In March 1960, an acute and particularly severe abdominal syndrome was recognized in the Bekaa Valley in Lebanon. The first cases were described in male patients from 4 to 25 years of age with an illness that consisted of three phases. Phase I was marked by fainting spells, asthenia, low-grade fever, and headache. Patients rarely sought medical treatment at this stage because the symptoms were not serious enough. In the few patients seen at this stage, physical examination disclosed flushing of the face and red conjunctivae, but appearance was not otherwise affected. The first impression of a general practitioner who saw patients during this phase of illness was that the patient had an early viral infection. Phase II started when, 24 hours later, early symptoms persisted, and abdominal pain of variable intensity supervened. The pain description ranged from mild to severe paroxysms to constant pain. Low-grade fever, nausea, and vomiting were frequent; diarrhea, if present, was mild. Physical examination usually showed a smooth, ill-defined mass in the right lower quadrant or the periumbilical area and abdominal distention. At that stage, patients were referred to a specialist for acute abdominal infection. Three clinical findings, however, did not fit this diagnosis: 1) the illness started with vague, generalized symptoms instead of abdominal pain; 2) evidence of ascites was found on examination, 3) and patients had severe weakness and intravascular depletion, findings uncommon in early appendicitis.

Phase III was recognized because most patients were referred at that stage with rapid increase in abdominal girth and paroxysms of abdominal pain. Occasionally, gastrointestinal bleeding concurred. Upon examination, some features were frequently present: shock, ascites, flushed face, and red conjunctivae. Because of unclear and questionable diagnosis, exploratory laparotomy was performed on several patients, invariably showing an abundant yellowish and thick ascitic fluid, soft hypertrophied mesenteric lymph nodes (3 cm–5 cm) mostly in the ileocecal region, and substantial edema involving one segment of small bowel, cecum, or ascending colon. When the diseased segment was not resected during surgery, many patients experienced a rapid but transient postoperative clinical improvement. Ascites, however, soon reaccumulated, and most of these patients died from an overwhelming state of shock. Definite and steady recovery was observed in most patients who underwent partial bowel resection. Examination of the bowel segment at surgery or autopsy disclosed a central necrotic lesion (2 cm–3 cm) encircled by small soft red nodules (0.5 cm–1 cm in diameter), and surrounded by severe edema of the bowel wall with areas of hemorrhage. Bowel perforation was uncommon, as patients usually died before that progression (1,2).

Most of the patient population had these signs and symptoms. In some cases, however, two unusual clinical pictures were observed: the surgical form and abortive form. In the surgical form, the illness started with severe abdominal pain without the generalized symptoms of phase I. Surgery was often performed with the provisional diagnosis of acute infection in the abdomen. In the abortive form, the illness was limited to phase I, probably because such patients had taken penicillin or other antibiotics, thus interrupting the cycle of the disease (2,3).

More cases were being described, but no clear diagnosis could be made until October 1960, when the cause of this curious clinical syndrome was elucidated. A 30-year-old woman with abdominal symptoms and ascites underwent laparotomy but died 12 hours later. Surgery showed ascites, an edematous small bowel loop, and huge mesenteric lymph nodes. Intestinal resection and lymph node biopsies were performed. Pathologic examination showed a central mucosal necrotic lesion along with hemorrhage and extensive edema. Gram-positive bacilli in chains were also observed. Bacterial cultures from blood and excised lymph nodes grew Bacillus anthracis. Inoculation of test animals resulted in death within 20 hours; their spleens had large amounts of anthrax bacilli (4). The identity of the bacillus was further confirmed when tissue specimens were sent to the Centers for Disease Control and Prevention (CDC) for analysis.

With this new information at hand, investigations were conducted to pinpoint the source of infection. The patient’s husband, a shepherd, was questioned and admitted that 4 days before the onset of his wife’s illness, he had slaughtered a dying goat. The meat was consumed raw by friends and family members (customary in some remote villages). All persons who ate meat were identified. The patient’s sister-in-law had had cutaneous anthrax above the lip and had an uneventful course with penicillin therapy. Another person had complained of vague abdominal pain and mild diarrhea; stool culture was negative. The goat’s skin was examined and found to have anthrax spores. Medical records of previous patients were then reviewed; their families were contacted, and a number of persons did recall eating raw meat few days before symptoms began. Furthermore, paraffin-embedded tissue blocs from a patient who died earlier of a similar illness were reexamined; evidence of gram-positive bacilli was found in the intestinal tissue (4).

Over 100 cases of intestinal anthrax were subsequently observed during a period of 14 years. Most patients were shepherds or their relatives who lived in remote villages of the Bekaa Valley. Their livestock was not vaccinated against anthrax, and they regularly lost some of their herds to a sickness they called “the disease of the spleen” because that organ was always massively swollen. Sick animals were slaughtered; the skin was sold, and meat was often ingested raw or inadequately cooked. The liver was usually eaten by children and young adults.

