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Childhood retroperitoneal lymphangioma presenting following minor trauma
Injury, Int. J. Care Injured 30 (1999) 380±383
Case report
Childhood retroperitoneal lymphangioma presenting following minor trauma E. Freud a,b,*, U. Farkash a,b, R. Cassella c, C. Freire c, M. Zer a,b a
Department of Surgery, Schneider Children's Medical Center of Israel, Beilinson Campus, Petah Tiqva 49 202, Israel b Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel c Hospital de NinÄos Ricardo Gutierrez, Buenos Aires, Argentina Accepted 20 January 1999
1. Introduction Retroperitoneal lymphangioma is an uncommon, benign condition which presents mainly up to the second year of life. Complete excision is possible in the majority of cases and is associated with a favorable prognosis. We describe a 6-year-old boy who presented with severe shock after a minor trauma which caused massive bleeding in a previously unknown retroperitoneal lymphangioma.
2. Case report A healthy 6-year-old boy presented to our department after falling from a slide 1 meter high and landing in a supine position. Though initially alert, he rapidly became unresponsive; pulse was ®liform, and blood pressure was hardly detected by the emergency paramedics at the site. He was given ¯uids and cardiopulmonary resuscitation (CPR) for 30 min with an endotracheal airway in place and arrived at the hospital approximately 50 min following the accident. On admission, the patient was pale and there was marked abdominal distention and the right side of the abdomen was tender. Temperature was 36.58C, pulse 160/min, respiration 45/min, and blood pressure 80/40 mmHg. Medical and surgical history was unremarkable except for excision of a lymphangioma from the right thigh two years before. Chest X-ray revealed a small heart; cervical spine and pelvic radiography were * Corresponding author. Tel.: +972-3-925-3741; Fax: +972-3-9253899. E-mail address: [email protected] (E. Freud)
normal. The initial hematocrit was 19%, and 2 units of packed red blood cells were given. The paleness, abdominal distention, and need for ongoing blood transfusion prompted us to perform emergency laparotomy. On preoperative intravenous pyelography performed on the operating table, both kidneys had adequate excretion of contrast material. The bowel loops were displaced to the left abdomen (Fig. 1). Exploration revealed a huge, black-bluish cystic mass in the right retroperitoneum, extending from the right lobe of the liver deep into the pelvic ¯oor and displacing the ascending colon to the left abdomen (Fig. 2). Exploration of the midline, performed to obtain control of the major vessels before approaching the huge lateral lobulated mass, indicated that the aorta and inferior vena cava were normal. The right gutter was opened and the ascending colon detached from the cystic mass; special care was taken not to damage the mesocolon. Macroscopically, the lesion appeared to be a lymphangioma containing a huge amount of serosanguineous ¯uid, which was attached to the iliac vessels and lateral aspect of the inferior vena cava and right paravertebral area. The mass was easily separated by blunt dissection. Excision was performed in toto, and the majority of the ¯uid aspirated. There seemed to be capsular remnants ®rmly attached to the retroperitoneum at the midline, very close to the renal hilus where some diuse oozing was observed. No active source of bleeding was found. A closed drainage system was left in the right retroperitoneal space for 48 h and then removed with no evidence of bleeding. The histological diagnosis was cystic lymphangioma composed of dilated lymphatic spaces surrounded by connective tissue that contained lymphocytes and
0020-1383/99/$ - see front matter # 1999 Elsevier Science Ltd. All rights reserved. PII: S 0 0 2 0 - 1 3 8 3 ( 9 9 ) 0 0 0 9 9 - 6
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
Fig. 1. Intravenous pyelogram obtained on the operating table. Note bilateral excretion of contrast material without evidence of extravasation. The right abdomen is opaque and all bowel loops are displaced to the left abdomen.
erythrocytes. The huge cystic spaces were lined with cuboidal cells. The postoperative course was uneventful. Oral nutrition was started on day 4, and the patient was discharged on day 8 in good general condition. Follow-up sonography at four weeks, and six months showed no signs of recurrence.
