominal pain. He complained of nausea and anorexia without emesis. He maintained normal bowel function. He complained of some subjective fevers. On physical examination his temperature was 38.6°C, pulse was 120, and respiratory rate was 32. He appeared well nourished and developed but was in mild distress. His abdominal examination found normoactive bowel sounds, fullness in the right upper quadrant, and tenderness in the midepigastrum and right upper quadrant with some voluntary guarding. Results of his laboratory studies showed a white blood count of 13,200 with 85% segmented neutrophils, and normal hemoglobin level, serum chemistry results, and urinalysis. His C-reactive protein value was 1.9. He had an abdominal sonogram and abdominal and pelvic computed tomography scan. These showed a 10-⫻ 6.1-⫻ 16.5-cm complex cystic lesion within the right upper quadrant inferior to the liver margin, displacing the transverse colon and duodenum. The scans also showed free fluid in the right paracolic gutter and pelvis (Fig 1). The differential diagnosis included a mesenteric cyst, a mesenchymal hamartoma of the liver, or an intraabdominal lymphangioma. He was hydrated, given perioperative antibiotics, and taken to the operating room for abdominal exploration. A firm but cystic mass, arising
From the Division of Pediatric Surgery, Emory University, Atlanta, GA. Address reprint requests to Richard R. Ricketts, MD, Division of Pediatric Surgery, 2040 Ridgewood Dr, NE, Atlanta, GA 30322. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3908-0027$30.00/0 doi:10.1016/j.jpedsurg.2004.04.032 1276
DISCUSSION
Lymphangiomas are hamartomatous or neoplastic tumors of the lymphatic system. The etiology and pathophysiology is not completely understood, but it is likely that they result from sequestration of segments of the primordial endothelial sacs from which the mature lymphatic system is derived. These abnormally isolated sacs then may proliferate to become lymphatic malformations (such as lymphangiomas).1 Lymphangiomas are benign but characteristically infiltrate surrounding structures to cause morbidity. They are found primarily in children, with two thirds apparent at birth and most discovered before the age of 2 years.2 The mean age of patients at the time of resection of abdominal lymphangiomas is reported between 2 and 10 years.3-5 They tend to progress and grow, unlike mesenteric cysts, which remain stable in size, or hemangiomas, which often involute. Lymphangiomas are commonly located in the skin and superficial subcutaneous tissues, although they can be found in deeper tissues, in particular the neck, axilla, mediastinum, and retroperitoneum. The incidence of intraabdominal lymphangioma is low. Hancock6, in a series of 193 cases of lymphangiomas in children over a 10-year period in Montreal, found the distribution of lymphangiomas to be cervical (31.4%), craniofacial Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: pp 1276-1279
FALCIFORM LIGAMENT LYMPHANGIOMA
1277
Fig 1. Serial CT images, from rostral (A) to caudal (D), show a cystic mass arising from just above the porta hepatis and extending inferiorly along the anterior abdominal wall.
(18.9%), extremity (18.9%), trunk (9.2%), intraabdominal (9.2%), cervicoaxillothoracic (4.9%), multiple (3.8%), cervicomediastinal (2.2%), and intrathoracic (1.6%). The occurrence of abdominal lymphangioma has been reported to be between 2% and 9.2%.2,6 Many rare sites have been reported, including cecum (causing intussusception),7 small bowel mesentery,4,8-15 omentum,4,9 pancreas,16 retroperitoneum,4,9,11,13,17,18 spleen,19 gallbladder,9,20 liver,21 adrenal,4,22 gluteus,13 pelvis,13 and inguinoscrotal region (one reportedly a 57-kg scrotal tumor).13,23 We encountered a lymphangioma of the falciform ligament in a 7-year-old boy. This location is unusual, and, on our review of the literature, has not been described previously. In addition, this patient presented with significant abdominal pain, fever, tachycardia, and mild leukocytosis. These symptoms and laboratory findings were probably caused by the acute inflammation present within this lymphangioma. Many descriptions of abdominal lymphangioma suggest a significant incidence of asymptom-
atic presentation. This may reflect a failure to distinguish between abdominal lymphangiomas and mesenteric cysts. Review of the literature, however, finds a high incidence of associated symptoms with lymphangioma, particularly in children.3,24,25 Kosir et al3, in a review of 13 cases of abdominal lymphangioma, found that 88% (12 of 13) were symptomatic. Abdominal pain (11 of 13), vomiting (8 of 13), increased abdominal girth (8 of 13), and nausea (6 of 13) were the most common symptoms. The author called for a reconsideration of abdominal lymphangioma as a cause for the “acute abdomen” in children.3 We concur and suggest that this entity should be a part of the differential diagnosis. The treatment of choice of abdominal lymphangioma is complete surgical resection, if possible, without damage to vital structures. The recurrence rate with incomplete resection was found by Hancock to be unacceptably high at 52.9% compared with 11.8% in total resection.6 The overall benign nature of these lesions, however, precludes sacrifice of vital structures to achieve complete
1278
MORGAN AND RICKETTS
Fig 2. Lymphangioma with septae, proteinaceous debris and lymphocytes in the lumen, and acute and chronic inflammation (arrow).