Gastrointestinal anthrax is extremely rare in the United States and western Europe but is more frequently encountered in developing countries around the globe. Research describing the clinical manifestations of this entity is scarce, poorly detailed, and inaccurate about appropriate management (5).

We performed a MEDLINE search to evaluate data published on gastrointestinal anthrax. Research that could be accessed and reviewed consisted of 11 reports from 1970 to 2000, most of which described single cases. Most patients were from developing countries, namely Iran, Bangladesh, Zimbabwe, Thailand, Uganda, India, and Turkey (6–15) (Table). Only two cases were reported from the United States, by CDC in 2000 (16). The infection was uniformly associated with eating contaminated meat, although theoretically, any ingested item could act as a vehicle for the transmission of anthrax spores. One study by Ndyabahinduka et al. was of an epidemic of gastrointestinal anthrax in Uganda in 1984, which affected 143 of 155 persons who ate meat from an infected zebu (Asian ox). In most cases, symptoms were those of gastroenteritis, with abdominal tenderness, vomiting, and diarrhea. Three children had blood-tinged stools from which anthrax bacilli were isolated. Thirteen patients had pharyngeal edema of variable severity. A fatal outcome from fulminant gastroenteritis was reported in nine patients, all children. All other patients responded quickly to tetracycline or penicillin (10). Another study by Phonboon et al. described an outbreak of gastrointestinal anthrax after an outbreak in cattle in Thailand; 74 persons become ill, and 3 died (9).

In published cases of gastrointestinal anthrax, death was more common in patients who had severe symptoms, including hematemesis, vomiting, abdominal pain, and distention (phase III), and who were only treated with antibiotics (6,11,13,14). Surgical exploration and bowel resection was performed in two patients first seen in phase III (8,15). The disease involved the ascending colon alone in one case and the cecum and ascending colon in the other. After surgery and antibiotic therapy, both patients recovered and were discharged. These two cases illustrate the benefit of surgery in the advanced form of gastrointestinal anthrax. These findings support our current approach for managing patients whose condition remains unstable after 6 to 12 hours of treatment, namely, administering antibiotics and adequate resuscitation and then resecting the diseased bowel segment. The rationale behind surgical resection is to overcome not only the large bacterial load but also the larger load of toxin in diseased tissues. In all our cases, the disease was confined to a single bowel segment, mostly small bowel or cecum. The clinical condition of patients improved rapidly and steadily after resection. Initial results were disappointing, since several patients died postoperatively of anastomotic leaks, dehiscence, and fistulization. Incomplete resection, severe hypoproteinemia, and rapid reaccumulation of protein-rich ascitic fluid were the main causes of surgical failure.

Based on our experience, the approach used in the management of cases of gastrointestinal anthrax should consist of: 1) initiation of intensive intravenous antibiotic therapy as soon as the diagnosis is made, 2) wide resection into seemingly healthy tissues with primary anastomosis in patients who did not improve with medical therapy, 3) continuous drainage of the ascites, as fluid will continue to accumulate for several days after surgery, 4) and aggressive replacement of protein and electrolyte losses (2,17). However, to make any generalization about the preferred mode of treatment of gastrointestinal anthrax in the absence of solid and reproducible clinical and epidemiologic data would be difficult. Furthermore, with the current improved access to medical care and advances in diagnostic techniques and supportive measures compared to the 1960s, surgical intervention might now be limited to few cases of advanced disease unresponsive to medical therapy.

Following the 1960s outbreak, some areas were recognized as being contaminated by anthrax spores. Grazing of livestock in these “damned fields” (as they were called by ancient French farmers) has since been avoided by shepherds, thereby virtually eliminating the disease from the Bekaa Valley (2,3).

We describe the clinical spectrum of gastrointestinal anthrax, a disease that was endemic in Lebanon in the 1960s. We also report on the success of surgical treatment in some of the advanced cases, emphasizing the vital role of aggressive supportive measures in patient management. Our report lacks detailed epidemiologic data describing the incidence, age and sex distribution, and outcome of patients because most cases date back to the 1960s, a time when epidemiologic studies were scarce in a developing country such as Lebanon. Our experience with gastrointestinal anthrax, however, remains valuable because of the rarity of this condition and the dearth of data on management approaches.

Naturally, the consumption of raw meat, still widely practiced in many countries, should be strongly discouraged through education of the mass population as to the health hazards associated with such a custom. Ultimately, however, the control of anthrax in humans is chiefly dependent on control of the disease in animals. In the event of an anthrax enzootic, extensive investigations should be conducted to identify the source of infection and eliminate it. Milk from areas experiencing enzootics should be discarded, and sick animals should be isolated. Affected farms should be quarantined for at least 2 weeks after the last death from anthrax. The release of B. anthracis in the environment should be avoided to prevent future disease; dead animals should be burned or buried deeply and covered with lime. Their carcasses should not be slaughtered or necropsied, as exposure of the vegetative forms to the ambient atmosphere enhances sporulation. Annual vaccination of livestock and avoidance of clandestine slaughtering remain vital to the prevention of disease in endemic areas (18–20).