Surg Today (2014) 44:197–200 DOI 10.1007/s00595-013-0779-y
CASE REPORT
Massive splenomegaly caused by cavernous hemangiomas associated with Klippel–Trenaunay syndrome: report of a case Takeyuki Misawa • Hiroaki Shiba • Yuki Fujiwara • Yasuro Futagawa • Toru Harada • Masahiro Ikegami Katsuhiko Yanaga
•
Received: 5 March 2013 / Accepted: 20 August 2013 / Published online: 20 November 2013 Ó Springer Japan 2013
Abstract The etiology of Klippel–Trenaunay syndrome (KTS) is not well understood. Although splenic involvement is very rare in KTS, life-threatening events such as spontaneous rupture of a splenic hemangioma may occur. We recently performed elective splenectomy for massive splenomegaly causing uncontrollable abdominal pain in a woman with KTS. The extracted spleen weighed 4260 g, and cavernous hemangiomas in the spleen were found to be the cause of the splenomegaly. The patient’s abdominal pain resolved after surgery and her postoperative course was uneventful, except for persistent bleeding from the bladder. This is a rare case of KTS with associated severe splenomegaly caused by hemangiomas.
associated with vascular malformations in visceral organs, the spleen is rarely involved [2]. Splenomegaly is unusual in KTS, although there are reports of minor splenic congestion [3] or splenomegaly associated with a mixture of hemangioma and lymphangioma [4]. Although involvement of the spleen is rare, life-threatening events such as spontaneous rupture of splenic hemangioma may occur in patients with KTS [4]. Therefore, elective splenectomy might be recommended for KTS patients with hemangiomas of the spleen. We report the case of a woman with KTS, who underwent elective splenectomy for massive splenomegaly associated with hemangioma causing uncontrollable abdominal pain.
Keywords Klippel–Trenaunay syndrome Splenomegaly Splenectomy Hemangioma
Case report
Introduction Klippel–Trenaunay syndrome (KTS) is a rare clinical entity characterized by hypertrophy of the bone and soft tissue, cutaneous hemangiomas, and varicose veins and venous malformations [1]. While KTS is sometimes
T. Misawa (&) H. Shiba Y. Fujiwara Y. Futagawa K. Yanaga Department of Surgery, The Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo 105-8461, Japan e-mail: [email protected] T. Harada M. Ikegami Department of Pathology, The Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo 105-8461, Japan
The patient was a slim 40-year-old woman (height, 168 cm; weight, 51 kg; BMI, 18.1), with diagnosed KTS and massive splenomegaly. She presented to a local hospital with severe dull abdominal pain, presumably due to compression by the splenomegaly, which was treated by periodic transdermal fentanyl patches and occasional oral or intravenous morphine on demand. This uncontrollable pain was compromising her activity of daily life (ADL) remarkably. Physical examination revealed severe abdominal distension with upper abdominal tenderness and a palpable enlarged spleen. We noted deformity and hypotrophy of the toes, as well as scars from more than ten operations for varicose veins in her left lower legs (Fig. 1). Other history included hemorrhoidectomy for persistent anal bleeding requiring blood transfusions, and endoscopic coagulation for bleeding from hemangiomas of the bladder. The preoperative peripheral blood picture showed white blood cells 4.5 9 103/ll, red blood cells 3.69 9 106/ll,
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hemoglobin 9.2 g/dl, platelets 111 9 103/ll, aPTT 30.6 s, PT-INR 1.0, and antithrombin 84 %. The results of other biochemical tests were normal. Preoperative computed tomography (CT) showed massive splenomegaly with multiple low-density internal nodules, diagnosed as diffuse hemangiomas in the spleen. Aneurysmal dilatation of the superior mesenteric vein and extrahepatic portal vein without proximal obstructive lesions were also recognized. Diffuse mural thickening and multifocal calcifications in the pelvis, compatible with the development of severe varicose veins and phleboliths of the rectum, colon, mesentery, bladder, and vagina were other notable findings on
