Massive splenomegaly caused by cavernous hemangiomas associated with Klippel-Trenaunay syndrome: report of a case. 2014

Takeyuki Misawa, and Hiroaki Shiba, and Yuki Fujiwara, and Yasuro Futagawa, and Toru Harada, and Masahiro Ikegami, and Katsuhiko Yanaga
Department of Surgery, The Jikei University School of Medicine, 3-25-8 Nishi-shinbashi, Minato-ku, Tokyo, 105-8461, Japan, take-misawa@jikei.ac.jp.

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.

UI MeSH Term Description Entries
D007715 Klippel-Trenaunay-Weber Syndrome A congenital disorder that is characterized by a triad of capillary malformations (HEMANGIOMA), venous malformations (ARTERIOVENOUS FISTULA), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the VG5Q gene which encodes a strong angiogenesis stimulator. Klippel-Trenaunay Disease,Angio-Osteohypertrophy Syndrome,Angioosteohypertrophy Syndrome,Congenital Dysplastic Angiopathy,KTW Syndrome,Klippel Trenaunay Syndrome,Klippel-Trenaunay Syndrome,Klippel-Trénaunay-Weber Syndrome,Angio Osteohypertrophy Syndrome,Angio-Osteohypertrophy Syndromes,Angioosteohypertrophy Syndromes,Angiopathies, Congenital Dysplastic,Angiopathy, Congenital Dysplastic,Congenital Dysplastic Angiopathies,Disease, Klippel-Trenaunay,Dysplastic Angiopathies, Congenital,Dysplastic Angiopathy, Congenital,KTW Syndromes,Klippel Trenaunay Disease,Klippel Trenaunay Weber Syndrome,Klippel Trénaunay Weber Syndrome,Klippel-Trenaunay Syndromes,Syndrome, Angio-Osteohypertrophy,Syndrome, Angioosteohypertrophy,Syndrome, KTW,Syndrome, Klippel Trenaunay,Syndrome, Klippel-Trenaunay,Syndrome, Klippel-Trenaunay-Weber,Syndrome, Klippel-Trénaunay-Weber,Syndromes, Angio-Osteohypertrophy,Syndromes, Angioosteohypertrophy,Syndromes, KTW,Syndromes, Klippel-Trenaunay
D005260 Female Females
D006392 Hemangioma, Cavernous A vascular anomaly that is a collection of tortuous BLOOD VESSELS and connective tissue. This tumor-like mass with the large vascular space is filled with blood and usually appears as a strawberry-like lesion in the subcutaneous areas of the face, extremities, or other regions of the body including the central nervous system. Angioma, Cavernous,Cavernous Angioma,Cavernous Hemangioma,Strawberry Hemangiomas,Cavernous Hemangiomas,Hemangioma, Strawberry,Hemangiomas, Cavernous,Hemangiomas, Strawberry,Strawberry Hemangioma
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013156 Splenectomy Surgical procedure involving either partial or entire removal of the spleen. Splenectomies
D013160 Splenic Neoplasms Tumors or cancer of the SPLEEN. Cancer of Spleen,Spleen Cancer,Cancer of the Spleen,Neoplasms, Splenic,Spleen Neoplasms,Splenic Cancer,Cancer, Spleen,Cancer, Splenic,Cancers, Spleen,Cancers, Splenic,Neoplasm, Spleen,Neoplasm, Splenic,Neoplasms, Spleen,Spleen Cancers,Spleen Neoplasm,Splenic Cancers,Splenic Neoplasm
D013163 Splenomegaly Enlargement of the spleen. Enlarged Spleen,Spleen, Enlarged
D015746 Abdominal Pain Sensation of discomfort, distress, or agony in the abdominal region. Colicky Pain,Abdominal Pains,Colicky Pains,Pain, Abdominal,Pain, Colicky,Pains, Abdominal,Pains, Colicky
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes

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