Clinical Features of Abdominal Painful Crisis in Sickle Cell Anemia By William A. Bonadio Milwaukee, 0 A retrospective review of children having sickle cell anemia and abdominal painful crisis IAPCI was performed in order to delineate the pattern of their presenting clinical characteristics. Df 43 children accounting for 108 cases of APC that occurred during a &year period. 94 cases involved a child who had had a previous episode of APC. Symptoms included vomiting (10) and concomitant bone or joint pain (42). Physical findings showed 98 children with normoactive bowel sounds, 96 with a nonrigid abdomen to palpation. two with involuntary guarding, and two with rebound tenderness. Certain clinical characteristics are useful in distinguishing children with sickle cell anemia and APC. As compared with traditional findings that suggest a “surgical” abdomen, most of the children who have sickle cell APC do not experience vomiting, and present with normoactive bowel sounds. nonrigid abdomen. and absence of involuntary guarding or rebound tenderness. Children having presumed APC who receive analgesic medication in an outpatient setting with symptomatic improvement should be hospitalized for observation to monitor for recurrence/progression of symptoms indicative of an abdominal disease process requiring surgery. 0 1990 by W. 8. Seunders Company. INDEX WORDS: Abdominal painful crisis (APC); sickle cell anemia: abdominal pain.
P
AINFUL CRISIS is a common complication of children who have sickle cell anemia. When a child with sickle cell anemia experiences abdominal pain, the physician must distinguish whether the underlying cause is due to ischemia from vasoocclusive sickling of erythrocytes, or an abdominal disease process requiring prompt surgical intervention. With the latter, the administration of analgesic medication could mask the progression of signs and symptoms that aid the physician in guiding management decisions and instituting appropriate therapy. There is a paucity of literature that describes the characteristics of children with sickle cell anemia and abdominal painful crisis (APC). The purpose of this study is to delineate the presentation and course of children with sickle cell anemia and APC.
Wisconsin RESULTS
During the study period, 43 patients with sickle cell disease accounted for 106 episodes of painful crisis with abdominal pain. Of these, 25 were boys and 18 were girls; patient age range was 2.5 to 18 years (mean, 5.7 years). The clinical features of these patients are delineated in Table 1. Thirty-eight patients had homozygous hemoglobin SS and five had heterozygous hemoglobin SC. In 38 cases, a urine culture was performed; two had Escherichiu coli isolated (> 100,000 colony forming units [CFU]/mL). After initial evaluation and administration of therapy in the emergency department, 35 cases resulted in discharge and 71 cases resulted in hospitalization. Of the patients who were hospitalized, the mean duration of stay was 3.4 days; all received parenterally administered fluids and narcotic analgesic medication, and 14 received transfusion of red blood cells. In each case there was complete resolution of abdominal pain with medical management measures. Of the patients who received therapy and were discharged from the emergency department, 28 received narcotic analgesic medication with symptomatic improvement of pain. In no instance did a study patient require surgical intervention for an abdominal disease process. DISCUSSION
Acute abdominal pain is a common component of sickle cell crisis. Abdominal crisis is thought to result from small infarcts of the mesentery and abdominal viscera, and can be characterized by severe abdominal pain with signs of peritoneal irritation.lp2 Specific causes for abdominal pain with sickle cell crisis include hepatic crisis, cholelithiasis, splenic sequestration, and pancreatitis. Painful crisis secondary to these disorders usually resolves with supportive measures of therapy, consisting of maintaining oxygenation, hydration, and analgesia. When a child with sickle cell anemia presents with
MATERIALS AND METHODS A retrospective review of the admission records to the Emergency Department of Children’s Hospital of Wisconsin, Milwaukee, from January 1984 to January 1988 was performed. Children with sickle cell anemia and a discharge diagnosis of painful crisis were identified. Their medical records were reviewed. Patients experiencing abdominal pain at the time of presentation were studied. All radiographic interpretations were performed by a pediatric radiolo gist.