Fig. 1 Deformity of the toes of the left lower extremity
Fig. 2 a Preoperative computed tomography showed massive splenomegaly (asterisks) with multiple lowdensity internal nodules. b Aneurysmal dilatation of the extrahepatic portal vein (white arrows). c Diffuse multiple mural thickening and multifocal calcifications in the pelvis were compatible with the development of severe varicose veins and phleboliths of the rectum, colon, mesentery, bladder, and vagina
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CT (Fig. 2). To relieve the intolerable, uncontrollable abdominal pain, we decided to perform elective splenectomy, but only with carefully informed consent, after explaining the risk of postoperative disseminated intravascular coagulation or portal thrombosis. Laparotomy was performed via a longitudinal midline incision with a left transverse incision, revealing a massive spleen with an uneven surface. After dissecting the gastrocolic and gastrosplenic ligaments, the splenic artery was ligated to prevent bleeding from the spleen. After dissecting the remnant ligamentous attachments around the spleen, we used a linear stapler to transect the splenic artery and the splenic vein, at the splenic hilum. There were enormous hemangiomas in all parts of the pelvis, including the sigmoid colon, rectum, mesocolon, and bladder. The intraoperative blood loss and operation time were 100 g and 119 min, respectively. Macroscopically, the removed spleen was huge, measuring 36 cm 9 26 cm and weighing 4260 g. The capsule of the spleen was grayish with an uneven surface. Its cut surface revealed enlarged and irregular vessels of various sizes (Fig. 3). Postoperatively, insignificant thrombi formed in the portal vein and the pulmonary vein, but resolved during heparin administration. Although her postoperative course was satisfactory and the abdominal pain disappeared, the patient was transferred to the Department of Urology for persistent macroscopic hematuria on postoperative day 18. The bleeding from the hemangioma of the bladder was successfully treated by endoscopic neodymium:yttrium
Surg Today (2014) 44:197–200
199
Fig. 3 a The removed spleen was remarkably enlarged, measuring 36 cm 9 26 cm and weighing 4260 g. b Cut surface of the spleen revealed enlarged vessels of various sizes
Fig. 4 a The splenic parenchyma was extensively replaced by proliferated cavernous vessels filled with erythrocytes (H&E stain; 920). b Within the thickened vessel wall (asterisks), consisting of collagenous tissue stained blue, many spindle cells were stained red, indicating the existence of muscle tissue (Masson’s trichrome
staining; 9200). c The same part of the wall (asterisks) as in Fig. 3b was stained brown, indicating the existence of smooth muscle cells (alpha-smooth muscle actin stain; 9200). d The wall of the vessels stained brown indicated the existence of endothelial cells (CD31 stain; 9100)
aluminum garnet (Nd:YAG) laser irradiation, and the patient was discharged 40 days after the splenectomy.
erythrocytes (Fig. 4a). The spindle cells in the wall of these vessels were stained red in Masson’s trichrome staining (Fig. 4b). Immunohistochemical analysis revealed that the majority of the vessel walls were stained positively for both alpha-smooth muscle actin (Fig. 4c) and CD 31 (Fig. 4d). However, the vessels were not significantly positive for D2-40 (data are not shown). These results were compatible with a diagnosis of splenomegaly caused by diffuse cavernous hemangiomas of the spleen.
