Acta Pzdiatr Scand 65: 753-755, 1976
CASE REPORT
MENETRIER’S SYNDROME IN CHILDREN C. L. NEWMAN. J. P. McCLURE and J . F. R. BENTLEY
From the Department of Child Health and the Royal Hospitulfor Sick Children, Glasgow, Scotland
ABSTRACT. Newman, C. L., McClure, J. P. and Bentley, J. F. R. (Department of Child Health and Royal Hospital for Sick Children, Glasgow, Scotland). Menetrier’s Syndrome in Childhood. Acta Paediatr Scand, 65: 753, 1976.Eince Menetrier’s original description in 1888 many cases of hypertrophic gastritis have been recorded in adults. Only recently has a similar syndrome been described in childhood. The case reported demonstrates the characteristic features of the condition (the duration was rather longer than in other reported cases) and diagnosis, clinical course and aetiology are discussed. In contrast to the adult disease this illness is usually short-lived with full clinical and histological remission occurring within a few months. KEW WORDS: Menetrier’s syndrome, hypertrophic gastritis
In his original article in 1888 ( 5 ) Menetrier described adult patients with two different types of gastric mucosal abnormality. The first “polyadenomes polypeux” characterised by discrete sessile or pedunculated polyps, and the second “polyadenomes en nappe” where more widespread changes due to rugal hypertrophy (thought at the time to represent multiple flat adenomata) were seen. Since this original description there have been numerous reports of Menetrier’s syndrome in adults, but only recently has a similar syndrome been described in childhood ( 1 , 4 , 6).
CASE REPORT A. S., a female child who presented at 3 years of age with an 8-week history of recurrent epigastric pain, vomiting and loss of appetite. The vomit was occasionaly bloodstained and the passage of a malaena stool prompted her admission to hospital. On examination she was well nourished and her abdomen was soft and not distended. A mild degree of ankle oedema was present. The haemaglobin was 6.83 mmol/l, blood leukocyte count 9.1 K 10y/l and eosinophil count
0.64x10y/1 (7%). The plasma proteins were low, total 45 g/l and albumin 14.4 g/l. There was no evidence of hepatic or renal disease and plasma immunoglobulins were normal. The barium meal showed a grossly abnormal pattern (Fig. I ) with multiple filling defects suggesting malignant infiltration. At laparotomy a thickened gastric mucosa was seen with considerable overlying mucus. The mucosa was thrown up into large folds especially over the fundus and greater curvature but there was no demarkation between normal and abnormal tissue. On histological examination of a biopsy taken from the body of the stomach there was dilatation of the gastric glands, retention of mucus in the gland lumens and prominence of mucus secreting globlet cells. A moderate degree of inflammatory cell infiltration was also present and eosinophils were quite prominent. There was no evidence of malignant infiltration, the mucosal appearances were those of hypertrophic gastritis similar to that seen in Menetrier’s syndrome in adults. Following recovery from surgery the child’s symptoms slowly settled over the next 8 weeks but this clinical improvement was not paralleled by a rise in plasma proteins or improvement in the gastric mucosal appearance on barium X-ray study. These were still abnormal one year later and it was only towards the end of the second year of follow-up that the plasma proteins finally returned to normal and there was gastric mucosal improvement on X-ray. An 59Fe-labelled Dextran study performed the acute phase of the illness showed evidence of increased protein loss from the gastro-intestinal tract. Actu Pipdiutr Scand 65
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C . L . Newman et al.
Fig. I . Barium X-ray showing gastric rugal hypertrophy.
