Wandering spleen as a cause of sinistral portal hypertension

Wandering spleen (WS) is a rare entity characterized by laxity of peritoneal ligaments that hold the spleen stationary. It is most commonly diagnosed in children and young women. Clinical presentation ranges from asymptomatic to acute abdomen. A 19-year-old woman came to the emergency department with history of progressive abdominal pain. She also had previous episodes of hematemesis. A computed tomography scan showed an ectopic spleen with a "whirlpool sign." Laparotomy and splenectomy were performed. WS is characterized by a long vascular pedicle and laxity of peritoneal attachments of the spleen. The etiology is usually congenital. Splenopexy is the main treatment; however, splenectomy is indicated when splenic infarction is present. Despite being rare, this condition may be considered in some cases of abdominal pain. An earlier diagnosis would have allowed us to perform a splenopexy, thus reducing morbidity. (AU)

Bazo errante gigante con torsión del pedículo e infarto esplénico: reporte de un caso / Giant Wandering Spleen with torsion of the pedicle and splenic infarction: a case report

INTRODUCCIÓN: El bazo errante, es una entidad clínica poco común. Su espectro clínico varía desde enfermedad asintomática hasta complicaciones asociadas y su manejo es predominantemente quirúrgico. CASO CLÍNICO: Hombre, con cuadro clínico de dolor y masa abdominal palpable, con hallazgos tomográficos sugestivos de patología con asiento en retroperitoneo, con obstrucción intestinal secundaria; por laparotomía se identifica de forma incidental bazo ectópico solo fijado a través de pedículo vascular torsionado y signos de hipertensión portal, realizándose esplenectomía. CONCLUSIONES: La torsión esplénica es la complicación más frecuente del bazo errante, una entidad bastante rara con muy pocos casos publicados en Colombia.

BACKGROUND: The wandering spleen is an uncommon clinical entity. Its clinical spectrum varies from asymptomatic disease to associated complications and its management is predominantly by surgery. CLINICAL CASE: Man with clinical picture of pain and palpable abdominal mass, with tomographic findings suggestive of pathology with retroperitoneal seating, with secondary intestinal obstruction; by laparotomy incidentally, an ectopic spleen is identified, only fixed through a torsioned vascular pedicle and signs of portal hypertension, performing splenectomy. CONCLUSIONS: Splenic torsion is the most frequent complication of the errant spleen, a very rare entity with very few cases published in Colombia.

Bazo errante: una causa infrecuente de abdomen agudo / Wandering spleen: an unusual cause of acute abdomen

El síndrome de bazo errante es una condición infrecuente en la cual existe ausencia o hiperlaxitud en los elementos de fijación esplénicos que predispone a una ubicación inusual en el abdomen y mayor riesgo de torsión e infarto visceral. Su etiología puede ser congénita o adquirida y la presentación clínica es variable. El diagnóstico se basa en sospecha clínica, laboratorio y estudios por imágenes. La cirugía es el único tratamiento definitivo para esta afección. Presentamos el caso de una mujer de 23 años de edad con antecedentes de episodios recurrentes de dolor en hipocondrio izquierdo desde la infancia. Al examen físico se encontraba hemodinámicamente estable, afebril y con dolor en hipocondrio izquierdo. La ecografía mostró esplenomegalia homogénea y la tomografía de abdomen evidenció un bazo aumentado de tamaño, de posición conservada, con arremolinamiento y congestión de los vasos en el hilio esplénico. Se interpretó el cuadro como isquemia esplénica secundaria a torsión del pedículo vascular. Se realizó laparoscopia exploradora que evidenció bazo de 18 cm libre en hipocondrio izquierdo, con venas varicosas en la periferia y sin ligamentos de fijación. Se realizó esplenectomía por vía laparoscópica. La anatomía patológica informó necrosis isquémica del órgano. Evolucionó favorablemente con alta hospitalaria al tercer día postoperatorio.

Wandering spleen syndrome is a rare condition in which absence or laxity of splenic fixing elements predisposes to an unusual location in the abdomen and an increasing risk of twisting and infarction. Its etiology may be congenital or acquired and clinical presentation is variable. Diagnosis is based on clinical suspicion, laboratory and imaging. Surgery is the only definitive treatment for this pathology. We report the case of a 23 year old woman with a history of recurrent episodes of abdominal pain in the left upper quadrant since childhood. On physical examination she was afebrile, hemodynamically stable, with marked abdominal tenderness in the left upper quadrant. Ultrasonography showed homogeneous splenomegaly. Abdominal CT-scan presented an enlarged, eutopic spleen, with swirling and congestion of hilum vessels. Splenic ischemia due to organ torsion was suspected. Exploratory laparoscopy was performed showing an 18 cm in diameter spleen free in left upper quadrant, with varicose veins in the periphery and without fixing ligaments. Laparoscopic splenectomy was completed. The pathology report showed ischemic necrosis of the organ. The patient progressed favorably and was discharged on the third postoperative day.

Wandering spleen presenting as recurrent abdominal pain in a young female.

A young female child presented to our OPD for evaluation of recurrent abdominal pain. During the process of investigation USG abdomen and subsequently CECT abdomen revealed pelvic location of spleen. Splenopexy was performed and patient discharged. Patient is asymptomatic and on regular follow up.

Sinal de enovelamento ("whirl-sign") em baço errante: relato de caso / The whirl-sign in the wandering spleen: a case report

O baço errante é uma rara anomalia congênita ou adquirida que pode causar sintomas abdominais inespecíficos. Dentre suas complicações, a torção do pedículo vascular constitui emergência cirúrgica e deve ser reconhecida. Relatamos o caso de uma jovem com dor abdominal aguda e massa palpável que demonstrou, à tomografia computadorizada, ter o baço fora da topografia usual, sem realce pelo contraste, associado à estrutura adjacente em espiral produzida pela torção do pedículo esplênico (“whirl-sign”).

Wandering spleen is a rare congenital or acquired anomaly that can cause nonespecific abdominal symptoms. Among its complications, torsion of the vascular pedicle is a surgical emergency, and must be recognized. We report a case of a young woman with acute abdominal pain and a palpable mass, whose computerized thomography revealed an ectopic spleen without enhancement, and a circular, whorled structure that proved to be the torsion of vascular pedicle (whirl-sign).

An unusual case of hemoperitoneum owing to acute splenic torsion in a child with immunoglobulin deficiency.

Wandering spleen is an uncommon clinical entity, which rarely affects children and adolescents. It is usually described in adults, being most common in the multiparous women of childbearing age. A case of a 14-year-old girl with a past history of splenomegaly and immunoglobulin A (IgA) deficiency, who presented with a sudden onset of abdominal pain, is presented. Diagnosis of hemoperitoneum secondary to torsion of a wandering spleen was made by computed tomography scan and Doppler ultrasound. Laparoscopy revealed hemoperitoneum owing to a ruptured and infarcted spleen. Laparotomy was undertaken and open splenectomy was successfully performed. The patient was discharged after an uneventful postoperative course that was not punctuated by any major complication. Management of this rare surgical emergency is discussed. Based on the details of this case, the authors hypothesize that IgA deficiency causes splenomegaly, which in turn predisposes to ligamentous laxity and splenic torsion.