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Lymphangioma of the Falciform Ligament—A Case ReportBy Katherine Morgan and Richard R. Ricketts

Atlanta, Georgia

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rare case of a lymphangioma of the falciform ligament in ahild is described. He presented with abdominal pain andas found to have an unusual intraabdominal mass. Resec-

ion was curative.

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Pediatr Surg 39:1276-1279. © 2004 Elsevier Inc. All rightseserved.

NDEX WORDS: Lymphangioma, intraabdominal, falciform

igament.

YMPHANGIOMAS are benign lesions of the lym-phatic system most likely congenital in origin.

hey may be locally aggressive. They are most com-only located in the skin but more importantly may

nvolve the deeper tissues, particularly of the neck, ax-lla, mediastinum, and retroperitoneum. Exceptionally,hey may be found intraabdominally. We describe 1atient who, on exploration, was found to have a lym-hangioma of the falciform ligament. This unusual loca-ion has not been described previously.

CASE REPORT

A 7-year-old white boy without significant past medical historyresented with a 1-day history of constant periumbilical and right upperuadrant abdominal pain. He complained of nausea and anorexiaithout emesis. He maintained normal bowel function. He complainedf some subjective fevers.On physical examination his temperature was 38.6°C, pulse was 120,

nd respiratory rate was 32. He appeared well nourished and developedut was in mild distress. His abdominal examination found normoac-ive bowel sounds, fullness in the right upper quadrant, and tendernessn the midepigastrum and right upper quadrant with some voluntaryuarding. Results of his laboratory studies showed a white blood countf 13,200 with 85% segmented neutrophils, and normal hemoglobinevel, serum chemistry results, and urinalysis. His C-reactive proteinalue was 1.9. He had an abdominal sonogram and abdominal andelvic computed tomography scan. These showed a 10-� 6.1-�6.5-cm complex cystic lesion within the right upper quadrant inferioro the liver margin, displacing the transverse colon and duodenum. Thecans also showed free fluid in the right paracolic gutter and pelvisFig 1).

The differential diagnosis included a mesenteric cyst, a mesenchy-al hamartoma of the liver, or an intraabdominal lymphangioma. Heas hydrated, given perioperative antibiotics, and taken to the operat-

ng room for abdominal exploration. A firm but cystic mass, arising

From the Division of Pediatric Surgery, Emory University, Atlanta,A.Address reprint requests to Richard R. Ricketts, MD, Division of

ediatric Surgery, 2040 Ridgewood Dr, NE, Atlanta, GA 30322.© 2004 Elsevier Inc. All rights reserved.0022-3468/04/3908-0027$30.00/0

rom the falciform ligament, was found. It extended from the umbilicuso the undersurface of the liver. It had a stalk of vascularized tissueoming out of the region of the porta hepatis, just superior to the portalriad. The mass was intimately adherent to the anterior abdominal wall,nd, therefore, the posterior rectus sheath was excised with the mass,hich was separated easily from the liver by division of the describedascular pedicle. An intraoperative frozen section initially suggestedesenchymal hamartoma of the liver. The final pathology report,

owever, showed lymphangioma of the falciform ligament with acutend chronic inflammation (Fig 2). The patient’s postoperative courseas uneventful, and he went home on a regular diet on the secondostoperative day. He was well on follow-up examination 18 monthsater.

DISCUSSION

Lymphangiomas are hamartomatous or neoplastic tu-ors of the lymphatic system. The etiology and patho-

hysiology is not completely understood, but it is likelyhat they result from sequestration of segments of therimordial endothelial sacs from which the mature lym-hatic system is derived. These abnormally isolated sacshen may proliferate to become lymphatic malformationssuch as lymphangiomas).1

Lymphangiomas are benign but characteristically in-ltrate surrounding structures to cause morbidity. Theyre found primarily in children, with two thirds apparentt birth and most discovered before the age of 2 years.2

he mean age of patients at the time of resection ofbdominal lymphangiomas is reported between 2 and 10ears.3-5 They tend to progress and grow, unlike mesen-eric cysts, which remain stable in size, or hemangiomas,hich often involute.Lymphangiomas are commonly located in the skin and

uperficial subcutaneous tissues, although they can beound in deeper tissues, in particular the neck, axilla,ediastinum, and retroperitoneum. The incidence of in-

raabdominal lymphangioma is low. Hancock6, in a se-ies of 193 cases of lymphangiomas in children over a0-year period in Montreal, found the distribution of

ymphangiomas to be cervical (31.4%), craniofacial

rnal of Pediatric Surgery, Vol 39, No 8 (August), 2004: pp 1276-1279

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1277FALCIFORM LIGAMENT LYMPHANGIOMA

18.9%), extremity (18.9%), trunk (9.2%), intraabdomi-al (9.2%), cervicoaxillothoracic (4.9%), multiple3.8%), cervicomediastinal (2.2%), and intrathoracic1.6%). The occurrence of abdominal lymphangioma haseen reported to be between 2% and 9.2%.2,6 Many rareites have been reported, including cecum (causing in-ussusception),7 small bowel mesentery,4,8-15 omen-um,4,9 pancreas,16 retroperitoneum,4,9,11,13,17,18 spleen,19

allbladder,9,20 liver,21 adrenal,4,22 gluteus,13 pelvis,13

nd inguinoscrotal region (one reportedly a 57-kg scrotalumor).13,23 We encountered a lymphangioma of thealciform ligament in a 7-year-old boy. This location isnusual, and, on our review of the literature, has not beenescribed previously.In addition, this patient presented with significant

bdominal pain, fever, tachycardia, and mild leukocyto-is. These symptoms and laboratory findings were prob-bly caused by the acute inflammation present within thisymphangioma. Many descriptions of abdominal lym-

Fig 1. Serial CT images, from rostral (A) to caudal (D), show a cyst

long the anterior abdominal wall.

hangioma suggest a significant incidence of asymptom- p

tic presentation. This may reflect a failure to distinguishetween abdominal lymphangiomas and mesentericysts. Review of the literature, however, finds a highncidence of associated symptoms with lymphangioma,articularly in children.3,24,25 Kosir et al3, in a review of3 cases of abdominal lymphangioma, found that 88%12 of 13) were symptomatic. Abdominal pain (11 of3), vomiting (8 of 13), increased abdominal girth (8 of3), and nausea (6 of 13) were the most common symp-oms. The author called for a reconsideration of abdom-nal lymphangioma as a cause for the “acute abdomen”n children.3 We concur and suggest that this entityhould be a part of the differential diagnosis.

The treatment of choice of abdominal lymphangiomas complete surgical resection, if possible, without dam-ge to vital structures. The recurrence rate with incom-lete resection was found by Hancock to be unacceptablyigh at 52.9% compared with 11.8% in total resection.6

he overall benign nature of these lesions, however,

ss arising from just above the porta hepatis and extending inferiorly

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recludes sacrifice of vital structures to achieve complete

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1278 MORGAN AND RICKETTS

adical resection in more extensive cases. Other less-nvasive adjunctive measures such as sclerotherapy or

adiation therapy have been used for large cystic hygro- b

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as and can be considered in the treatment of unresect-ble lymphangiomas, although these methods have not

6,26-30

Fig 2. Lymphangioma with sep-

tae, proteinaceous debris and lym-

phocytes in the lumen, and acute and

chronic inflammation (arrow).

een evaluated sufficiently.

CES

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