radical resection in more extensive cases. Other lessinvasive adjunctive measures such as sclerotherapy or radiation therapy have been used for large cystic hygro-
mas and can be considered in the treatment of unresectable lymphangiomas, although these methods have not been evaluated sufficiently.6,26-30
REFERENCES 1. Gross RE: Cystic hygroma, in Gross RE (ed): The Surgery of Infancy and Childhood. Philadelphia, PA, Saunders, 1953, pp 960-970 2. Guemes I, Ibanez V, Barrios JE, et al: Acute abdomen: Other initial symptom of abdominal lymphangioma. Cirug Pediatr 11:73-75, 1998 3. Kosir MA, Sonnino RE, Gauderer MW: Pediatric abdominal lymphangiomas: A plea for early recognition. J Pediatr Surg 26:13091313, 1991 4. Rygl M, Snajdauf J, Pycha K, et al: Abdominal lymphangiomas in childhood. Rozhledy VChir 79:609-612, 2000 5. Steyaert H, Guitard J, Moscovici J, et al: Abdominal cystic lymphangioma in children: Benign lesions that can have a proliferative course. J PediatrSurg 31:677-680, 1996 6. JSt-Vil D, Luks FI, et al: Complications of lymphangiomas in children. Pediatr Surg 27:220-226, 1992 7. Wan YL, Lee TY, Hung CF, et al: Ultrasound and CT findings of a cecal lymphangioma presenting with intussusception. Euro J Radiol 27:77-79, 1998 8. Calderon-Garciduenas AL, Padron-Trevino M, Holguin-Aragon J, et al: Cystic lymphangioma of the ileum. A case report. Boletin Medico del Hospital Infantil de Mexico 48:267-270, 1991 9. Cjung JH, Suh YL, Park IA, et al: A pathologic study of abdominal lymphangiomas. J Korean Med Science 14:257-262, 1999 10. Garay J, Barriola M, Gutierrez-Hoyos A, et al: Mesenteric cystic lymphangiomas (report of three cases). Anales Espanoles de Pediatria 11:913-920, 1978 11. Liessi G, Sandini F, Spaliviero B: Cystic lymphangiomas of the abdomen. CT and US findings. Radiologia Medica 78:204-209, 1989 12. Melcher GA, Ruedi T, Allemann J, et al: Cystic lymphangioma of the mesenterial root as a rare cause of acute abdomen. Chirurg 66:229-231, 1995
13. Pandit SK, Rattan KN, Budhiraja S, et al: Cystic lymphangioma with special reference to rare sites. Indian J Pediatrics 67:339-341, 2000 14. Scheye T, Aufauvre B, Vanneuville G, et al: Abdominal cystic lymphangiomas in children. Apropos of 6 cases. J de Chirurg 131:2733, 1994 15. Seki J, Ueda T, Kasuya T, et al: Lymphangioma of the jejunum and mesentary presenting with acute abdomen in an adult. J Gastroenterol 33:107-111, 1998 16. Itterbeek P, Vanclooster P, deGheldere C: Cystic lymphangioma of the pancreas: An unusual cause of the acute surgical abdomen. Acta Chir Belgica 97:297-298, 1997 17. Chang PF, Teng RJ, Chang MH: Retroperitoneal cystic lymphangioma: Report of one case. Chung-Hua Min Kuo Hsiao Erh Ko I Hsueh Hui Tsa Chih 38:306-308, 1997 18. Waisberg J, Pezzolo S, Henrique AC, et al: Retroperitoneal cystic lymphangioma. Arquivos de Gastroenterologia 36:37-41, 1999 19. Komatsuda T, Ishida H, Konno K, et al: Splenic lymphangioma: US and CT diagnosis and clinical manifestations. Abdom Imaging 24:414-417, 1999 20. Ohba K, Sugauchi F, Orito E, et al: Cystic lymphangioma of the gallbladder: A case report. J Gastroenterol Hepatol 10:693-696, 1995 21. Koh CC, Sheu JC: Hepatic lymphangioma—A case report. Pediatr Surg Int 16:515-516, 2000 22. Longo JM, Jafri SZ, Bis KB: Adrenal lymphangioma: A case report. Clin Imaging 24:104-106, 2000 23. Moreno J, Wingartz H, Gonzalez J, et al: Linfangioma gigante de escroto: Un tumor de 57 kg. Gaceta Medica de Mexico 136:369-371, 2000 24. DePerrot M, Rostan O, Morel P, et al: Abdominal lymphangioma in adults and children. Br J Surg 85:395-397, 1998
FALCIFORM LIGAMENT LYMPHANGIOMA
25. Takiff J, Calabria R, Yin L, et al: Mesenteric cysts and intraabdominal cystic lymphangiomas. Arch Surg 120:1266-1269, 1985 26. Mabrut JY, Grandjean JP, Henry L, et al: Mesenteric and mesocoloc cystic lymphangiomas. Diagnostic and therapeutic management. Annales de Chirurgie 127:343-349, 2002 27. Ng E, Shah V, Armstron D, et al: Cavernous lymphangioma. J Pediatr 138:146, 2001 28. Ogita S, Tsuto T, Tokiwa K, et al: Intracystic injection of
1279
OK-432: A new sclerosing therapy for cystic hygroma in children. Br J Surg 74:690-691, 1987 29. Ogita S, Tsuto T, Nakamura K, et al: OK-432 therapy for lymphangioma in children: Why and how does it work? J Pediatr Surg 31:477-480, 1996 30. Samuel M, McCarthy L, Boddy SA: Efficacy and safety of OK-432 sclerotherapy for giant cystic hygroma in a newborn. Fetal Diagn Ther 15:93-96, 2000