Alimentary tract and pancreas      

Alimentarni trakt i pankreas

ARCH GASTROENTEROHEPATOL 2002; 21 ( No 1 – 2 ):

 

Endoscopic ultrasonoghraphy in  Menetrier ’s  disease: A case report

 

Endoskopska ultrasonorafija u Menetrijerovoj  bolesti: Prikaz slucaja

 

( accepted February 10th, 2002 )

 

¹Miodrag Krstić, ²Gradimir Golubović, ¹Marijan Micev, ¹ Predrag Dugalić, ¹Dragan Tomić, ¹Aleksandra Pavlović.

 

¹Institute of Digestive Diseases, Clinical Center of Serbia, Belgrade,

²Department of Internal Medicine. Clinical Hospital Zemun, Belgrade.

 

 

 

 

 

Abbreviations used in this article: :

LGF, large gastric folds; EUS, endoscopic ultrasound.

 

 

 

 

 

 

Addres correspondence to: Docent Dr Miodrag N. Krstic,

                                            Institute of Digestive Diseases,

                                            Clinical Center of Serbia,

                                             6 Koste Todorovica St, YU-11000 Belgrade

                                             Serbia, Yugoslavia

                                              E-mail: [email protected]

 

 

 

………………                                                             ----------------------------

EUS in Menetrier ^, s disease                                          Gastroenteroloska sekcija SLD-                                    

                                                                                      01730, 2002.   

 

 

ABSTRACT

 

The evaluation of patients with giant gastric rugal folds presents many problems to the gastroenterologist and pathologist. The variety of benign and malignant diseases must be considered in the differential diagnosis and all of them are difficult to separate by means of endoscopy and even biopsy. The unique ability of endoscopic ultrasound           ( EUS ) to visualize the all layers of the gut wall makes it very useful in assessing patients with large gastric folds. EUS can precisely define which layers are thickened, characterise the echo pattern of thickness and whether the layer structure is preserved.

Menetrier’s disease is a prototype disease wich caracterises deep mucosal thickening which can be clearly delineated  by EUS. Here, we report the very  first case of Menetrier’s disease which was assessed by EUS in our country. The role of EUS in differential diagnosis of large gastric folds is discussed in detail.

 

Key words: Menetrier’s disease, large gastric folds, EUS.

SAŽETAK

 

Evaluacija pacijenata sa  uvećanim želudačnim naborima je teška i za gastroenterologe i za patologe.  Veliki broj benignih i malignih stanja mora da se uzeme u razmatranje dok endoskopija, pa čak i biopsija najčešće ne mogu da napravi razliku izmedju njih. Endoskopski ultrazvuk je posebno precizan u prikazivanjui slojeva zida digestivnog trakta te se stoga se veoma uspešno koristi u ispitivanju pacijenata sa velikim želudačnim naborima. On može jasno da ukaže koji sloj je zadebljao, kakva je ehogenost zadebljanja i postoji li očuvan kontinuitet slojeva zida.

Menetrierova bolest je tipičan predstavnik ove grupe obolenja i ona se odlikuje značajnim  zadebljanjem dubokog dela mukoze koje se jasno prikazuje na endosonogafiji. U radu je prikazan prvi pacijent sa Menetrierovom bolešću koji je u našoj zemlji analiziran endoskopskim ultrazvukom. U radu je takodje detaljno diskutovana uloga EUS-a u diferencijalnoj  dijagnozi uvećanih nabora želuca.

 

Kljucne reci: Menetrierova bolest, uvećani želudačni nabori, endoskopska ultrasonografija.

 

Figure 1. Menetrier disease – endoscopic view of large gastric folds

In body and fundus

 

INTRODUCTION

The evaluation of the patients with giant gastric mucosal usually imposes many problems to the gastroenterologist, the pathologist and even surgeons (1).  A large scale of benign and malignant diseases should be considered in the differential diagnosis (2). Standard endoscopic biopsy is oftenly superficial for adequate histological diagnosis. The unique ability of endoscopic ultrasound to visualize the layers of the gut wall makes it very useful in assessing such patients (3).

Menetrier's disease is a prototype disease in this category with typical thickening of deep mucosa (4).  Here, we report a EUS finding in a patients with Menetrier's disease. 

 

CASE REPORT

A middle aged man with Menetrier's disease was refeered from Clinical Hospital Zemun for EUS assessment in October 1999. The diagnosis of Menetrier's disease was established on previous hospitalization a couple of months ago.  He had typical symptoms: profound weght loss, anorexia, voliting, diarrhoea as well as symetric edema on legs. Biochemical blood tests disclosed iron-defficiency anemia and marked hypoalbuminemia (<25g/l). On endoscopy, the giant mucosal folds (>10mm) were observed in the stomach body and fundus. Figure 1. Histology disclosed foveolar hyperplasia and glandular atrophy without inflammation in lamina propria mucosae. Figure 4. Thus, the diagnosis of advanced Menetrier's disease was confirmed on the basis of typical clinical, laboratory, endoscopical and histology findings.

On EUS, extended and diffuse thickening of the second layer corresponding to deep mucosa was clearly demonstrated. Figure 2,3. A strict preservation of five layer pattern of gastric wall was observed, too. The depth of 3rd (submucosal) and 4th (muscle) layers was  not changed. The thickeness of 2nd layer was inhomogenous and more hyperechoic.

Figure 2. EUS Menetrier – thickening of II layer corresponding

to deep mucosa.

 

 

Figure 3. EUS Menetrier – thickening of deep mucosa with unchanged

submucosa and muscularis propria

 

Figure 4. Menetrier disease. Hystology show marked fovelar semicystic hyperplasia of deep mucosa (lamina propria mucosae)

 

Figure 5. EUS in linitis plastica: uniform thickening of mucosa, submucosa and muscularis propria: hypoechoic lamina propria mucosae between black arroeheds: submucosa and muscularis propriabetween white arrowheds.