DISCUSSION In the adult Menetrier’s syndrome is a chronic non-remitting disease with a varying degree of morbidity and approximately an 8 % risk of secondary malignant change (2). In sharp contrast to this, in childhood the reports are of a transient condition usually lasting a few weeks or months (somewhat longer in the case described above) with full clinical and histological recovery. Of the 10 previously reported cases gastrointestinal protein loss occurred in the majority and hypoproteinaemic oedema was usually a presenting feature of the condition. This protein loss was shown by Citrin et al. ( 3 ) ,in adults, to be from the stomach. The aetiology of the mucosal changes remains obscure although with a periferal eosinophilia in most cases a hypersensitivity reaction has been suggested. A possible infective cause has also been postulated, and in one of three cases described by Lachmann in Actu PEdiutr Scund 65
1971 (4) cytomegalic inclusion bodies were found on histological examination of the gastric biopsy. The similarity of the barium X-ray changes of gastric mucosal hypertrophy to diffuse intragastric malignancy makes gastric biopsy necessary. In past cases this has been obtained at laparotomy following a provisional diagnosis of intragastric neoplasm. If a diagnosis of hypertrophic gastritis is considered endoscopy and superficial mucosa biopsy would probably give enough information to avoid a major surgical procedure for the diagnosis of what is a benign condition.
REFERENCES 1. Bums, B. & Gay, B. B.: Menetrier’s disease of the
stomach in children. A m J Roentgen01 Radium Ther Nucl Med, 103: 300, 1968. 2 . Chusid, F. L., Hirsch, R. L. & Colcher, H . : Hypertrophic gastropathy-a clinical review. A m J Gasfroenferol,43: 303, 1965.
Menetrier’s syndrome in children 3. Citrin, Y . , Sterling, K. & Halstead, J. A.: Mechanism of hypoproteinaemia associated with giant hypertrophy of gastric mucosa. N Engl J Med, 257:906, 1957. 4. Lachmann, R. S., Martin, D. J. & Vawter, G. F.: Thick gastric folds in childhood. A m J Roentgenol Radium Ther Nucl Med, 112: 83, 1971. 5. Menetrier, P.: Des polyadenomes gastriques et de leurs rapports avec le cancer de I’estomac. Arch de Phys, 4 : 3 2 , 1888.
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6. Schuster, W.: Clinical roentgenologic signs of giant hypertrophic gastritis in childhood (Menetrier’s disease). Ann Radio1 (Paris), 10: 221, 1967. Submitted Febr. 19, 1976 Accepted May 26, 1976 (C. L. N . ) Hospital for Sick Children Great Ormond Street London W C l N 3JH England
CASE REPORT
MENETRIER’S SYNDROME IN CHILDREN C. L. NEWMAN. J. P. McCLURE and J . F. R. BENTLEY
From the Department of Child Health and the Royal Hospitulfor Sick Children, Glasgow, Scotland
ABSTRACT. Newman, C. L., McClure, J. P. and Bentley, J. F. R. (Department of Child Health and Royal Hospital for Sick Children, Glasgow, Scotland). Menetrier’s Syndrome in Childhood. Acta Paediatr Scand, 65: 753, 1976.Eince Menetrier’s original description in 1888 many cases of hypertrophic gastritis have been recorded in adults. Only recently has a similar syndrome been described in childhood. The case reported demonstrates the characteristic features of the condition (the duration was rather longer than in other reported cases) and diagnosis, clinical course and aetiology are discussed. In contrast to the adult disease this illness is usually short-lived with full clinical and histological remission occurring within a few months. KEW WORDS: Menetrier’s syndrome, hypertrophic gastritis
In his original article in 1888 ( 5 ) Menetrier described adult patients with two different types of gastric mucosal abnormality. The first “polyadenomes polypeux” characterised by discrete sessile or pedunculated polyps, and the second “polyadenomes en nappe” where more widespread changes due to rugal hypertrophy (thought at the time to represent multiple flat adenomata) were seen. Since this original description there have been numerous reports of Menetrier’s syndrome in adults, but only recently has a similar syndrome been described in childhood ( 1 , 4 , 6).
