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Colonic volvulus: A rare presentation of Hirschsprung's disease
Colonic Volvulus: A Rare Presentation of Hirschsprung’s By Aklle
Sarioglu,
F. Cahit Tanyel,
Nebil Ankara,
b Although rare, patients with Hirschsprung’s disease (HD) may be admitted with colonic volvulus. Among 302 patients with HD two patients were admitted with colonic volvulus (0.66%). Involved segments were cecum and sigmoid colon. During the same period, 11 patients including those with HD were diagnosed with colonic volvulus. An aganglionic segment below sigmoid colon, and a freely mobile mesosigmoid seem to be the cause of sigmoid volvulus. However, extension of aganglionosis above the sigmoid colon in addition to anatomic tendency seems to result in volvulus of proximal segments. Because HD has been associated with colonic volvulus, children who present with colonic volvulus should be suspect for underlying HD. Copyrigftt o 1997 by W.B. Saunders Company
Btiytikpamuk~u,
Hirschsprung’s
disease,
cecal
volvulus,
Table 1. Patients Study
A
CASE Case
Age/Sex
Dean et aI5
4 yr/M
Hirschsprung’s
Disease
Treatment
TVP~
Srgmoid
and Colonic
Result
Detorsion Enema
Volvulus
Recovered +
et al6 et al7
12 yr/M 3 yr/M
Srgmord Sigmoid
operation Detorsion Not recorded
Ciardimi et al7 Valla et al*
5 yr/M
Sigmoid
Not recorded
Not recorded
5 d/M 5 yr/M
Srgmoid Sigmoid
Resection Resection
Recovered Recovered
2 d/M
Sigmord
Detorsron
Sheperd Crardimi
Valla et al* McCalla
et aI9
Dred Not recorded
Recovered
Reoperation
+
colostomy et al4
11 yr/M
Transverse
Detorsron
+
Recovered
Cecal
colostomy Detorsion + colostomy
Present
series
Present
series
5 d/M
Recovered
11 yr/M
Srgmoid
Detorsion + colostomy
Recovered
tresptration rate, Wmin) and abdommal distenston and tenderness. The plain abdominal radtograph in the erect position showed air fluid levels. Following Initial resuscttation he underwent emergency laparotomy. Exploratton showed a 270-degree counterclockwise volvulus of the stgmoid colon. The srgmoid colon was found to be viable after detorsion The appearance of the bowel suggested HD Biopsy of the rectal wall above the peritoneal reflection confirmed the diagnoses. Therefore. a transverse colostomy was performed. He subsequently underwent a Swenson procedure and closure of the stoma He IS asymptomahc and doing well 1.5 years after the mrtral operation.
DISCUSSION
REPORTS
I
A 5-day-old boy was admttted with farlure to pass meconium and bilious vomiting. His parents refused hospitalization on hts second day of life. Because his general condition worsened. he was admitted to our department. Physical examination showed a dehydrated newborn boy with severe abdominal distension. Plain abdominal radiographs showed au fluid levels with dtstended mtestinal loops. After imtial fluid resuscitation, the patient underwent laparotomy through a transverse abdominal incisron. Abdominal exploration showed a near 270.degree counterclockwise twisting of cecum. Following detorsion. the twisted segment was found to be viable. Long-segment HD extendmg up to the descending colon was considered and a transverse colostomy was performed. A rectal biopsy confirmed the diagnoses of HD. A Swenson pull-through operation was performed at 2 years of age. After this operation, an ileorectal fistula occurred which requued operattve closure. He did well and currently is doing well after 3 years of follow-up. Case
With
sig-
LTHOUGH INTESTINAL obstruction is the common mode of presentation of Hirschsprung’s disease, rare clinical forms and some underestimated complications. especially those requiring emergency surgical treatment may cause diagnostic difficulties.‘-3 If the association of those complications with HD remains undiagnosed, life-threatening conditions may occur. Colonic volvulus is one of the rare complications of HD with only eight cases reported to date.4-9 Two additional cases of colonic volvulus complicating HD are reported herein to stress the place of HD among the etiologic factors of colonic volvulus during childhood.