3. Discussion We report a rare case in which an asymptomatic retroperitoneal lymphangioma became life-threatening and required emergency laparotomy following a minor trauma. Lymphangiomas are benign tumors which may be classi®ed into three histological types [1,2]: (1) lymphangioma simplex, consisting of a ®lmy mass of thinwalled, dilated lymphatics with a rich cellular connective tissue stroma which appear as small vesicles protruding into the epidermis; (2) cavernous
381
lymphangiomas, which are soft compressible tumors of dilated lymph vessels and lymphoid stroma invading the surrounding tissue; and (3) cystic lymphangiomas or cystic hygromas, which are poorly circumscribed, multilocular cystic masses containing serous, serosanguineous or chylous ¯uid. The masses range from 1 mm to 5 cm in diameter and often communicate with each other. Microscopically there are countless dilated lymphatic channels, often between lobules of adipose tissue. During the third or fourth month of embryonic development, two paired and two unpaired endothelial sacs form the primordia of the lymphatic system. Two jugular sacs in the neck, one sac in the mesenteric root, the cysterna chyli and two posterior sacs which appear at the bifurcation of the femoral and sciatic veins; the latter later form the retroperitoneal lymphatic system [3]. There is clinical and experimental evidence of retroperitoneal lymphaticovenous communications and pathways [4±6]. The most widely accepted etiological theory of cystic lymphangioma is early developmental sequestration of lymphatic vessels that fail to establish connections with normal draining lymphatics and became markedly dilated under the pressure of accumulating lymph [2]. A similar mechanism is thought to produce cavernous and simple lymphangioma, the site of lymphatic obstruction determining the pathologic type. About one-third of lymphangiomas occur in the neck and head. Cutaneous lymphangioma may occur in any area of the skin [7]. Uncommon sites include the groin, bone, mediastinum, uterus, stomach, small intestine, colon, rectum, omentum, mesentery, spleen, liver and retroperitoneal space [1,8,9]. The incidence of abdominal lymphangioma is unknown, but estimates vary from one case in 27,000 to one in 250,000 hospital admissions [10]. Of the 185 cases of abdominal lymphatic cysts reviewed by Burnett et al. [11] in 1950, only 5.4% were retroperitoneal. Skandalakis et al. [3] suggested that some retroperitoneal cysts are almost certainly of nonlymphatic origin. Though similar in structure to cysts within the leaves of the mesentery, if glomerular remnants can be demonstrated, they are likely to be nephrogenic rather than lymphatic [12]. The majority derive from the embryonic retroperitoneal lymph sac. Cystic retroperitoneal lymphangiomas usually present in infancy: 50±60% during the ®rst year of life and up to 90% by the second year [13]. The most common clinical manifestation is a palpable mass [14,15] which may appear suddenly and grow rapidly, then stop growing or even regress spontaneously. The presence of acute symptoms, such as abdominal pain, tenderness, guarding, fever, and leukocytosis, may indicate either obstruction (volvulus, extrinsic compression or entrapment in the pelvis) or peritonitis
382
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
Fig. 2. Huge black-bluish cystic mass protrudes into the right gutter, medially displacing and elevating the ascending colon.
(rupture, torsion, hemorrhage into the cyst or infected cyst), both of which require emergency surgery [14]; otherwise, extensive work-up is performed. Ultrasonography has high sensitivity and speci®city for a cystic abdominal mass [16]. Computerized tomography and magnetic resonance imaging may help, but the dierential diagnosis can be extensive [16,17]. Complete excision is possible in the majority of cases since, by de®nition, the cysts are not connected with any structure except loose areolar tissue [14]. When mesenteric cysts lie in close contact with the bowel, the attached bowel may require resection along with the cyst. If the cyst cannot be removed safely, internal marsupialization is indicated. Simple aspiration is associated with high rates of infection and recurrence. Prognosis is believed to be favorable, but there are no reports on recurrence rates. In one follow-up of 20 patients for a mean period of 23 months, 14% had a recurrence of intra-abdominal disease, and 9% (2 cases) had extra-abdominal lymphangioma [14]. One patient followed for 17 years after partial resection of mesenteric cysts had diuse extra-abdominal lymphangiomas necessitating many hospitalizations for infectious complications [14]. We speculate that in our case the ostensibly minor trauma ruptured a lumbar or other small retroperitoneal vein(s) closely connected by embryological pathways to a lymphangioma which may otherwise have
remained asymptomatic. The patient had undergone surgery for cutaneous lymphangioma two years earlier. We found only one similar case of an association between cutaneous and retroperitoneal lymphangioma in the English literature [18]. References [1] Koshy A, Tandon RK, Kapur BML, et al. Retroperitoneal lymphangioma. Am J Gastroenterol 1978;69:485±90. [2] Leonidas JC, Brill WB, Bhan I, et al. Cystic retroperitoneal lymphangioma in infants and children. Radiology 1978;127:203± 8. [3] Skandalakis JE, Gray SW, Ricketts RR. The lymphatic system. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. Baltimore, Hong Kong, London, Sydney: Williams and Wilkins, 1994. p. 877±97. [4] Pick JW, Anson BJ, Burnett HW. Communications between lymphatic and venous systems at renal level in man. Q Bull Northwest Univ Med School 1944;18:307±16. [5] Pressman JJ, Simon MB. Experimental evidence of direct communications between lymph nodes and veins. Surg Gynecol Obstet 1961;113:537±41. [6] Roddenberry H, Allen L. Observations on the abdominal lymphaticovenous communications of the squirrel monkey (Saimiri sciureus). Anat Rec 1967;159:147±58. [7] Flanagan BP, Helwig EB. Cutaneous lymphangioma. Arch Dermatol 1977;113:24±30. [8] Asch MJ, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery 1974;76:334±9. [9] Larson DL, Myhre BA, Scmidt ER. Lymphangiomatosis in
