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Childhood sigmoid volvulus
CASE REPORT colon, obstruction, sigmoid volvulus; sigmoid volvulus, pediatric; volvulus, sigmoid, pediatric
Childhood Sigmoid Volvulus A 14-year-old boy presented to the emergency department with abdominal pain. Flat and upright abdominal films and a barium enema revealed the classic finddngs of sigmoid volv~lus. Sigmoidoscopy allowed a soft rubber tube to be passed beyond the obstruction with immediate relief of pain. The patient subsequently underwent a descending sigmoid colectomy. Sigmoid volvulus is a rare entity in childhood. Case discussion and review of the literature is presented. [Seger DL, Middleton D: Childhood sigmoid volvulus. Ann Emerg Med February 1984;13:133-135.]
INTRODUCTION Sigmoid volvulus is seldom considered in the differential diagnosis of abdominal pain in children. It is classically a disease of the elderly and is second only to carcinoma as a cause of acute large bowel obstruction. 1 Seventy percent of reported cases occur in patients over 50 years of age, with peak incidence at age 71.1-5 Sigmoid volvulus rarely occurs in children, as evidenced by 53 reported cases in the world literature and 22 reported cases in the English language. 6 The severity and potential consequences of unrecognized sigmoid volvulus can be devastating. The possibility of volvulus should be considered in any child presenting with a bowel obstruction. Our case report illustrates the classic features of sigmoid volvulus.
Donna L Seger, MD Don Middleton, MD Jacksonville, Florida From the Department of Emergency Medicine, University Hospital of Jacksonville, Jacksonville, Florida. Received for publication July 18, 1983. Revision received September 15, 1983. Accepted for publication November 8, 1983. Address for reprints: Donna L Seger, MD, Department of Emergency Medicine, University Hospital of Jacksonville, 655 West Eighth Street, Jacksonville, Florida 32209.
CASE REPORT A 14-year-old boy was admitted to University Hospital of Jacksonville with a four-day history of abdominal pain. He had been in good health until three days prior to admission. At that time he was awakened by epigastric and periumbilical pain. The severity of the pain waxed and waned for a few hours and then began to increase in intensity over the next 24 hours. During that period, the patient had two episodes of vomiting following meals. Two days prior to admission, the pain subsided but did not completely resolve. One episode of vomiting occurred. On the afternoon prior to admission the pain recurred, and there was another episode of vomiting. The pain did not abate, and the patient was brought to the emergency department. According to the child, his last bowel movement had been five days prior to admission. He denied hematemesis, melena, and hematochezia. The patient denied chronic constipation and stated that he had regular daily bowel habits until the onset of his illness. On physical examination, the patient appeared to be in moderate distress. Vital signs were as follows: temperature, 37.7 C; pulse, 132 beats per minute; respirations, 28/rain; blood pressure 110/70 m m Hg; and weight, 35 kg. Physical examination was within normal limits with the exception of the abdominal examination, which revealed a scaphoid abdomen with the appearance of a tubular mass extending from the right lower quadrant to the left upper quadrant. Minimal peristalsis was seen. Bowel sounds were hyperactive and high-pitched. The abdomen was diffusely tender to palpation and tympanitic to percussion. There was no hepatosplenomegaly. Rebound was diffusely present initially, but was not substantiated consistently on repeated examination. The rectal examination was normal and no stool was present for guaiac. 13:2 February 1984
Annals of Emergency Medicine
133/95
CHILDHOOD SIGMOID VOLVULUS Seger & Middleton
Fig. 1. Supine (A) and upright (B) ab-
dominal films show classic "coffee bean" configuration of sigmoid vol~lus. Laboratory data revealed a WBC of 6,800/mm3 and hemoglobin of 15.8 g/ dL. Electrolytes were within normal limits. Urinalysis and amylase were not obtained. A b d o m i n a l films revealed a greatly distended sigmoid loop occupying the right half of the abdomen. The ends of the horseshoeshaped loop were in the pelvis and the bow was near the diaphragm, forming the classic "coffee-bean" configuration (Figure 1). A barium enema revealed a tapered obstruction at the proximal sigmoid colon - - the typical "bird's beak" sign of sigmoid volvulus (Figure 2). The patient underwent sigmoidoscopy with a rigid sigmoidoscope. A soft rubber tube was passed beyond the obstruction, resulting in immediate decompression. Position of the tube was confirmed with fluoroscopy. 96/134
