Extensive Retroperitoneal Lymphangiectasia With Bone Involvement: A Case Report
Isolated Extensive Retroperitoneal Lymphangiectasia With Bone Involvement.
A 21- year-old male presented with complaints of swelling over bilateral groin region, left more than right, since 2 months. There was an increase in the size of the swelling on walking and reduced while lying down. There was no history of loin pain, dysuria or facial puffiness. The patient gave no history of passing whitish urine nor any constitutional symptoms . There was no other significant past or medical history.
On local examination he had bilateral inguinal region more on the left side. Swelling showed no cough impulse / warmth/ tenderness. Scrotum was normal. Pulse rate and blood pressure were within normal limits. Laboratory investigations revealed a hemoglobin level of 14.6 g / dl. A total neutrophil count of 6,030 cell/ cu.mm, with 81.2 % neutrophils, 15.9 % lymphocytes and 0.7 % eosinophils. The erythrocyte sedimentation rate,serum creatinine and urea were normal. The urine routine examination was normal with absence of casts, dysmorphic red blood cells, white blood cells or malignant cells. 24 h urinary protein was within normal limits. Urine culture and blood culture were negative. Blood smear was negative for microfilaria. Ultrasound of the abdomen revealed multiple, ill-defined, cystic tubular areas encasing the aorta and Inferior vena cava in the retroperitoneum, extending into the left inguinal region. Both kidneys were normal (Fig. 1 ). The rest of the abdominal solid organs appeared to be normal. The patient then underwent magnetic resonance imaging. Magnetic Resonance Imaging of the abdomen showed fluid filled, tubular T2 hyperintense structures communicating with each other, extending along the course of retroperitoneal lymphatics - starting just below hilum of both kidneys along the infrarenal abdominal aorta, inferior vena cava into pelvis along the iliac vessels on both sides of urinary bladder upto bilateral inguinal region, left more than right (Fig. 2A and B ).There were T2 hyperintense lesions involving L4 Vertebral body, bilateral pelvic bones and sacrum (Fig. 3A and B ). The imaging findings were thus consistent with dilated lymphatics suggestive of extensive retroperitoneal lymphangiectasia with bone involvement. To confirm the diagnosis ultrasonography guided fine needle aspiration cytology of inguinal swelling was performed which confirmed it to be lymphatic fluid.
Retroperitoneal lymphangiectasia [RPL] is a very rare benign anomaly leading to abnormal dilatation of retroperitoneal lymphatic channels with changes commonly occurring in the kidneys and rest of the retroperitoneal tissues. Based on the location, it can be named as peri-pelvic lymphangiectasia, renal lymphangiectasia, renal lymphangiomatosis, renal lymphangioma, or polycystic disease of the renal sinus . Limited literature is available and the origin remains unclear . Retroperitoneal lymphangiectasia can be congenital or acquired Recently congenital lymphangiectasia is classified as low flow vascular malformations . It is mostly idiopathic in nature but sometimes results from the lymphatic obstruction secondary to granulomatous disease or malignancies . The most common cause for acquired obstructive dilatation of lymphatics seen in endemic regions is filariasis . Previous case reports have described lymphatic obstruction by filariasis causing extensive retroperitoneal, pelvic lymphangiectasia with intrathoracic extension . In our case, filariasis workup was negative. Other causes are trauma, pregnancy, neoplastic, inflammatory conditions like tuberculosis which were all negative in this case. Hence most likely cause in this case, is congenital. Most cases of RPL are asymptomatic and detected incidentally . Clinical evolution and prognosis are speculative . Radiological cross-sectional imaging studies are characteristic5and, vary based on the location of lymphangiectasia . Ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) are useful modalities in establishing the correct diagnosis. The disease is usually identified on CT as cystic structures along the route of the retroperitoneum. The presence of fluid density, non-enhancing nature of the cystic structures in the retroperitoneum on CECT, are suggestive of RPL. However, MRI better delineates the extent of involvement and is also radiation free. Usually bone involvement is extremely rare in retroperitoneal lymphangiectasia. Here we report one such case of retroperitoneal lymphangiectasia which was detected by MRI with associated bone involvement. Ultrasound, CT and MRI will show characteristic findings that will help the confirm diagnosis . Cystic nature of the lesion and its extent can be visualized on cross-sectional imaging such as CT and MRI . CT shows multiple cystic/ fluid attenuation lesions in retroperitoneum surrounding the retroperitoneal vessels and ureters without causing compression. The CT appearance can be confused with retroperitoneal fibrosis, however fluid density and tubular nature of these lesions, with extension along the anticipated line of lymphatics, absence of ureteric deviation and obstruction, help in differentiating it from retroperitoneal fibrosis. On MRI the dilated lymphatics appear as hyperintense, tubular structures communicating with each other extending along the antic
Retroperitoneal lymphangiectasia has characteristic imaging findings and can be easily diagnosed by ultrasound, CT and MRI. It has benign course, usually incidentally detected and rarely needs surgical intervention. This case is unique as to the best of our knowledge; congenital retroperitoneal lymphangiectasia with bone involvement, without any concomitant renal involvement has never been described previously.