Mènétrier ’ s disease treated with gastrectomy

A previously well 55-year old man presented to our unit with progressively worsening dyspnoea, swelling of the feet, and loss of weight for 3 months and passing black, tarry stools for 2 weeks. On examination he was pale, had bilateral pitting ankle oedema and hepatomegaly. The rest of the examination was unremarkable. Investigations showed Hb 7.4 g/dl, blood picture hypochromic microcytic erythrocytes, serum protein 5.6 g/dl, serum albumin 2.4g/dl (3.5-5), liver biochemistry otherwise normal, serum iron 2.3 (normal range 13-32umol/l). Upper gastrointestinal endoscopy showed giant polypoidal mucosal folds which are characteristic of Mènétrier's disease (figure 1). He did not respond to Helicobacter pylori eradication therapy. A total gastrectomy was performed. Apart from mild abdominal discomfort the patient has remained asymptomatic for the past 6 months. Mènétrier’s disease treated with gastrectomy


Introduction
Mènétrier's disease is a rare cause of protein losing enteropathy.We report a case that was successfully treated by gastrectomy.

Case report
A previously well 55-year old man presented to our unit with progressively worsening dyspnoea, swelling of the feet, and loss of weight for 3 months and passing black, tarry stools for 2 weeks.On examination he was pale, had bilateral pitting ankle oedema and hepatomegaly.The rest of the examination was unremarkable.Investigations showed Hb 7.4 g/dl, blood picture hypochromic microcytic erythrocytes, serum protein 5.6 g/dl, serum albumin 2.4g/dl (3.5-5), liver biochemistry otherwise normal, serum iron 2.3 (normal range 13-32umol/l).Upper gastrointestinal endoscopy showed giant polypoidal mucosal folds which are characteristic of Mènétrier's disease (figure 1).He did not respond to Helicobacter pylori eradication therapy.A total gastrectomy was performed.Apart from mild abdominal discomfort the patient has remained asymptomatic for the past 6 months.

Mènétrier's disease treated with gastrectomy
A P de Silva 1 , S Aryasingha 1 , A S Dassanayake 2 , J Hevavisenthi 3 , B G N Ratnasena 4 and H J de Silva 1   Departments of 1

Discussion
Mènétrier's disease was first described in 1888 by Pierre Mènétriers.It usually involves most of the stomach but spares the antrum (figure 2).Histologically the characteristic features are foveolar hyperplasia with cystic dilatation (figure 3).Mènétrier's disease is thought to carry Vol.53, No. 1, March 2008 Case report (Picture story) an increased risk for gastric cancer [1].H pylori eradication is recommended [2].Gastrectomy remains the final option in patients who do not respond to medical therapy.We chose gastrectomy because of his severe disease and nonresponse to standard therapy.

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Figure 1 .
Figure 1.Endoscopy view of stomach, with large convoluted folds.

Figure 3 .
Figure 3. Histological examination of the stomach showing the characteristic foveolar hyperplasia with cystic dilation of the surface mucous cells.

Figure 2 .
Figure 2. Gastrectomy specimen, with convoluted folds and sparing of the antrum.