Subsequently a large (20-Fr) plastic rectal tube was passed over the smaller tube. The patient experienced immediate relief of his pain: During the following two weeks, the patient was maintained on a clear liquid diet with supplemental hyperalimentation. Prior to the removal of the rectal tube, m u l t i p l e b a r i u m enemas were attempted. These studies were unsatisfactory due to the persistence of stool in the colon despite frequent preparatory colon irrigations. Twelve days after admission, the barium enema revealed a large redundant sigmoid loop with normal motility and emptying. A rectal biopsy was performed the following day to rule out Hirschprung's disease. The biopsy was normal. Fifteen days after admission the patient underwent a descending sigmoid colectomy. At the time of surgery, there was evidence of chronic inflammation of the sigmoid mesentery. The pathologist's report noted mild and chronic mucosal inflammation and edema, focal hypertrophied musAnnals of Emergency Medicine
c u l a r i s p r o p r i a , and m i l d a c u t e serositis. The patient's postoperative course was uneventful. He was discharged from the hospital five weeks after his presentation to the emergency department.
DISCUSSION Sigmoid volvulus occurs when a redundant sigmoid loop rotates around its narrow m e s e n t e r i c a t t a c h m e n t . This produces venous and arterial obstruction of the mesentery, with rapid distention of the closed sigmoid. loop. Intraluminal pressure increases, exerting pressure on the submucosal plexuses. When the intraluminal pressure equals or exceeds the diastolic pressure, hemorrhagic infarction, perforation, and shock may occur.2, 3 Many factors contribute to the genesis of a sigmoid volvulus, one of which may be the age of the patient. In adults, sigmoid volvulus develops from a functional megacolon resulting from poor bowel habits, chronic constipation, excessive use of cathartics, and/or increased residue in the diet. 13:2 February1984
Fig. 2. Barium e n e m a shows typical "bird's beak" sign of sigmoid volvulus.
Contributing anatomical factors include the absence of peritoneal fixation of the sigmoid colon, narrowing of the mesocolon, or bands and adhesions.7 The etiology of the disorder in children is less well understood. Investigators postulate that children with the disorder have a congenital elongation of the sigmoid colon, with redundancy secondary to chronic constipation.Z,7, s However, barium studies reveal the same anatomic finding in children without the disorder. 9 Carter and Hinshaw 3 reported two distinct clinical entities occurring as a result of sigmoid volvulus. The acute fulminating type presents with sudden onset of severe crampy abdominal pain, distention, nausea, and vomiting. 2 This type is associated with early gangrene secondary to obstruction of the blood supply to the sigmoid loop, resulting in shock and circulatory collapse.2, 3 In contrast, subacute progressive sigmoid volvulus presents with less severe symptoms of gradual onset and a history of recurrent attacks. Necrosis is the result of tension ischemia secondary to extreme intracolic pressure. 3 A high index of suspicion is necessary to make the diagnosis of sigrnoid volvulus in children. Symptoms and roentgenographic findings in children with sigmoid volvulus m a y differ from those classically seen in adults.7 In adults, plain abdominal films frequently reveal a huge, dilated loop of colon with the "coffee-bean" or "bent inner tube" configuration. 13:2 February1984