CASE REPORT A. S., a female child who presented at 3 years of age with an 8-week history of recurrent epigastric pain, vomiting and loss of appetite. The vomit was occasionaly bloodstained and the passage of a malaena stool prompted her admission to hospital. On examination she was well nourished and her abdomen was soft and not distended. A mild degree of ankle oedema was present. The haemaglobin was 6.83 mmol/l, blood leukocyte count 9.1 K 10y/l and eosinophil count
0.64x10y/1 (7%). The plasma proteins were low, total 45 g/l and albumin 14.4 g/l. There was no evidence of hepatic or renal disease and plasma immunoglobulins were normal. The barium meal showed a grossly abnormal pattern (Fig. I ) with multiple filling defects suggesting malignant infiltration. At laparotomy a thickened gastric mucosa was seen with considerable overlying mucus. The mucosa was thrown up into large folds especially over the fundus and greater curvature but there was no demarkation between normal and abnormal tissue. On histological examination of a biopsy taken from the body of the stomach there was dilatation of the gastric glands, retention of mucus in the gland lumens and prominence of mucus secreting globlet cells. A moderate degree of inflammatory cell infiltration was also present and eosinophils were quite prominent. There was no evidence of malignant infiltration, the mucosal appearances were those of hypertrophic gastritis similar to that seen in Menetrier’s syndrome in adults. Following recovery from surgery the child’s symptoms slowly settled over the next 8 weeks but this clinical improvement was not paralleled by a rise in plasma proteins or improvement in the gastric mucosal appearance on barium X-ray study. These were still abnormal one year later and it was only towards the end of the second year of follow-up that the plasma proteins finally returned to normal and there was gastric mucosal improvement on X-ray. An 59Fe-labelled Dextran study performed the acute phase of the illness showed evidence of increased protein loss from the gastro-intestinal tract. Actu Pipdiutr Scand 65
154
C . L . Newman et al.
Fig. I . Barium X-ray showing gastric rugal hypertrophy.
DISCUSSION In the adult Menetrier’s syndrome is a chronic non-remitting disease with a varying degree of morbidity and approximately an 8 % risk of secondary malignant change (2). In sharp contrast to this, in childhood the reports are of a transient condition usually lasting a few weeks or months (somewhat longer in the case described above) with full clinical and histological recovery. Of the 10 previously reported cases gastrointestinal protein loss occurred in the majority and hypoproteinaemic oedema was usually a presenting feature of the condition. This protein loss was shown by Citrin et al. ( 3 ) ,in adults, to be from the stomach. The aetiology of the mucosal changes remains obscure although with a periferal eosinophilia in most cases a hypersensitivity reaction has been suggested. A possible infective cause has also been postulated, and in one of three cases described by Lachmann in Actu PEdiutr Scund 65
1971 (4) cytomegalic inclusion bodies were found on histological examination of the gastric biopsy. The similarity of the barium X-ray changes of gastric mucosal hypertrophy to diffuse intragastric malignancy makes gastric biopsy necessary. In past cases this has been obtained at laparotomy following a provisional diagnosis of intragastric neoplasm. If a diagnosis of hypertrophic gastritis is considered endoscopy and superficial mucosa biopsy would probably give enough information to avoid a major surgical procedure for the diagnosis of what is a benign condition.
REFERENCES 1. Bums, B. & Gay, B. B.: Menetrier’s disease of the
stomach in children. A m J Roentgen01 Radium Ther Nucl Med, 103: 300, 1968. 2 . Chusid, F. L., Hirsch, R. L. & Colcher, H . : Hypertrophic gastropathy-a clinical review. A m J Gasfroenferol,43: 303, 1965.
Menetrier’s syndrome in children 3. Citrin, Y . , Sterling, K. & Halstead, J. A.: Mechanism of hypoproteinaemia associated with giant hypertrophy of gastric mucosa. N Engl J Med, 257:906, 1957. 4. Lachmann, R. S., Martin, D. J. & Vawter, G. F.: Thick gastric folds in childhood. A m J Roentgenol Radium Ther Nucl Med, 112: 83, 1971. 5. Menetrier, P.: Des polyadenomes gastriques et de leurs rapports avec le cancer de I’estomac. Arch de Phys, 4 : 3 2 , 1888.
755
6. Schuster, W.: Clinical roentgenologic signs of giant hypertrophic gastritis in childhood (Menetrier’s disease). Ann Radio1 (Paris), 10: 221, 1967. Submitted Febr. 19, 1976 Accepted May 26, 1976 (C. L. N . ) Hospital for Sick Children Great Ormond Street London W C l N 3JH England