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
[10] [11] [12] [13] [14]
unusual sites: spleen, mesentery, retroperitoneum, mediastinum and greater omentum. Wisc Med J 1931;60:279±87. Moore TC. Congenital cysts of the mesentery. Ann Surg 1957;145:128±31. Burnett WE, Rosemond GP, Bucher RM. Mesenteric cysts: report of 3 cases, in one of which a calci®ed cysts was present. Arch Surg 1950;60:699±706. Maury JM. Retroperitoneal cysts of wolan origin. Surg Gynecol Obstet 1918;26:663. Rekhi BM, Esselstyn Jr BC, Levy I. Retroperitoneal cystic lymphangioma: report of two cases and review of the literature. Clev Clin Q 1972;39:125±8. Hebra A, Brown MF, McGeehin KM, Ross AJ. Mesenteric,
[15] [16] [17] [18]
383
omental and retroperitoneal cysts in children: a clinical study of 22 cases. South Med J 1993;86:173±6. Vanek VW, Philips AK. Retroperitoneal, mesenteric and omental cysts. Arch Surg 1984;119:838±42. Davidson AJ, Hartman DS. Lymphangioma of the retroperitoneum: CT and sonographic characteristics. Radiology 1990;175:507±10. Feldberg MA, Hendriks AV, Van Leeuwen MS, et al. Retroperitoneal cystic lymphangioma section imaging in two cases and review of the literature. Clin Imaging 1990;14:26±30. Irvine AD, Sweeney L, Corbett JR. Lymphangioma circumscriptum associated with paravesical cystic retroperitoneal lymphangioma. Br J Dermatol 1996;134:1135±7.
Case report
Childhood retroperitoneal lymphangioma presenting following minor trauma E. Freud a,b,*, U. Farkash a,b, R. Cassella c, C. Freire c, M. Zer a,b a
Department of Surgery, Schneider Children's Medical Center of Israel, Beilinson Campus, Petah Tiqva 49 202, Israel b Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel c Hospital de NinÄos Ricardo Gutierrez, Buenos Aires, Argentina Accepted 20 January 1999
1. Introduction Retroperitoneal lymphangioma is an uncommon, benign condition which presents mainly up to the second year of life. Complete excision is possible in the majority of cases and is associated with a favorable prognosis. We describe a 6-year-old boy who presented with severe shock after a minor trauma which caused massive bleeding in a previously unknown retroperitoneal lymphangioma.
2. Case report A healthy 6-year-old boy presented to our department after falling from a slide 1 meter high and landing in a supine position. Though initially alert, he rapidly became unresponsive; pulse was ®liform, and blood pressure was hardly detected by the emergency paramedics at the site. He was given ¯uids and cardiopulmonary resuscitation (CPR) for 30 min with an endotracheal airway in place and arrived at the hospital approximately 50 min following the accident. On admission, the patient was pale and there was marked abdominal distention and the right side of the abdomen was tender. Temperature was 36.58C, pulse 160/min, respiration 45/min, and blood pressure 80/40 mmHg. Medical and surgical history was unremarkable except for excision of a lymphangioma from the right thigh two years before. Chest X-ray revealed a small heart; cervical spine and pelvic radiography were * Corresponding author. Tel.: +972-3-925-3741; Fax: +972-3-9253899. E-mail address: [email protected] (E. Freud)
normal. The initial hematocrit was 19%, and 2 units of packed red blood cells were given. The paleness, abdominal distention, and need for ongoing blood transfusion prompted us to perform emergency laparotomy. On preoperative intravenous pyelography performed on the operating table, both kidneys had adequate excretion of contrast material. The bowel loops were displaced to the left abdomen (Fig. 1). Exploration revealed a huge, black-bluish cystic mass in the right retroperitoneum, extending from the right lobe of the liver deep into the pelvic ¯oor and displacing the ascending colon to the left abdomen (Fig. 2). Exploration of the midline, performed to obtain control of the major vessels before approaching the huge lateral lobulated mass, indicated that the aorta and inferior vena cava were normal. The right gutter was opened and the ascending colon detached from the cystic mass; special care was taken not to damage the mesocolon. Macroscopically, the lesion appeared to be a lymphangioma containing a huge amount of serosanguineous ¯uid, which was attached to the iliac vessels and lateral aspect of the inferior vena cava and right paravertebral area. The mass was easily separated by blunt dissection. Excision was performed in toto, and the majority of the ¯uid aspirated. There seemed to be capsular remnants ®rmly attached to the retroperitoneum at the midline, very close to the renal hilus where some diuse oozing was observed. No active source of bleeding was found. A closed drainage system was left in the right retroperitoneal space for 48 h and then removed with no evidence of bleeding. The histological diagnosis was cystic lymphangioma composed of dilated lymphatic spaces surrounded by connective tissue that contained lymphocytes and
0020-1383/99/$ - see front matter # 1999 Elsevier Science Ltd. All rights reserved. PII: S 0 0 2 0 - 1 3 8 3 ( 9 9 ) 0 0 0 9 9 - 6
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
Fig. 1. Intravenous pyelogram obtained on the operating table. Note bilateral excretion of contrast material without evidence of extravasation. The right abdomen is opaque and all bowel loops are displaced to the left abdomen.