HiGs6nmez
Turkey
Neilson
INDEX WORDS: moid volvulus.
and Akgtin
Disease
Colonic volvulus is an unusual occurrence in children. Colonic volvulus may involve the cecum, transverse colon. or sigmoid colon. It is known that cecal volvulus might be the result of a mobile cecum in the presence of precipitating factors such as distension of the cecum, obstructing lesions of the left colon, high-roughage diet, chronic constipation and frequent purgation, pregnancy, artificial ventilation and previous abdominal surgery.‘” Sigmoid volvulus is more common in Eastern Europe and Asia.‘O The anatomic features of sigmoid volvulus are a long and freely mobile sigmoid colon and mesosigmoid and a short mesenteric attachment of the proximal and distal mesocolic limbs.‘”
2
An II-year-old boy was admitted to the hospital wtth abdominal pain, abdominal distension that caused dyspnea. and bilious vomiting of l-day duration. The past history showed chronic constipation since birth. The physical examination at admission revealed tachypnea Journal
of Pediatric
Surgery,
Vol32,
No 1 (January),
1997: pp 117-l 18
117
118
SARIOGLU
Both sigmoid volvulus and transverse colon volvulus are rarely associated with HD.4s5Between 1976 and 1993, among 302 patients with HD, only two (0.66%) patients at our institution had a colonic volvulus. As far as we know no instances of cecal volvulus associated with HD have been reported in the literature, and only eight patients with Hirschsprung’s disease had a colonic volvulus. All were boys (Table 1).4-9One of these patients had volvulus of the transverse colon and of the others had sigmoid volvulu~.~ The evaluation of these 10 cases suggests that the incidence of HD-associated colonic volvulus increases with age (Table 1). Although there are three reported neonates, other patients are older than 3 years of age. It seems that patients with sigmoid volvulus have shortsegment HD. If the mesosigmoid is freely mobile, the dilated ganglionic sigmoid colon seems to have a predilec-
ET AL
tion to sigmoid volvulus. However, if the involved segment extends beyond sigmoid colon, other segments such as transverse colon and cecum may undergo volvulus in the presence of anatomic factors. Among patients with colonic volvulus, HD is an important etiologic factor. During the same period 11 patients, including those with HD have been encountered in the same instution. Five patients had a sigmoid volvulus and five patients have had a cecal volvulus, but only one had a transverse colonic volvulus. Each one of five sigmoid or cecal volvuluses have been found to be associated with HD. Therefore, among children who present with colonic volvulus. HD should be considered. Past history examination should be directed toward the signs and symptoms of HD. If colonic volvulus is encountered during emergency laparotomy, the colon should be evaluated for the presence of HD.
REFERENCES I. Sieber WK: Hirschsprung’s disease, m Welch KJ, Randolph JG, Ravitch MM, et al teds): Pedtatric Surgery, chap 106. Chicago, IL, Year Book Medical, 1986, pp 995-1019 2. Sarioglu A, Tanyel FC, Btiytikpamuk9u N, et al. Appendiceal perforation: A potentially lethal initial mode of presentation of Hirschsprung’s disease. J Pediatr Surg 32: 123-124, 1997 3. Akgtir F, Tanyel FC. Btiytikpamukq N, et al: Colonic atresia and Hirschsprung’s disease association reveals further evidences for migration of enteric neurons. J Pediatr Surg 28:635-636, 1993 4. Neilson IR, Youssef S: Delayed presentation of Hirschsprung’s disease: Acute obstruction secondary to megacolon with transverse colonic volvulus. J Pediatr Surg 25: 1177-I 179, 1990
5. Dean GO, Murry JW: Volvulus of the stgmoid colon. Ann Surg 135:830-840. 1952 6. Sheperd JJ: The epidemiology and clinical presentatton of sigmoid volvulus. Br J Surg 56:353, 1969 7. Ciardimt A, Vizzom L, Dimelli P: I1 volvola de1 sigma in eta infantile. Osp Ital Ped 12:663-675. 1977 8 Valla JS, Luis D, Berard J. et al: Volvulus du stgmoide chez I’enfant. Char Pediatr 23.93-96, 1982 9. McCalla TH, Arensman RM. Falterman KW: Stgmoid volvulus in children. Am Surg 9:514-519. 1985 10. Shackelford RT, Zuidema GD: Diseases of the colon, in Shackelford RT. Zuidema GD (eds): Surgery of the Alimentary Tract, chap 5 (vol 3). Philadelphia, PA, Saunders. 1982. pp 75-189