Journal of Pediatric Surgery. Vol 25, No 3 (March), 1990: pp 301-302
From the Department of Pediatrics, Medical College of Wisconsin, and the Childrenf Hospital of Wisconsin, Milwaukee, WI. Date accepted: December 3.1988. Address reprint requests to William A. Bonadio. MD, Department of Pediatrics. Children’s Hospital of Wisconsin, PO Box I997, MS No. 756. Milwaukee. WI 53201. 0 1990 by W.B. Saunders Company. 0022-346a/90/2503&36$03&/0
301
302
WILLIAM A. BONADIO Table 1. Clinical Features of Children With Abdominal Painful Crisis History Previous episode of APC
94
Symptoms Nausea
5
Vomiting
10
Diarrhea
5
Accompanying pain Extremity Chest Ba&
42 3 15
Abdominal pain location Diffuse
04
Focal
22
Physical findings Temperature >38OC
50
Abdomen Tenderness
60
Distension
23
Rigidity
10
Diminished/absent bowel sounds
8
Normal rectal examiantion Rebound tenderness
6 (6) 2
Involuntary guarding
2
Data base Normal abdominal radiograph
18 (19)
NOTE. Number in parenthesis denotes total who received respective evaluation.
abdominal pain, it can be difficult for the physician to accurately distinguish between crisis and an acute intraabdominal disease process requiring surgery.374 This distinction is important because administration of analgesic medication to the patient with an acute surgical disease process may mask the character and progression of signs and symptoms that aid the physician in instituting prompt, appropriate therapy. On the other hand, inappropriate laparotomy during sickle cell crisis may lead to aggravation of crisis due to intraoperative hypoxia.5 The clinical features of abdominal pain due to crisis may be indistinguishable from those of any acute intraabdominal disease process-for example, chorecystitis, appendicitis, salpingitis, bowel obstruction or infarction, and perforated viscus. With many of these disorders, the classic features of the “surgical abdomen” would be expected-vomiting, a tender and distended abdomen with rigidity, diminished or absent bowel
sounds, involuntary guarding, and rebound tenderness. Yet, the clinical picture that characterizes the child with sickle cell anemia and APC can be uniquely different. In accord with other reports,4’6’7this study found that the majority of children with APC had experienced a prior episode of APC, and that physical examinations consistently showed normoactive bowel sounds. In addition, this study showed that most patients did not experience vomiting or exhibit signs of abdominal rigidity, involuntary guarding, or rebound tenderness on examination. As opposed to previous reports, over one half of the children in our series presented with pain localized solely to the abdomen, in the absence of bone or joint pain; an almost equal number were febrile. Of note was the large number of children with abdominal pain who were discharged from the outpatient .setting after experiencing symptomatic improvement through administration of analgesic medication. Narcotic analgesics can mask the character and progression of signs and symptoms essential to giving definition to the causes of an abdominal disease process; they can also induce sedation, which can blunt a patient’s accurate and timely reporting of symptoms. For these reasons, we recommend that children with sickle cell anemia and abdominal pain who receive narcotic analgesic medication with symptomatic improvement be hospitalized for observation to monitor for recurrence or progression of symptoms. In conclusion, children with sickle cell anemia and APC characteristically have experienced a prior episode of APC, do not experience vomiting, and present with concomitant bone or joint pain, normoactive bowel sounds, a nonrigid abdomen, and absence of involuntary guarding or rebound tenderness. It must be emphasized that close clinical monitoring of patients with APC is essential; if signs of peritoneal irritation are present, hospitalization for parenteral fluid therapy and observation is necessary. For children with presumed APC who receive narcotic analgesic medication in an outpatient setting with symptomatic improvement, hospitalization is recommended for observation during monitoring for recurrence or progression of symptoms indicative of an abdominal disease process requiring surgery.