In the pediatric age group, the abdominal films may reveal a nondiagnostic bowel gas pattern. Our case was an exception. There are, however, a number of roentgenographic findings in children with this entity which can be summarized as follows: s 1) nondiagnostic bowel gas pattern; 2) absence of the single sigmoid loop characteristic in adult patients; 3) proximal loop obstruction as distinct from the distal loop obstruction seen in adults; 4) hydrostatic reduction observed fluoroscopically at routine barium enema study; and 5) the "twisted taper" or "bird's beak" configuration of the barium column proximally at the site of the mesosigmoid and axial torsion. Obviously, due to the small number of reported cases, there is not a pathognomonic radiographic feature indicating the diagnosis of sigmoid volvulus. In adults, nonoperative detorsion followed by elective resection is the treatment of choice, ao The distal end of the obstruction can be visualized by sigmoidoscopy. Immediate surgery is indicated if m u c o s a l u l c e r a t i o n , sloughing, or dark blood is seen on sigmoidoscoy.lO T h r o u g h the sigmoidoscope, a long, Well-lubricated rectal tube can be passed gently beyond the site of obstruction. Tube deflation is successful in 80% to 90% of patients.lO Failure of tube deflation or signs of peritonitis are also indications for immediate operative intervention. Moreover, because of the frequency of recurrenee,2-4,n, 12 elective resection with primary anastomosis must be performed after detorsion. The management of children with sigmoid volvulus is controversial for several reasons: 1) there has been little experience with the disorder in children; 2} volvuhis is a difficult diagnosis to make in children; 3) the clinical course in children tends to be more fulminant, and the signs of gangrene and deterioration can be very subtle in the very young; 4) the passing of a rectal tube by sigrnoidoscopy can be very difficult or even impossible in children. In some small children, reduction of the volvulus can be accomplished by hydrostatic pressure during barium enema.6 Nevertheless, early operative intervention is required more often in the pediatric age group than in adults. Annals of Emergency Medicine
SUMMARY Acute large bowel obstruction resulting from sigmoid volvulus has a high m o r t a l i t y rate. The clinical course in children can be fulminant. The emergency physician must have a high index of suspicion and should be aware of the possible presentations of a child with this type of volvulus. The diagnosis of sigmoid volvulus should be considered in any patient of any age presenting with colonic obstruction. 14
REFERENCES 1. Miller EM: Gangrene of the sigmoid flexure of the colon due to volvulus. Arch Surg 1940;41:403-407. 2. Wilk PJ, Ross M: Sigmoid volvulus in an ll-year-old girl. A m J Dis Child 1974;127:400-402. 3. Carter R, Hinshaw DB: Acute sigmoid volvulus in children. A m J Dis Child 1961;101:631-634. 4. Hunter JG, Keats TF: Sigmoid volvulus in children: A case report. Am J Roentgenol Radium Ther Nucl Med 1970;108: 621-623. 5. Lillard RL, Allen RP, Nordstrom JE: Sigmoid volvulus in children: A case report. A m J Roentgenol Radium Ther Nucl Med 1966;97:223-226. 6. Ballantyne GH: Review of sigmoid volvulus: Clinical patterns and pathogenesis. Dis Colon Rectum 1982;25:823-830. 7. Nadalo LA, Ramirez H: The successful hydrostatic reduction of sigmoid volvulus in an infant: Case report and literature review. Milit Med 1980;145:132-134. 8. Taneja SB, Kalear A, Aygar RD: Sigmoid volvulus in childhood: Report of two cases. Dis Colon Rectum 1977;20:6267. 9. Dean GO, Murry JW: Volvulus of the sigmoid colon. A n n Surg 1952;135: 830-840. 10. Allen RP, Nordstrom JE: Volvulus of the sigmoid in children. A m l RoentgenoI Radium Ther Nucl Med 1964;91:690-693. 11. Storer EH, Goldberg SM, Nivatvongs S: Sigmoid volvulus, in Schwartz 8E (ed): Principles of Surgery, ed 2. Blakeston, New York, McGraw-Hill, 1974, p 11431144. 12. Keramidas DC, Skondras C, Anagnoston D, et ah Volvulus of the sigmoid colon. J Pediatr Surg 1979;14:479-480. 13. Metheny D, Nichols HE: Volvulus of the sigmoid. Surg Gynecol Obstet 1943; 76:239-246. 14. Campbell JR, Blank E: Sigmoid volvulus in children. Pediatrics 1974;53: 702-705. 135/97
Childhood Sigmoid Volvulus A 14-year-old boy presented to the emergency department with abdominal pain. Flat and upright abdominal films and a barium enema revealed the classic finddngs of sigmoid volv~lus. Sigmoidoscopy allowed a soft rubber tube to be passed beyond the obstruction with immediate relief of pain. The patient subsequently underwent a descending sigmoid colectomy. Sigmoid volvulus is a rare entity in childhood. Case discussion and review of the literature is presented. [Seger DL, Middleton D: Childhood sigmoid volvulus. Ann Emerg Med February 1984;13:133-135.]
