Dear Sir: In a recent issue (11, Faber et al. from Jerusalem report two Jewish Iranian families with five members suffering from chronic neuropathic intestinal pseudoobstruction. In 1985 we described an Arab family with two daughters affected by a similar disease (2). The most striking features in our cases were cachexia, motility disorders of the gastrointestinal tract, malabsorption, sensory deafness, and peripheral nerve demyelization. We suggested the disorder be called “GrollHirschowitz syndrome,” honoring the authors who first described the condition (3). It seems to be an extremely uncommon autosomal recessive syndrome. However, the fact that 7 patients belonging to three different families have hitherto been reported from a small country might suggest that this condition is less rare than initially believed. N. LEVY E. STERMER
Gastroenterology Service Haifa City Medical Center [Rothschild) Faculty of Medicine, Technion P. 0. Box 4940 Haifa, Israel Faber J, Fich A, Steinberg A, et al. Familial intestinal pseudoobstruction dominated by a progressive neurologic disease at a young age. Gastroenterology 1987;92:786-90. Potasman I, Stermer E, Levy N, et al. The Groll Hirschowitz syndrome. Clin Genet 1985;28:76-9. Groll A, Hirschowitz BJ. Steatorrhea and familial deafness in two siblings (abstr). Clin Res 1966;14:47.
More About Internist-Led Surgeons Dear Sir: In our previous letter (Gastroenterology 1987;92:841-21, which was attributed solely to me, the name of my coauthor, Dr. Norman D. Grace, Tufts University, School of Medicine, Boston, Massachusetts, was inadvertently omitted. When chastising surgeons, one’s safety is in numbers. In this instance it is especially important that internist-gastroenterologists and internist-hepatologists speak with one voice. HAROLD 0. CONN, M.D.
Professor of Medicine Liver Disease Unit Veterans Administration Medical Center West Spring Street West Haven, Connecticut 06516
GASTROENTEROLOGY Vol. 93, No. 4
Screening for Colorectal Cancer in a High Risk Population Dear Sir: I have read with interest the recent article by Eddy et al. (1) recommending barium enema for screening a high risk population with first degree relatives having colon cancer. The mode1 used overlooks several important practical issues. Barium enema has no therapeutic potential for colonic neoplasia. Thus, virtually all patients with polyps discovered on barium enema require colonoscopic polypectomy. Barium enema cannot provide a tissue diagnosis. Thus, patients felt to have nonobstructing carcinoma on barium enema usually undergo preoperative colonoscopy to confirm the diagnosis, exclude synchronous carcinoma, and remove synchronous polyps. Patients in either of these categories would require a second inconvenient, uncomfortable preparation if screened first with a barium enema. The authors estimate that 10%15% of the screened cohort eventually develop carcinoma. Certainly, a substantial additional fraction of the cohort would develop polyps and require both procedures at least once. Both tests would be required also after a false-positive barium enema. Because an abnormal barium enema usually leads to colonoscopy, the screening recommendations of Eddy et al. generate a sizable subset of patients requiring two expensive and uncomfortable procedures after two expensive and uncomfortable preparations. Unfortunately, screening such a cohort with barium enema tends to penalize the unfortunate patient with colonic neoplasia. Because colonoscopic screening is more efficient and has therapeutic potential that barium enema lacks, reduction in the cost of colonoscopy may be the most reasonable approach. CHARLES F. GHOLSON, M.D.
St. Francis Medical Center Cape Girardeau, Missouri
1. Eddy DM, Nugent FW, Eddy JF, et al. Screening for colorectal cancer in a high risk population. Gastroenterology 1987;92: 682-92. Reply. We concur with Dr. Gholson’s observation that, unlike sigmoidoscopic and colonoscopic examinations, the barium enema cannot provide a definitive diagnosis. Patients who have a “positive” barium enema will require further evaluation often involving endoscopy. As we point out in our paper (p. 688), this is one of the important factors that should be considered in the choice of a screening policy. We also concur that a reduction in the cost of colonoscopy is highly desirable. DAVID M. EDDY, M.D., Ph.D. Center for Health Policy Research and Education P. 0. Box GM Duke Station Durham. North Carolina 27706