Sarioglu,
F. Cahit Tanyel,
Nebil Ankara,
b Although rare, patients with Hirschsprung’s disease (HD) may be admitted with colonic volvulus. Among 302 patients with HD two patients were admitted with colonic volvulus (0.66%). Involved segments were cecum and sigmoid colon. During the same period, 11 patients including those with HD were diagnosed with colonic volvulus. An aganglionic segment below sigmoid colon, and a freely mobile mesosigmoid seem to be the cause of sigmoid volvulus. However, extension of aganglionosis above the sigmoid colon in addition to anatomic tendency seems to result in volvulus of proximal segments. Because HD has been associated with colonic volvulus, children who present with colonic volvulus should be suspect for underlying HD. Copyrigftt o 1997 by W.B. Saunders Company
Btiytikpamuk~u,
Hirschsprung’s
disease,
cecal
volvulus,
Table 1. Patients Study
A
CASE Case
Age/Sex
Dean et aI5
4 yr/M
Hirschsprung’s
Disease
Treatment
TVP~
Srgmoid
and Colonic
Result
Detorsion Enema
Volvulus
Recovered +
et al6 et al7
12 yr/M 3 yr/M
Srgmord Sigmoid
operation Detorsion Not recorded
Ciardimi et al7 Valla et al*
5 yr/M
Sigmoid
Not recorded
Not recorded
5 d/M 5 yr/M
Srgmoid Sigmoid
Resection Resection
Recovered Recovered
2 d/M
Sigmord
Detorsron
Sheperd Crardimi
Valla et al* McCalla
et aI9
Dred Not recorded
Recovered
Reoperation
+
colostomy et al4
11 yr/M
Transverse
Detorsron
+
Recovered
Cecal
colostomy Detorsion + colostomy
Present
series
Present
series
5 d/M
Recovered
11 yr/M
Srgmoid
Detorsion + colostomy
Recovered
tresptration rate, Wmin) and abdommal distenston and tenderness. The plain abdominal radtograph in the erect position showed air fluid levels. Following Initial resuscttation he underwent emergency laparotomy. Exploratton showed a 270-degree counterclockwise volvulus of the stgmoid colon. The srgmoid colon was found to be viable after detorsion The appearance of the bowel suggested HD Biopsy of the rectal wall above the peritoneal reflection confirmed the diagnoses. Therefore. a transverse colostomy was performed. He subsequently underwent a Swenson procedure and closure of the stoma He IS asymptomahc and doing well 1.5 years after the mrtral operation.
DISCUSSION
REPORTS
I
A 5-day-old boy was admttted with farlure to pass meconium and bilious vomiting. His parents refused hospitalization on hts second day of life. Because his general condition worsened. he was admitted to our department. Physical examination showed a dehydrated newborn boy with severe abdominal distension. Plain abdominal radiographs showed au fluid levels with dtstended mtestinal loops. After imtial fluid resuscitation, the patient underwent laparotomy through a transverse abdominal incisron. Abdominal exploration showed a near 270.degree counterclockwise twisting of cecum. Following detorsion. the twisted segment was found to be viable. Long-segment HD extendmg up to the descending colon was considered and a transverse colostomy was performed. A rectal biopsy confirmed the diagnoses of HD. A Swenson pull-through operation was performed at 2 years of age. After this operation, an ileorectal fistula occurred which requued operattve closure. He did well and currently is doing well after 3 years of follow-up. Case
With
sig-
LTHOUGH INTESTINAL obstruction is the common mode of presentation of Hirschsprung’s disease, rare clinical forms and some underestimated complications. especially those requiring emergency surgical treatment may cause diagnostic difficulties.‘-3 If the association of those complications with HD remains undiagnosed, life-threatening conditions may occur. Colonic volvulus is one of the rare complications of HD with only eight cases reported to date.4-9 Two additional cases of colonic volvulus complicating HD are reported herein to stress the place of HD among the etiologic factors of colonic volvulus during childhood.