 

         

 

Figure 6. EUS in linitis plastica: Thickening of whole gut wall

 

 

DISCUSSION

A broad spectrum of malignant and benign stomach diseases may clinically and endoscopically present with giant mucosal folds (1-7).  Table 1. Barium X ray studies, CT,  MR are of no value in differential diagnosis of this condition. In the majority of cases endoscopic biopsies are not sensitive enough (3).  Full-thickeness surgical biopsy used to be  the golden standard for diagnosis in past decades.

However, EUS is highly accurate in visualisation of the gut wall (3) Echo-endoscopes with 12MHz transducers allows demonstration of the 5-layer gut wall structue which almost completely correlates with anatomic layers of the normal gut wall (I and II layer belongs to the mucosa; III layer ressembles submucosa, IV muscularis propria and V is adventitia or serosa). EUS can accurately disclose the disruption in the continuity of the normal layer pattern which is invariably observed in malignant diseases (4,5).  EUS can also determine with high sensitivity  which  layers are thickened as well as ECHO pattern of such enlargement (6).  Infiltrative gastric neoplasms, such as lymphoma or linitis plastica type carcinoma produce diffuse thickening of all layers (6).  Figure 5,6.  Thus, EUS can facilitate the differentation of benign from malignant aetiololgies (1-6).

Recent studies confirmed this statement (7-10) Diffuse thickening of all layers or significant enlargement of 4th layer (muscularis propria) was recorded only in malignant conditions (7). Diffuse enlargement of  3 and 4 layer (submucosa and muscularis propria) was present in patients with scihirrous carcinoma (7,8). On the other hand, malignancy did not develop in all patients with gastric wall thickening limited to layer 2 (deep mucosa) during a mean follow-up period of 35 months (8). When the second layer alone is thickened, Menetrier's disease should be at first considered as possible diagnosis and when third layer alone is abnormally enlarged, anisakiasis might be suspected (8). However,  2 and 3  layer enlargement  may be present in healthy subjects with hyperrugosity, but also in patients with lymphoma (8,9). In all malignant conditions, thickening of the second layer was hypoechoic, too (1-9).

EUS finding in our patient was typical: isolated, predominantly hyperechoic enlargement of second layer (deep mucosa) with preservation of 5-layer wall structure. These features strongly suggested benign aetiology of giant gastric folds, accoridng to the literature data (4-9). On the other hand, EUS finding in Menetrier's disease, although typical is not pathognomonic and diagnosis cannot be made with certainity without histology (8-10). Histologic hallmark of diagnosis is marked elongation and turtuosity of the pits (foveolar hyperplasia), accompanied by a reduction in the number of oxyntric glands. Lamina propria is markedly edematous as well (10). Endoscopic biopsy may not sample the full thickness of the mucosa thus revealing only the foveolar hyperplasia. This is suggestive of diagnosis, but does not prove it (10).   

Typical EUS finding in conjuction with foveolar hyperplasia on biopsy diminish the need for surgical biopsy  (10). It can provide reassurance that Menetrier's disease is likely or at least that thickened folds are benign (10). In our case this was confirmed too.

 

REFERENCES:

 

1.Yasuda K. High-resolution endoluminal sonography of the upper gastrointestinal tract: The radial scanning ultrasound probe. Part II. In: Van Dam J, Sivak MV. Gastrointestinal endosonography. Philadelphia: W.B.Saunders; 1999;95-100.

 

2.Yasuda K. The handbook of endoscopic ultrasonography in digestive tract. Ied. Oxford.:  Blackwell Science; 2000.

 

3.Dancygier H, Lightdale C, Stevens P.Endoscopic ultrasonography of the upper gastrointestinal tract and colon. In: Dancygier H, Lightdale J. Endosonography in gastroenterology Ied. Stuttgart-New York: Thieme-Verlag; 1999; 13-174.

 

4.      Michael B. Kimmey, Peter Vilmann: Endoscopic ultrasonography In. Yamada, et al: Textbook of gastroenterology VIed. Philadelphia : Lippnicott Wiliams and Wilkins; 1999; Chapter 136.

5.      Carletti G, Fusaroli P, Bocus P. Endoscopic Ultrasonography. Digestion 1998; 59: 509-530.

6.      Chak A. Endoscopic Ultrasonography. Endoscopy 2000; 32: 146-152

7.    Mendis RE, Gerdes H, Lightdale C, et al. Large gastric folds: a diagnostic approach using endoscopic ultrasonography. Gastrointestinal Endosc 1994;40:437-441.

8.    Songur Y, Okai T, Watanabe H, et al. Endosonographic evaluation of giant gastric folds. Gastrointestinal Endos 1995; 41: 468-474.

9.    Manuory V, Klein O, Houcke ML, et al. Endoscopic ultrasonography in the diagnosis of hypertrophic gastropathy (letter). Gastroenterology 1994; 106:820.

10.  Okuda M, Iiyuka Y, Oh K, et al. Gastritis cystica profunda presenting as giant gastric mucosal folds. The role of endoscopic ultrasonography and mucosectomy in the diagnostic work up. Gastrointestinal Endosc 1994; 40: 640-44.

 

 

BENIGN  DISEASES	MALIGNANT DISEASES
Menetrier's disease	Carcinoma
Zollinger-Ellison syndrome	Lymphoma
Lymphocytic and Eosinophylic gastritis	Lymphoma of MALT
Normal hyperrugosity	Carcinoid
Sarcoidoses and amyloidoses
Crohn's disease
TB and syphilis
H.pyloris; CMV and H.simplex virus

 

Table 1.  Classification of large gastric folds.