INTRODUCTION Sigmoid volvulus is seldom considered in the differential diagnosis of abdominal pain in children. It is classically a disease of the elderly and is second only to carcinoma as a cause of acute large bowel obstruction. 1 Seventy percent of reported cases occur in patients over 50 years of age, with peak incidence at age 71.1-5 Sigmoid volvulus rarely occurs in children, as evidenced by 53 reported cases in the world literature and 22 reported cases in the English language. 6 The severity and potential consequences of unrecognized sigmoid volvulus can be devastating. The possibility of volvulus should be considered in any child presenting with a bowel obstruction. Our case report illustrates the classic features of sigmoid volvulus.
Donna L Seger, MD Don Middleton, MD Jacksonville, Florida From the Department of Emergency Medicine, University Hospital of Jacksonville, Jacksonville, Florida. Received for publication July 18, 1983. Revision received September 15, 1983. Accepted for publication November 8, 1983. Address for reprints: Donna L Seger, MD, Department of Emergency Medicine, University Hospital of Jacksonville, 655 West Eighth Street, Jacksonville, Florida 32209.
CASE REPORT A 14-year-old boy was admitted to University Hospital of Jacksonville with a four-day history of abdominal pain. He had been in good health until three days prior to admission. At that time he was awakened by epigastric and periumbilical pain. The severity of the pain waxed and waned for a few hours and then began to increase in intensity over the next 24 hours. During that period, the patient had two episodes of vomiting following meals. Two days prior to admission, the pain subsided but did not completely resolve. One episode of vomiting occurred. On the afternoon prior to admission the pain recurred, and there was another episode of vomiting. The pain did not abate, and the patient was brought to the emergency department. According to the child, his last bowel movement had been five days prior to admission. He denied hematemesis, melena, and hematochezia. The patient denied chronic constipation and stated that he had regular daily bowel habits until the onset of his illness. On physical examination, the patient appeared to be in moderate distress. Vital signs were as follows: temperature, 37.7 C; pulse, 132 beats per minute; respirations, 28/rain; blood pressure 110/70 m m Hg; and weight, 35 kg. Physical examination was within normal limits with the exception of the abdominal examination, which revealed a scaphoid abdomen with the appearance of a tubular mass extending from the right lower quadrant to the left upper quadrant. Minimal peristalsis was seen. Bowel sounds were hyperactive and high-pitched. The abdomen was diffusely tender to palpation and tympanitic to percussion. There was no hepatosplenomegaly. Rebound was diffusely present initially, but was not substantiated consistently on repeated examination. The rectal examination was normal and no stool was present for guaiac. 13:2 February 1984
Annals of Emergency Medicine
133/95
CHILDHOOD SIGMOID VOLVULUS Seger & Middleton
Fig. 1. Supine (A) and upright (B) ab-
dominal films show classic "coffee bean" configuration of sigmoid vol~lus. Laboratory data revealed a WBC of 6,800/mm3 and hemoglobin of 15.8 g/ dL. Electrolytes were within normal limits. Urinalysis and amylase were not obtained. A b d o m i n a l films revealed a greatly distended sigmoid loop occupying the right half of the abdomen. The ends of the horseshoeshaped loop were in the pelvis and the bow was near the diaphragm, forming the classic "coffee-bean" configuration (Figure 1). A barium enema revealed a tapered obstruction at the proximal sigmoid colon - - the typical "bird's beak" sign of sigmoid volvulus (Figure 2). The patient underwent sigmoidoscopy with a rigid sigmoidoscope. A soft rubber tube was passed beyond the obstruction, resulting in immediate decompression. Position of the tube was confirmed with fluoroscopy. 96/134