Pathological findings Microscopically, the normal splenic structure of red pulp and white pulp was destroyed. The entire splenic parenchyma was diffusely replaced by the lesion with the proliferation of cavernous large and small vessels filled with
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Discussion In 1900, noted French physicians Klippel and Trenaunay described a syndrome in two patients presenting with a port-wine stain and varicosities of an extremity associated with hypertrophy of the affected limb’s bony and soft tissue. KTS is sometimes associated with other visceral vascular malformations, such as hemangiomas in the colon [5], urinary bladder [6], spleen [7], and central nervous system [8]. However, KTS is rarely associated with splenomegaly and in the few reported cases, splenomegaly was not significantly attributed to congestion [3] or a mixture of hemangioma and lymphangioma [4, 9]. Furthermore, patients rarely present with uncontrollable abdominal pain caused by massive splenomegaly. In the present case, microscopic examination revealed that the splenic parenchyma was diffusely replaced by the proliferated vessels. Many spindle cells in the wall of vessels were positive in Masson’s trichrome staining, representing the existence of muscle tissue. Immunohistochemically, the wall of the vessels was positive for alpha-smooth muscle actin staining, which indicates the existence of smooth muscle. The walls were also positive for CD31 staining, which is used primarily to demonstrate the presence of endothelial cells. On the other hand, most of the vessel walls were negative for D2-40, which primarily stains the endothelium of lymphatic channels but not of blood vessels. These pathological findings strongly suggest that the massive splenomegaly in our patient was predominantly caused by a diffuse hemangioma rather than by lymphangioma or congestion. This case of massive splenomegaly caused by diffuse hemangiomas in the spleen is, therefore, exceedingly rare. Complications of splenic hemangioma include hypersplenism, rupture, and malignant degeneration [10]. Although involvement of the spleen is unusual, life-threatening events such as spontaneous rupture of a splenic hemangioma may occur in KTS. Karakayali et al. [11] reported a case of serious morbidity in a KTS patient, caused by the rupture of hemangiomas of the spleen. Generally, the prognosis associated with splenic hemangiomas is good and clinical follow-up is not usually possible without surgical intervention; however, even minor trauma can cause spontaneous rupture, resulting in high morbidity and mortality. Moreover, when there are symptoms of compression, anemia, and pain, splenectomy may be indicated, especially for giant hemangiomas. Therefore, elective splenectomy might be recommended for patients with KTS and hemangiomas of the spleen or splenomegaly
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causing compression symptoms [4]. Postoperative blood examinations, including red blood cell and platelet counts did not show remarkable change in our patient. This suggests that the splenic function, such as the filtering of effete erythrocytes or the storage of platelets, was already insufficient in the red pulp of the splenic parenchyma since the whole spleen was occupied and replaced by hemangioma. The aneurysmal dilatation of the portal vein might also have contributed to the postoperative development of portal vein thrombus, which was transient and successfully treated by heparin infusion. Conflict of interest None of the authors have any conflict of interest to declare and have no financial relationship with the organization that sponsored this research.
References 1. Klippel M, Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med. 1900;185:641–50. 2. Choi YJ, Jee SR, Park KS, Ryu CH, Seo HR, Ha SI, et al. Involvement of splenic hemangioma and rectal varices in a patient with Klippel–Trenaunay syndrome. Korean J Gastroenterol. 2011;58:157–61. 3. Hamano K, Hiraoka H, Kouchi Y, Fujioka K, Esato K. Klippel– Trenaunay syndrome associated with splenomegaly: report of a case. Surg Today. 1995;25:272–4. 4. Jindal R, Sullivan R, Rodda B, Arun D, Hamady M, Cheshire NJ. Splenic malformation in a patient with Klippel–Trenaunay syndrome: a case report. J Vasc Surg. 2006;43:848–50. 5. Gharemani GG, Kangarloo H, Volberg F, Meyers MA. Diffuse cavernous hemangioma of the colon in the Klippel–Trenaunay syndrome. Radiology. 1976;118:673–8. 6. Klein TW, Kaplan GW. Klippel–Trenaunay syndrome associated with urinary tract hemangiomas. J Urol. 1975;114:596–600. 7. Pakter RL, Fishman EK, Nussbaum A, Giargiana FA, Zerhouni EA. CT findings in splenic hemangiomas in the Klippel–Trenaunay–Weber syndrome. J Comput Assist Tomogr. 1987;11: 88–91. 8. Gourie-Devi M, Prakash B. Vertebral and epidural hemangioma with paraplegia in Klippel–Trenaunay–Weber syndrome. J Neurosurg. 1978;48:814–7. 9. Spasic´ M, Radovanovic´ D, Canovic´ D, Azanjac G, Djurdjevic´ P, Mitrovic´ S. Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Tre´naunay syndrome. Srp Arh Celok Lek. 2012;140:777–81. 10. Kocaman O, Alponat A, Aygu¨n C, Gu¨rbu¨z Y, Sarisoy HT, Celebi A, et al. Lower gastrointestinal bleeding, hematuria and splenic hemangiomas in Klippel–Trenaunay syndrome: a case report and literature review. Turk J Gastroenterol. 2009;20:62–6. 11. Karakayali F, Basaran C, Soy EA, Karakus S, Yabanoglu H, Moray G, et al. Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel–Trenaunay syndrome: report of a case. Surg Today. 2010;40:154–7.