REFERENCES 1. Lukens JN: Sickle cell disease. Dis Mon 27:1-X, 1981 2. Diggs LW: Sickle cell crisis. Am J Clin Pathol44:1-19, 1965 3. Ariyan S, Shessel FS, Pickett LK: Cholecystitis and cholelithiasis masking as abdominal crisis in sickle cell disease. Pediatrics 58:252-258,1976 4. Kudsk KA, Tranbaugh AF, Sheldon GF: Acute surgical illness in patients with sickle cell anemia. Am J Surg 142:113-l 17,198l
5. Tempest MN: Sickle cell disease and its surgical implications in the African child. Br J Oral Surg 7:96-103, 1969 6. Crastnopol P, Stewart CF: Acute abdominal manifestations sickle cell disease. Arch Surg 59:993-1000, 1949 7. Campbell EH: Acute abdominal Arch Surg 31:607-621,1935
in
pain in sickle cell anemia.
P
AINFUL CRISIS is a common complication of children who have sickle cell anemia. When a child with sickle cell anemia experiences abdominal pain, the physician must distinguish whether the underlying cause is due to ischemia from vasoocclusive sickling of erythrocytes, or an abdominal disease process requiring prompt surgical intervention. With the latter, the administration of analgesic medication could mask the progression of signs and symptoms that aid the physician in guiding management decisions and instituting appropriate therapy. There is a paucity of literature that describes the characteristics of children with sickle cell anemia and abdominal painful crisis (APC). The purpose of this study is to delineate the presentation and course of children with sickle cell anemia and APC.
Wisconsin RESULTS
During the study period, 43 patients with sickle cell disease accounted for 106 episodes of painful crisis with abdominal pain. Of these, 25 were boys and 18 were girls; patient age range was 2.5 to 18 years (mean, 5.7 years). The clinical features of these patients are delineated in Table 1. Thirty-eight patients had homozygous hemoglobin SS and five had heterozygous hemoglobin SC. In 38 cases, a urine culture was performed; two had Escherichiu coli isolated (> 100,000 colony forming units [CFU]/mL). After initial evaluation and administration of therapy in the emergency department, 35 cases resulted in discharge and 71 cases resulted in hospitalization. Of the patients who were hospitalized, the mean duration of stay was 3.4 days; all received parenterally administered fluids and narcotic analgesic medication, and 14 received transfusion of red blood cells. In each case there was complete resolution of abdominal pain with medical management measures. Of the patients who received therapy and were discharged from the emergency department, 28 received narcotic analgesic medication with symptomatic improvement of pain. In no instance did a study patient require surgical intervention for an abdominal disease process. DISCUSSION
Acute abdominal pain is a common component of sickle cell crisis. Abdominal crisis is thought to result from small infarcts of the mesentery and abdominal viscera, and can be characterized by severe abdominal pain with signs of peritoneal irritation.lp2 Specific causes for abdominal pain with sickle cell crisis include hepatic crisis, cholelithiasis, splenic sequestration, and pancreatitis. Painful crisis secondary to these disorders usually resolves with supportive measures of therapy, consisting of maintaining oxygenation, hydration, and analgesia. When a child with sickle cell anemia presents with
MATERIALS AND METHODS A retrospective review of the admission records to the Emergency Department of Children’s Hospital of Wisconsin, Milwaukee, from January 1984 to January 1988 was performed. Children with sickle cell anemia and a discharge diagnosis of painful crisis were identified. Their medical records were reviewed. Patients experiencing abdominal pain at the time of presentation were studied. All radiographic interpretations were performed by a pediatric radiolo gist.
Journal of Pediatric Surgery. Vol 25, No 3 (March), 1990: pp 301-302
From the Department of Pediatrics, Medical College of Wisconsin, and the Childrenf Hospital of Wisconsin, Milwaukee, WI. Date accepted: December 3.1988. Address reprint requests to William A. Bonadio. MD, Department of Pediatrics. Children’s Hospital of Wisconsin, PO Box I997, MS No. 756. Milwaukee. WI 53201. 0 1990 by W.B. Saunders Company. 0022-346a/90/2503&36$03&/0
301
302
WILLIAM A. BONADIO Table 1. Clinical Features of Children With Abdominal Painful Crisis History Previous episode of APC
94
Symptoms Nausea
5
Vomiting
10
Diarrhea
5
Accompanying pain Extremity Chest Ba&
42 3 15
Abdominal pain location Diffuse
04
Focal
22
Physical findings Temperature >38OC
50
Abdomen Tenderness
60
Distension
23
Rigidity
10
Diminished/absent bowel sounds
8
Normal rectal examiantion Rebound tenderness
6 (6) 2
Involuntary guarding
2
Data base Normal abdominal radiograph
18 (19)
NOTE. Number in parenthesis denotes total who received respective evaluation.