Subsequently a large (20-Fr) plastic rectal tube was passed over the smaller tube. The patient experienced immediate relief of his pain: During the following two weeks, the patient was maintained on a clear liquid diet with supplemental hyperalimentation. Prior to the removal of the rectal tube, m u l t i p l e b a r i u m enemas were attempted. These studies were unsatisfactory due to the persistence of stool in the colon despite frequent preparatory colon irrigations. Twelve days after admission, the barium enema revealed a large redundant sigmoid loop with normal motility and emptying. A rectal biopsy was performed the following day to rule out Hirschprung's disease. The biopsy was normal. Fifteen days after admission the patient underwent a descending sigmoid colectomy. At the time of surgery, there was evidence of chronic inflammation of the sigmoid mesentery. The pathologist's report noted mild and chronic mucosal inflammation and edema, focal hypertrophied musAnnals of Emergency Medicine
c u l a r i s p r o p r i a , and m i l d a c u t e serositis. The patient's postoperative course was uneventful. He was discharged from the hospital five weeks after his presentation to the emergency department.
DISCUSSION Sigmoid volvulus occurs when a redundant sigmoid loop rotates around its narrow m e s e n t e r i c a t t a c h m e n t . This produces venous and arterial obstruction of the mesentery, with rapid distention of the closed sigmoid. loop. Intraluminal pressure increases, exerting pressure on the submucosal plexuses. When the intraluminal pressure equals or exceeds the diastolic pressure, hemorrhagic infarction, perforation, and shock may occur.2, 3 Many factors contribute to the genesis of a sigmoid volvulus, one of which may be the age of the patient. In adults, sigmoid volvulus develops from a functional megacolon resulting from poor bowel habits, chronic constipation, excessive use of cathartics, and/or increased residue in the diet. 13:2 February1984
Fig. 2. Barium e n e m a shows typical "bird's beak" sign of sigmoid volvulus.
Contributing anatomical factors include the absence of peritoneal fixation of the sigmoid colon, narrowing of the mesocolon, or bands and adhesions.7 The etiology of the disorder in children is less well understood. Investigators postulate that children with the disorder have a congenital elongation of the sigmoid colon, with redundancy secondary to chronic constipation.Z,7, s However, barium studies reveal the same anatomic finding in children without the disorder. 9 Carter and Hinshaw 3 reported two distinct clinical entities occurring as a result of sigmoid volvulus. The acute fulminating type presents with sudden onset of severe crampy abdominal pain, distention, nausea, and vomiting. 2 This type is associated with early gangrene secondary to obstruction of the blood supply to the sigmoid loop, resulting in shock and circulatory collapse.2, 3 In contrast, subacute progressive sigmoid volvulus presents with less severe symptoms of gradual onset and a history of recurrent attacks. Necrosis is the result of tension ischemia secondary to extreme intracolic pressure. 3 A high index of suspicion is necessary to make the diagnosis of sigrnoid volvulus in children. Symptoms and roentgenographic findings in children with sigmoid volvulus m a y differ from those classically seen in adults.7 In adults, plain abdominal films frequently reveal a huge, dilated loop of colon with the "coffee-bean" or "bent inner tube" configuration. 13:2 February1984
