SICKLE CELL CRISIS AND THE

ACUTE ABDOMEN
Ahmad Nazem, MD, and Mark Walker, MD
Washington, DC

Abdominal pain is one of the most common A review of symptoms was positive for pneu-
symptoms in sickle cell crisis, and its cause monia, urinary tract infection, and for multiple
remains controversial. Simple vaso-occlusive sickle cell crises in the past.
crisis may be an explanation. The abdominal On physical examination, his pulse was 138
pain may also reflect an acute surgical abdo- beats per minute, respiration 25 per minute, tem-
men. perature 103°F, and blood pressure 110/80 mmHg.
A patient presented with sickle cell crisis He was a well-developed, asthenic black man in
and abdominal pain; he had a periappen- moderate distress. Examinations of head, ears,
diceal abscess at the site of an appendiceal eyes, nose, and throat were within normal limits.
stump five months after appendectomy. The Respiration was normal. Breath sounds were clear
role of sickle cell anemia in the pathogenesis bilaterally. The cardiovascular system revealed a
of this abscess is uncertain. II/VI systolic ejection murmur heard at LSB with
normal S1 and S2. Abdominal examination re-
vealed healed incisions in right lower quadrant and
right upper quadrant. There were diffuse abdominal
tenderness and rebound tenderness in the right
A 24-year-old black man with sickle cell disease lower quadrant. Bowel sounds were hypoactive.
was in his usual state of health until one day prior No organomegaly was present. Rectal examina-
to admission, when he developed sharp stabbing tion revealed good sphincter tone. The guaiac test
pain while dancing. He also had dull, deep, aching for occult blood was negative. He had bilateral
pain in both lower legs and in the mid-abdominal lower extremity joint tenderness. The neurological
region. He experienced nausea and vomiting con- examination was normal.
sisting of a greenish liquid. The pain became Laboratory findings were as follows: hemoglo-
gradually worse; he came to Howard University bin 8.2 g/dL, hematocrit 22.2 percent, white cell
Hospital for evaluation. count 33.5/mm3, sodium 131 mEq/L, potassium
His past medical history was significant for 5.2 mEq/L, chloride 103 mEq/L, carbon dioxide
cholecystectomy three years prior to this admis- 18.7 mEq/L, glucose 118 mg/dL, blood urea nitro-
sion and appendectomy in another hospital five gen (BUN) 83 mg/dL, total bilirubin 5.1 mg/dL,
months before. conjugated bilirubin 0.7 mg/dL, creatinine 1.2
mg/dL, prothrombin time 10.4/9.6 s, partial
thromboplastin time 35/34 s, albumin 3.9 g/dL,
reticulocyte count 23.1 percent, sedimentation
rate 150, urinalysis, specific gravity 1,015, and the
Requests for reprints should be addressed to Dr. Ahmad chest x-ray film was unremarkable. The electro-
Nazem, Department of Surgery, Howard University Hospi- cardiogram was within normal limits.
tal, 2041 Georgia Avenue, NW, Washington, DC 20060. The patient was admitted to the medical service
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 78, NO. 8, 1986 787
SICKLE CELL CRISIS

with a diagnosis of veno-occlusive crisis and tenderness and increased white blood cell count
possible sepsis; he was treated with hydration, in- suggested an acute surgical abdomen. Because of
travenous antibiotics, and meperidine hydrochlo- fluctuance in the cul-de-sac, a pelvic abscess was
ride (Demerol). Blood cultures showed no growth. suspected.
On the fourth hospital day, a surgical consultation In reviewing the literature, no increased inci-
was obtained. At that time he had severe, sharp, dence of intraabdominal abscesses in the patients
right lower quadrant abdominal pain, and the presenting with sickle cell crisis was found. The
physical examination revealed a temperature of cause of the abdominal infection in this case is
1030F, pulse 110 beats per minute, respiration 24, obscure. The role of previous appendectomy with
and blood pressure 110/80 mmHg. The abdomen necrosis distal to the ligated appendiceal stump is
was moderately distended and bowel sounds were unclear. The stump was not clearly identified at
hypoactive. There was bilateral lower quadrant the time of exploratory surgery.
tenderness with rebound. On rectal examination In patients with sickle cell crisis and abdominal
there was fluctuance and tenderness in the cul- pain with a history of previous abdominal surgery,
de-sac. one should have a high index of suspicion of
Laboratory findings at this time revealed: hemo- intraabdominal pathology at the time of presenta-
globin 6.1 g/dL, hematocrit 18.8 percent, and WBC tion (abscess, ruptured viscus, or gangrenous
47,000/mm3. The abdominal x-ray films showed bowel).
distended small bowel and multiple air-fluid levels. It is worth mentioning that the incidence of in-
The patient was transfused, and with the diagnosis fection is higher in sickle cell disease patients.5'6
of intraabdominal abscess, he was taken to the This incidence has been ascribed to impaired
operating room. An exploratory laparotomy re- splenic function in these patients. In sickle cell
vealed two intraabdominal abscesses, one in the disease the spleen has lost its filtering and opsoniz-
right lower quadrant and the other in the left lower ing actions.7-9 It is speculated that this loss of
quadrant with pus in the cul-de-sac. These collec- splenic function may increase the incidence of
tions were walled off with bowel and omentum. intraabdominal infection.
Three hundred milliliters of pus was drained. It In terms of preoperative preparation in sickle
was not clear whether the stump of the appendix cell crisis patients, transfusions before the opera-
was in the area of the right lower quadrant abscess tion have been advocated.10 In the case reported
cavity. Penrose and Jackson-Pratt drains were here, the patient was transfused with packed red
used. He was continued on triple antibiotic blood cells.
therapy (ampicillin, gentamicin and clindamycin).
The operative cultures grew enterococcus and Es- Literature Cited
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including enlarged mesenteric and retroperitoneal 4. Gage TP, Gagiez JM. Ischemic colitis complicat-
lymph nodes,1 2 marrow hyperplasia, or infarction ing sickle cell crisis. Gastroenterology 1983; 84:171-174.
5. Krivitin W. Overwhelming postsplenectomy infec-
of vertebral bodies resulting in pressure on nerve tion. Am J Dis Child 1977; 131:784-787.
roots, hepatobiliary disease (examples being he- 6. Landesman, SH. Infections in children with sickle
patic microinfarction and cholelithiasis) and cell anemia. Special reference to pneumococcal and sal-
monella infections. Am J Pediatr Hematol Oncol 1982;
splenitis,3 splenic hemorrhage, or splenic throm- 4(4) :407-418.
bosis, or to mesenteric arterial thrombosis. Gage 7. Charache S. Treatment of sickle cell anemia. Annu
and Gagiez4 recently reported a case of sickle cell Rev Med 1981; 32:195.
8. Barett-Connoz E. Bacteriodes infection in sickle cell
crisis with ischemic colitis due to sickling in which anemia. Medicine 1971; 50:97-112.
the patient presented with right lower quadrant 9. Hughes J, Carrot DS. Salmonella osteomyellitis
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