CASE REPORT
Massive splenomegaly caused by cavernous hemangiomas associated with Klippel–Trenaunay syndrome: report of a case Takeyuki Misawa • Hiroaki Shiba • Yuki Fujiwara • Yasuro Futagawa • Toru Harada • Masahiro Ikegami Katsuhiko Yanaga
•
Received: 5 March 2013 / Accepted: 20 August 2013 / Published online: 20 November 2013 Ó Springer Japan 2013
Abstract The etiology of Klippel–Trenaunay syndrome (KTS) is not well understood. Although splenic involvement is very rare in KTS, life-threatening events such as spontaneous rupture of a splenic hemangioma may occur. We recently performed elective splenectomy for massive splenomegaly causing uncontrollable abdominal pain in a woman with KTS. The extracted spleen weighed 4260 g, and cavernous hemangiomas in the spleen were found to be the cause of the splenomegaly. The patient’s abdominal pain resolved after surgery and her postoperative course was uneventful, except for persistent bleeding from the bladder. This is a rare case of KTS with associated severe splenomegaly caused by hemangiomas.
associated with vascular malformations in visceral organs, the spleen is rarely involved [2]. Splenomegaly is unusual in KTS, although there are reports of minor splenic congestion [3] or splenomegaly associated with a mixture of hemangioma and lymphangioma [4]. Although involvement of the spleen is rare, life-threatening events such as spontaneous rupture of splenic hemangioma may occur in patients with KTS [4]. Therefore, elective splenectomy might be recommended for KTS patients with hemangiomas of the spleen. We report the case of a woman with KTS, who underwent elective splenectomy for massive splenomegaly associated with hemangioma causing uncontrollable abdominal pain.
Keywords Klippel–Trenaunay syndrome Splenomegaly Splenectomy Hemangioma
Case report
Introduction Klippel–Trenaunay syndrome (KTS) is a rare clinical entity characterized by hypertrophy of the bone and soft tissue, cutaneous hemangiomas, and varicose veins and venous malformations [1]. While KTS is sometimes
T. Misawa (&) H. Shiba Y. Fujiwara Y. Futagawa K. Yanaga Department of Surgery, The Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo 105-8461, Japan e-mail: [email protected] T. Harada M. Ikegami Department of Pathology, The Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo 105-8461, Japan
The patient was a slim 40-year-old woman (height, 168 cm; weight, 51 kg; BMI, 18.1), with diagnosed KTS and massive splenomegaly. She presented to a local hospital with severe dull abdominal pain, presumably due to compression by the splenomegaly, which was treated by periodic transdermal fentanyl patches and occasional oral or intravenous morphine on demand. This uncontrollable pain was compromising her activity of daily life (ADL) remarkably. Physical examination revealed severe abdominal distension with upper abdominal tenderness and a palpable enlarged spleen. We noted deformity and hypotrophy of the toes, as well as scars from more than ten operations for varicose veins in her left lower legs (Fig. 1). Other history included hemorrhoidectomy for persistent anal bleeding requiring blood transfusions, and endoscopic coagulation for bleeding from hemangiomas of the bladder. The preoperative peripheral blood picture showed white blood cells 4.5 9 103/ll, red blood cells 3.69 9 106/ll,
123
198
hemoglobin 9.2 g/dl, platelets 111 9 103/ll, aPTT 30.6 s, PT-INR 1.0, and antithrombin 84 %. The results of other biochemical tests were normal. Preoperative computed tomography (CT) showed massive splenomegaly with multiple low-density internal nodules, diagnosed as diffuse hemangiomas in the spleen. Aneurysmal dilatation of the superior mesenteric vein and extrahepatic portal vein without proximal obstructive lesions were also recognized. Diffuse mural thickening and multifocal calcifications in the pelvis, compatible with the development of severe varicose veins and phleboliths of the rectum, colon, mesentery, bladder, and vagina were other notable findings on