abdominal pain, it can be difficult for the physician to accurately distinguish between crisis and an acute intraabdominal disease process requiring surgery.374 This distinction is important because administration of analgesic medication to the patient with an acute surgical disease process may mask the character and progression of signs and symptoms that aid the physician in instituting prompt, appropriate therapy. On the other hand, inappropriate laparotomy during sickle cell crisis may lead to aggravation of crisis due to intraoperative hypoxia.5 The clinical features of abdominal pain due to crisis may be indistinguishable from those of any acute intraabdominal disease process-for example, chorecystitis, appendicitis, salpingitis, bowel obstruction or infarction, and perforated viscus. With many of these disorders, the classic features of the “surgical abdomen” would be expected-vomiting, a tender and distended abdomen with rigidity, diminished or absent bowel
sounds, involuntary guarding, and rebound tenderness. Yet, the clinical picture that characterizes the child with sickle cell anemia and APC can be uniquely different. In accord with other reports,4’6’7this study found that the majority of children with APC had experienced a prior episode of APC, and that physical examinations consistently showed normoactive bowel sounds. In addition, this study showed that most patients did not experience vomiting or exhibit signs of abdominal rigidity, involuntary guarding, or rebound tenderness on examination. As opposed to previous reports, over one half of the children in our series presented with pain localized solely to the abdomen, in the absence of bone or joint pain; an almost equal number were febrile. Of note was the large number of children with abdominal pain who were discharged from the outpatient .setting after experiencing symptomatic improvement through administration of analgesic medication. Narcotic analgesics can mask the character and progression of signs and symptoms essential to giving definition to the causes of an abdominal disease process; they can also induce sedation, which can blunt a patient’s accurate and timely reporting of symptoms. For these reasons, we recommend that children with sickle cell anemia and abdominal pain who receive narcotic analgesic medication with symptomatic improvement be hospitalized for observation to monitor for recurrence or progression of symptoms. In conclusion, children with sickle cell anemia and APC characteristically have experienced a prior episode of APC, do not experience vomiting, and present with concomitant bone or joint pain, normoactive bowel sounds, a nonrigid abdomen, and absence of involuntary guarding or rebound tenderness. It must be emphasized that close clinical monitoring of patients with APC is essential; if signs of peritoneal irritation are present, hospitalization for parenteral fluid therapy and observation is necessary. For children with presumed APC who receive narcotic analgesic medication in an outpatient setting with symptomatic improvement, hospitalization is recommended for observation during monitoring for recurrence or progression of symptoms indicative of an abdominal disease process requiring surgery.
REFERENCES 1. Lukens JN: Sickle cell disease. Dis Mon 27:1-X, 1981 2. Diggs LW: Sickle cell crisis. Am J Clin Pathol44:1-19, 1965 3. Ariyan S, Shessel FS, Pickett LK: Cholecystitis and cholelithiasis masking as abdominal crisis in sickle cell disease. Pediatrics 58:252-258,1976 4. Kudsk KA, Tranbaugh AF, Sheldon GF: Acute surgical illness in patients with sickle cell anemia. Am J Surg 142:113-l 17,198l
5. Tempest MN: Sickle cell disease and its surgical implications in the African child. Br J Oral Surg 7:96-103, 1969 6. Crastnopol P, Stewart CF: Acute abdominal manifestations sickle cell disease. Arch Surg 59:993-1000, 1949 7. Campbell EH: Acute abdominal Arch Surg 31:607-621,1935
in
pain in sickle cell anemia.