In the pediatric age group, the abdominal films may reveal a nondiagnostic bowel gas pattern. Our case was an exception. There are, however, a number of roentgenographic findings in children with this entity which can be summarized as follows: s 1) nondiagnostic bowel gas pattern; 2) absence of the single sigmoid loop characteristic in adult patients; 3) proximal loop obstruction as distinct from the distal loop obstruction seen in adults; 4) hydrostatic reduction observed fluoroscopically at routine barium enema study; and 5) the "twisted taper" or "bird's beak" configuration of the barium column proximally at the site of the mesosigmoid and axial torsion. Obviously, due to the small number of reported cases, there is not a pathognomonic radiographic feature indicating the diagnosis of sigmoid volvulus. In adults, nonoperative detorsion followed by elective resection is the treatment of choice, ao The distal end of the obstruction can be visualized by sigmoidoscopy. Immediate surgery is indicated if m u c o s a l u l c e r a t i o n , sloughing, or dark blood is seen on sigmoidoscoy.lO T h r o u g h the sigmoidoscope, a long, Well-lubricated rectal tube can be passed gently beyond the site of obstruction. Tube deflation is successful in 80% to 90% of patients.lO Failure of tube deflation or signs of peritonitis are also indications for immediate operative intervention. Moreover, because of the frequency of recurrenee,2-4,n, 12 elective resection with primary anastomosis must be performed after detorsion. The management of children with sigmoid volvulus is controversial for several reasons: 1) there has been little experience with the disorder in children; 2} volvuhis is a difficult diagnosis to make in children; 3) the clinical course in children tends to be more fulminant, and the signs of gangrene and deterioration can be very subtle in the very young; 4) the passing of a rectal tube by sigrnoidoscopy can be very difficult or even impossible in children. In some small children, reduction of the volvulus can be accomplished by hydrostatic pressure during barium enema.6 Nevertheless, early operative intervention is required more often in the pediatric age group than in adults. Annals of Emergency Medicine
SUMMARY Acute large bowel obstruction resulting from sigmoid volvulus has a high m o r t a l i t y rate. The clinical course in children can be fulminant. The emergency physician must have a high index of suspicion and should be aware of the possible presentations of a child with this type of volvulus. The diagnosis of sigmoid volvulus should be considered in any patient of any age presenting with colonic obstruction. 14
REFERENCES 1. Miller EM: Gangrene of the sigmoid flexure of the colon due to volvulus. Arch Surg 1940;41:403-407. 2. Wilk PJ, Ross M: Sigmoid volvulus in an ll-year-old girl. A m J Dis Child 1974;127:400-402. 3. Carter R, Hinshaw DB: Acute sigmoid volvulus in children. A m J Dis Child 1961;101:631-634. 4. Hunter JG, Keats TF: Sigmoid volvulus in children: A case report. Am J Roentgenol Radium Ther Nucl Med 1970;108: 621-623. 5. Lillard RL, Allen RP, Nordstrom JE: Sigmoid volvulus in children: A case report. A m J Roentgenol Radium Ther Nucl Med 1966;97:223-226. 6. Ballantyne GH: Review of sigmoid volvulus: Clinical patterns and pathogenesis. Dis Colon Rectum 1982;25:823-830. 7. Nadalo LA, Ramirez H: The successful hydrostatic reduction of sigmoid volvulus in an infant: Case report and literature review. Milit Med 1980;145:132-134. 8. Taneja SB, Kalear A, Aygar RD: Sigmoid volvulus in childhood: Report of two cases. Dis Colon Rectum 1977;20:6267. 9. Dean GO, Murry JW: Volvulus of the sigmoid colon. A n n Surg 1952;135: 830-840. 10. Allen RP, Nordstrom JE: Volvulus of the sigmoid in children. A m l RoentgenoI Radium Ther Nucl Med 1964;91:690-693. 11. Storer EH, Goldberg SM, Nivatvongs S: Sigmoid volvulus, in Schwartz 8E (ed): Principles of Surgery, ed 2. Blakeston, New York, McGraw-Hill, 1974, p 11431144. 12. Keramidas DC, Skondras C, Anagnoston D, et ah Volvulus of the sigmoid colon. J Pediatr Surg 1979;14:479-480. 13. Metheny D, Nichols HE: Volvulus of the sigmoid. Surg Gynecol Obstet 1943; 76:239-246. 14. Campbell JR, Blank E: Sigmoid volvulus in children. Pediatrics 1974;53: 702-705. 135/97