Fig. 1 Deformity of the toes of the left lower extremity
Fig. 2 a Preoperative computed tomography showed massive splenomegaly (asterisks) with multiple lowdensity internal nodules. b Aneurysmal dilatation of the extrahepatic portal vein (white arrows). c Diffuse multiple mural thickening and multifocal calcifications in the pelvis were compatible with the development of severe varicose veins and phleboliths of the rectum, colon, mesentery, bladder, and vagina
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CT (Fig. 2). To relieve the intolerable, uncontrollable abdominal pain, we decided to perform elective splenectomy, but only with carefully informed consent, after explaining the risk of postoperative disseminated intravascular coagulation or portal thrombosis. Laparotomy was performed via a longitudinal midline incision with a left transverse incision, revealing a massive spleen with an uneven surface. After dissecting the gastrocolic and gastrosplenic ligaments, the splenic artery was ligated to prevent bleeding from the spleen. After dissecting the remnant ligamentous attachments around the spleen, we used a linear stapler to transect the splenic artery and the splenic vein, at the splenic hilum. There were enormous hemangiomas in all parts of the pelvis, including the sigmoid colon, rectum, mesocolon, and bladder. The intraoperative blood loss and operation time were 100 g and 119 min, respectively. Macroscopically, the removed spleen was huge, measuring 36 cm 9 26 cm and weighing 4260 g. The capsule of the spleen was grayish with an uneven surface. Its cut surface revealed enlarged and irregular vessels of various sizes (Fig. 3). Postoperatively, insignificant thrombi formed in the portal vein and the pulmonary vein, but resolved during heparin administration. Although her postoperative course was satisfactory and the abdominal pain disappeared, the patient was transferred to the Department of Urology for persistent macroscopic hematuria on postoperative day 18. The bleeding from the hemangioma of the bladder was successfully treated by endoscopic neodymium:yttrium
Surg Today (2014) 44:197–200
199
Fig. 3 a The removed spleen was remarkably enlarged, measuring 36 cm 9 26 cm and weighing 4260 g. b Cut surface of the spleen revealed enlarged vessels of various sizes
Fig. 4 a The splenic parenchyma was extensively replaced by proliferated cavernous vessels filled with erythrocytes (H&E stain; 920). b Within the thickened vessel wall (asterisks), consisting of collagenous tissue stained blue, many spindle cells were stained red, indicating the existence of muscle tissue (Masson’s trichrome
staining; 9200). c The same part of the wall (asterisks) as in Fig. 3b was stained brown, indicating the existence of smooth muscle cells (alpha-smooth muscle actin stain; 9200). d The wall of the vessels stained brown indicated the existence of endothelial cells (CD31 stain; 9100)
aluminum garnet (Nd:YAG) laser irradiation, and the patient was discharged 40 days after the splenectomy.
erythrocytes (Fig. 4a). The spindle cells in the wall of these vessels were stained red in Masson’s trichrome staining (Fig. 4b). Immunohistochemical analysis revealed that the majority of the vessel walls were stained positively for both alpha-smooth muscle actin (Fig. 4c) and CD 31 (Fig. 4d). However, the vessels were not significantly positive for D2-40 (data are not shown). These results were compatible with a diagnosis of splenomegaly caused by diffuse cavernous hemangiomas of the spleen.
