Acta gastro-enterologica Belgica · 2016
Sclerosing mesenteric panniculitis in a young patient : common cause of diagnostic dilemma and treatment refractoriness.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Metha R, Gahlot G P S, Das P, Jindal G, Mouli V P, Madhusudhan K S, Sharma R, Pal S, Ahuja V, DattaGupta S
Аннотация
BACKGROUND: Sclerosing mesenteric panniculitis (SMP) is an idiopathic chronic fibroinflammatory disorder of the intra-abdominal fat.
CASE PRESENTATION: Herin, we report a case of SMP, involving the omentum, mesentery and peri-colic fat in a 18 year old male, who presented with significant and recurrent abdominal distension for 4.5 years. Computed tomogram revealed ascites, with nodular and irregular omental thickening and foci of calcification. Non-specific radiological and histological features made an accurate diagnosis extremely difficult. After a thorough work up and exclusion of other differentials, diagnosis of a nodular SMP (Weber Christian disease) was given. After showing resistance to chemotherapeutic agents, slow response was noted with cyclophosphamide, followed by rapid symptomatic improvement with mesenterectomy.
CONCLUSION: SMP is an uncommon benign mesenteric/ omental inflammation, and is a diagnosis of exclusion. As treatment refractoriness is common, management should be individualized and continued for along period. Surgical omentectomy may be helpful.
CASE PRESENTATION: Herin, we report a case of SMP, involving the omentum, mesentery and peri-colic fat in a 18 year old male, who presented with significant and recurrent abdominal distension for 4.5 years. Computed tomogram revealed ascites, with nodular and irregular omental thickening and foci of calcification. Non-specific radiological and histological features made an accurate diagnosis extremely difficult. After a thorough work up and exclusion of other differentials, diagnosis of a nodular SMP (Weber Christian disease) was given. After showing resistance to chemotherapeutic agents, slow response was noted with cyclophosphamide, followed by rapid symptomatic improvement with mesenterectomy.
CONCLUSION: SMP is an uncommon benign mesenteric/ omental inflammation, and is a diagnosis of exclusion. As treatment refractoriness is common, management should be individualized and continued for along period. Surgical omentectomy may be helpful.