erythrocytes. The huge cystic spaces were lined with cuboidal cells. The postoperative course was uneventful. Oral nutrition was started on day 4, and the patient was discharged on day 8 in good general condition. Follow-up sonography at four weeks, and six months showed no signs of recurrence.
3. Discussion We report a rare case in which an asymptomatic retroperitoneal lymphangioma became life-threatening and required emergency laparotomy following a minor trauma. Lymphangiomas are benign tumors which may be classi®ed into three histological types [1,2]: (1) lymphangioma simplex, consisting of a ®lmy mass of thinwalled, dilated lymphatics with a rich cellular connective tissue stroma which appear as small vesicles protruding into the epidermis; (2) cavernous
381
lymphangiomas, which are soft compressible tumors of dilated lymph vessels and lymphoid stroma invading the surrounding tissue; and (3) cystic lymphangiomas or cystic hygromas, which are poorly circumscribed, multilocular cystic masses containing serous, serosanguineous or chylous ¯uid. The masses range from 1 mm to 5 cm in diameter and often communicate with each other. Microscopically there are countless dilated lymphatic channels, often between lobules of adipose tissue. During the third or fourth month of embryonic development, two paired and two unpaired endothelial sacs form the primordia of the lymphatic system. Two jugular sacs in the neck, one sac in the mesenteric root, the cysterna chyli and two posterior sacs which appear at the bifurcation of the femoral and sciatic veins; the latter later form the retroperitoneal lymphatic system [3]. There is clinical and experimental evidence of retroperitoneal lymphaticovenous communications and pathways [4±6]. The most widely accepted etiological theory of cystic lymphangioma is early developmental sequestration of lymphatic vessels that fail to establish connections with normal draining lymphatics and became markedly dilated under the pressure of accumulating lymph [2]. A similar mechanism is thought to produce cavernous and simple lymphangioma, the site of lymphatic obstruction determining the pathologic type. About one-third of lymphangiomas occur in the neck and head. Cutaneous lymphangioma may occur in any area of the skin [7]. Uncommon sites include the groin, bone, mediastinum, uterus, stomach, small intestine, colon, rectum, omentum, mesentery, spleen, liver and retroperitoneal space [1,8,9]. The incidence of abdominal lymphangioma is unknown, but estimates vary from one case in 27,000 to one in 250,000 hospital admissions [10]. Of the 185 cases of abdominal lymphatic cysts reviewed by Burnett et al. [11] in 1950, only 5.4% were retroperitoneal. Skandalakis et al. [3] suggested that some retroperitoneal cysts are almost certainly of nonlymphatic origin. Though similar in structure to cysts within the leaves of the mesentery, if glomerular remnants can be demonstrated, they are likely to be nephrogenic rather than lymphatic [12]. The majority derive from the embryonic retroperitoneal lymph sac. Cystic retroperitoneal lymphangiomas usually present in infancy: 50±60% during the ®rst year of life and up to 90% by the second year [13]. The most common clinical manifestation is a palpable mass [14,15] which may appear suddenly and grow rapidly, then stop growing or even regress spontaneously. The presence of acute symptoms, such as abdominal pain, tenderness, guarding, fever, and leukocytosis, may indicate either obstruction (volvulus, extrinsic compression or entrapment in the pelvis) or peritonitis
382
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
Fig. 2. Huge black-bluish cystic mass protrudes into the right gutter, medially displacing and elevating the ascending colon.