HiGs6nmez
Turkey
Neilson
INDEX WORDS: moid volvulus.
and Akgtin
Disease
Colonic volvulus is an unusual occurrence in children. Colonic volvulus may involve the cecum, transverse colon. or sigmoid colon. It is known that cecal volvulus might be the result of a mobile cecum in the presence of precipitating factors such as distension of the cecum, obstructing lesions of the left colon, high-roughage diet, chronic constipation and frequent purgation, pregnancy, artificial ventilation and previous abdominal surgery.‘” Sigmoid volvulus is more common in Eastern Europe and Asia.‘O The anatomic features of sigmoid volvulus are a long and freely mobile sigmoid colon and mesosigmoid and a short mesenteric attachment of the proximal and distal mesocolic limbs.‘”
2
An II-year-old boy was admitted to the hospital wtth abdominal pain, abdominal distension that caused dyspnea. and bilious vomiting of l-day duration. The past history showed chronic constipation since birth. The physical examination at admission revealed tachypnea Journal
of Pediatric
Surgery,
Vol32,
No 1 (January),
1997: pp 117-l 18
117
118
SARIOGLU
Both sigmoid volvulus and transverse colon volvulus are rarely associated with HD.4s5Between 1976 and 1993, among 302 patients with HD, only two (0.66%) patients at our institution had a colonic volvulus. As far as we know no instances of cecal volvulus associated with HD have been reported in the literature, and only eight patients with Hirschsprung’s disease had a colonic volvulus. All were boys (Table 1).4-9One of these patients had volvulus of the transverse colon and of the others had sigmoid volvulu~.~ The evaluation of these 10 cases suggests that the incidence of HD-associated colonic volvulus increases with age (Table 1). Although there are three reported neonates, other patients are older than 3 years of age. It seems that patients with sigmoid volvulus have shortsegment HD. If the mesosigmoid is freely mobile, the dilated ganglionic sigmoid colon seems to have a predilec-
ET AL
tion to sigmoid volvulus. However, if the involved segment extends beyond sigmoid colon, other segments such as transverse colon and cecum may undergo volvulus in the presence of anatomic factors. Among patients with colonic volvulus, HD is an important etiologic factor. During the same period 11 patients, including those with HD have been encountered in the same instution. Five patients had a sigmoid volvulus and five patients have had a cecal volvulus, but only one had a transverse colonic volvulus. Each one of five sigmoid or cecal volvuluses have been found to be associated with HD. Therefore, among children who present with colonic volvulus. HD should be considered. Past history examination should be directed toward the signs and symptoms of HD. If colonic volvulus is encountered during emergency laparotomy, the colon should be evaluated for the presence of HD.
REFERENCES I. Sieber WK: Hirschsprung’s disease, m Welch KJ, Randolph JG, Ravitch MM, et al teds): Pedtatric Surgery, chap 106. Chicago, IL, Year Book Medical, 1986, pp 995-1019 2. Sarioglu A, Tanyel FC, Btiytikpamuk9u N, et al. Appendiceal perforation: A potentially lethal initial mode of presentation of Hirschsprung’s disease. J Pediatr Surg 32: 123-124, 1997 3. Akgtir F, Tanyel FC. Btiytikpamukq N, et al: Colonic atresia and Hirschsprung’s disease association reveals further evidences for migration of enteric neurons. J Pediatr Surg 28:635-636, 1993 4. Neilson IR, Youssef S: Delayed presentation of Hirschsprung’s disease: Acute obstruction secondary to megacolon with transverse colonic volvulus. J Pediatr Surg 25: 1177-I 179, 1990
5. Dean GO, Murry JW: Volvulus of the stgmoid colon. Ann Surg 135:830-840. 1952 6. Sheperd JJ: The epidemiology and clinical presentatton of sigmoid volvulus. Br J Surg 56:353, 1969 7. Ciardimt A, Vizzom L, Dimelli P: I1 volvola de1 sigma in eta infantile. Osp Ital Ped 12:663-675. 1977 8 Valla JS, Luis D, Berard J. et al: Volvulus du stgmoide chez I’enfant. Char Pediatr 23.93-96, 1982 9. McCalla TH, Arensman RM. Falterman KW: Stgmoid volvulus in children. Am Surg 9:514-519. 1985 10. Shackelford RT, Zuidema GD: Diseases of the colon, in Shackelford RT. Zuidema GD (eds): Surgery of the Alimentary Tract, chap 5 (vol 3). Philadelphia, PA, Saunders. 1982. pp 75-189