Pathological findings Microscopically, the normal splenic structure of red pulp and white pulp was destroyed. The entire splenic parenchyma was diffusely replaced by the lesion with the proliferation of cavernous large and small vessels filled with
123
200
Discussion In 1900, noted French physicians Klippel and Trenaunay described a syndrome in two patients presenting with a port-wine stain and varicosities of an extremity associated with hypertrophy of the affected limb’s bony and soft tissue. KTS is sometimes associated with other visceral vascular malformations, such as hemangiomas in the colon [5], urinary bladder [6], spleen [7], and central nervous system [8]. However, KTS is rarely associated with splenomegaly and in the few reported cases, splenomegaly was not significantly attributed to congestion [3] or a mixture of hemangioma and lymphangioma [4, 9]. Furthermore, patients rarely present with uncontrollable abdominal pain caused by massive splenomegaly. In the present case, microscopic examination revealed that the splenic parenchyma was diffusely replaced by the proliferated vessels. Many spindle cells in the wall of vessels were positive in Masson’s trichrome staining, representing the existence of muscle tissue. Immunohistochemically, the wall of the vessels was positive for alpha-smooth muscle actin staining, which indicates the existence of smooth muscle. The walls were also positive for CD31 staining, which is used primarily to demonstrate the presence of endothelial cells. On the other hand, most of the vessel walls were negative for D2-40, which primarily stains the endothelium of lymphatic channels but not of blood vessels. These pathological findings strongly suggest that the massive splenomegaly in our patient was predominantly caused by a diffuse hemangioma rather than by lymphangioma or congestion. This case of massive splenomegaly caused by diffuse hemangiomas in the spleen is, therefore, exceedingly rare. Complications of splenic hemangioma include hypersplenism, rupture, and malignant degeneration [10]. Although involvement of the spleen is unusual, life-threatening events such as spontaneous rupture of a splenic hemangioma may occur in KTS. Karakayali et al. [11] reported a case of serious morbidity in a KTS patient, caused by the rupture of hemangiomas of the spleen. Generally, the prognosis associated with splenic hemangiomas is good and clinical follow-up is not usually possible without surgical intervention; however, even minor trauma can cause spontaneous rupture, resulting in high morbidity and mortality. Moreover, when there are symptoms of compression, anemia, and pain, splenectomy may be indicated, especially for giant hemangiomas. Therefore, elective splenectomy might be recommended for patients with KTS and hemangiomas of the spleen or splenomegaly
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causing compression symptoms [4]. Postoperative blood examinations, including red blood cell and platelet counts did not show remarkable change in our patient. This suggests that the splenic function, such as the filtering of effete erythrocytes or the storage of platelets, was already insufficient in the red pulp of the splenic parenchyma since the whole spleen was occupied and replaced by hemangioma. The aneurysmal dilatation of the portal vein might also have contributed to the postoperative development of portal vein thrombus, which was transient and successfully treated by heparin infusion. Conflict of interest None of the authors have any conflict of interest to declare and have no financial relationship with the organization that sponsored this research.
References 1. Klippel M, Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med. 1900;185:641–50. 2. Choi YJ, Jee SR, Park KS, Ryu CH, Seo HR, Ha SI, et al. Involvement of splenic hemangioma and rectal varices in a patient with Klippel–Trenaunay syndrome. Korean J Gastroenterol. 2011;58:157–61. 3. Hamano K, Hiraoka H, Kouchi Y, Fujioka K, Esato K. Klippel– Trenaunay syndrome associated with splenomegaly: report of a case. Surg Today. 1995;25:272–4. 4. Jindal R, Sullivan R, Rodda B, Arun D, Hamady M, Cheshire NJ. Splenic malformation in a patient with Klippel–Trenaunay syndrome: a case report. J Vasc Surg. 2006;43:848–50. 5. Gharemani GG, Kangarloo H, Volberg F, Meyers MA. Diffuse cavernous hemangioma of the colon in the Klippel–Trenaunay syndrome. Radiology. 1976;118:673–8. 6. Klein TW, Kaplan GW. Klippel–Trenaunay syndrome associated with urinary tract hemangiomas. J Urol. 1975;114:596–600. 7. Pakter RL, Fishman EK, Nussbaum A, Giargiana FA, Zerhouni EA. CT findings in splenic hemangiomas in the Klippel–Trenaunay–Weber syndrome. J Comput Assist Tomogr. 1987;11: 88–91. 8. Gourie-Devi M, Prakash B. Vertebral and epidural hemangioma with paraplegia in Klippel–Trenaunay–Weber syndrome. J Neurosurg. 1978;48:814–7. 9. Spasic´ M, Radovanovic´ D, Canovic´ D, Azanjac G, Djurdjevic´ P, Mitrovic´ S. Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Tre´naunay syndrome. Srp Arh Celok Lek. 2012;140:777–81. 10. Kocaman O, Alponat A, Aygu¨n C, Gu¨rbu¨z Y, Sarisoy HT, Celebi A, et al. Lower gastrointestinal bleeding, hematuria and splenic hemangiomas in Klippel–Trenaunay syndrome: a case report and literature review. Turk J Gastroenterol. 2009;20:62–6. 11. Karakayali F, Basaran C, Soy EA, Karakus S, Yabanoglu H, Moray G, et al. Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel–Trenaunay syndrome: report of a case. Surg Today. 2010;40:154–7.