(rupture, torsion, hemorrhage into the cyst or infected cyst), both of which require emergency surgery [14]; otherwise, extensive work-up is performed. Ultrasonography has high sensitivity and speci®city for a cystic abdominal mass [16]. Computerized tomography and magnetic resonance imaging may help, but the dierential diagnosis can be extensive [16,17]. Complete excision is possible in the majority of cases since, by de®nition, the cysts are not connected with any structure except loose areolar tissue [14]. When mesenteric cysts lie in close contact with the bowel, the attached bowel may require resection along with the cyst. If the cyst cannot be removed safely, internal marsupialization is indicated. Simple aspiration is associated with high rates of infection and recurrence. Prognosis is believed to be favorable, but there are no reports on recurrence rates. In one follow-up of 20 patients for a mean period of 23 months, 14% had a recurrence of intra-abdominal disease, and 9% (2 cases) had extra-abdominal lymphangioma [14]. One patient followed for 17 years after partial resection of mesenteric cysts had diuse extra-abdominal lymphangiomas necessitating many hospitalizations for infectious complications [14]. We speculate that in our case the ostensibly minor trauma ruptured a lumbar or other small retroperitoneal vein(s) closely connected by embryological pathways to a lymphangioma which may otherwise have
remained asymptomatic. The patient had undergone surgery for cutaneous lymphangioma two years earlier. We found only one similar case of an association between cutaneous and retroperitoneal lymphangioma in the English literature [18]. References [1] Koshy A, Tandon RK, Kapur BML, et al. Retroperitoneal lymphangioma. Am J Gastroenterol 1978;69:485±90. [2] Leonidas JC, Brill WB, Bhan I, et al. Cystic retroperitoneal lymphangioma in infants and children. Radiology 1978;127:203± 8. [3] Skandalakis JE, Gray SW, Ricketts RR. The lymphatic system. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. Baltimore, Hong Kong, London, Sydney: Williams and Wilkins, 1994. p. 877±97. [4] Pick JW, Anson BJ, Burnett HW. Communications between lymphatic and venous systems at renal level in man. Q Bull Northwest Univ Med School 1944;18:307±16. [5] Pressman JJ, Simon MB. Experimental evidence of direct communications between lymph nodes and veins. Surg Gynecol Obstet 1961;113:537±41. [6] Roddenberry H, Allen L. Observations on the abdominal lymphaticovenous communications of the squirrel monkey (Saimiri sciureus). Anat Rec 1967;159:147±58. [7] Flanagan BP, Helwig EB. Cutaneous lymphangioma. Arch Dermatol 1977;113:24±30. [8] Asch MJ, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery 1974;76:334±9. [9] Larson DL, Myhre BA, Scmidt ER. Lymphangiomatosis in
E. Freud et al. / Injury, Int. J. Care Injured 30 (1999) 380±383
[10] [11] [12] [13] [14]
unusual sites: spleen, mesentery, retroperitoneum, mediastinum and greater omentum. Wisc Med J 1931;60:279±87. Moore TC. Congenital cysts of the mesentery. Ann Surg 1957;145:128±31. Burnett WE, Rosemond GP, Bucher RM. Mesenteric cysts: report of 3 cases, in one of which a calci®ed cysts was present. Arch Surg 1950;60:699±706. Maury JM. Retroperitoneal cysts of wolan origin. Surg Gynecol Obstet 1918;26:663. Rekhi BM, Esselstyn Jr BC, Levy I. Retroperitoneal cystic lymphangioma: report of two cases and review of the literature. Clev Clin Q 1972;39:125±8. Hebra A, Brown MF, McGeehin KM, Ross AJ. Mesenteric,
[15] [16] [17] [18]
383
omental and retroperitoneal cysts in children: a clinical study of 22 cases. South Med J 1993;86:173±6. Vanek VW, Philips AK. Retroperitoneal, mesenteric and omental cysts. Arch Surg 1984;119:838±42. Davidson AJ, Hartman DS. Lymphangioma of the retroperitoneum: CT and sonographic characteristics. Radiology 1990;175:507±10. Feldberg MA, Hendriks AV, Van Leeuwen MS, et al. Retroperitoneal cystic lymphangioma section imaging in two cases and review of the literature. Clin Imaging 1990;14:26±30. Irvine AD, Sweeney L, Corbett JR. Lymphangioma circumscriptum associated with paravesical cystic retroperitoneal lymphangioma. Br J Dermatol 1996;134:1135±7.