Eur J Pediatr DOI 10.1007/s00431-015-2489-5
SHORT COMMUNICATION
Presentation and endoscopic management of sigmoid volvulus in children Stéphanie Colinet & Julie Rebeuh & Frederic Gottrand & Nicolas Kalach & Isabelle Paquot & Djamal Djeddi & Gaelle Le Henaff & Laurent Rebouissoux & Virginie Robert & Laurent Michaud & on behalf of the French-speaking Pediatric Hepatology Gastroenterology and Nutrition Group (GFHGNP)
Received: 3 August 2014 / Revised: 20 November 2014 / Accepted: 9 January 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients.
Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy. Conclusion: Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence.
Communicated by Jaan Toelen S. Colinet (*) : I. Paquot Department of Pediatrics, CHC Liège Clinique de L’Espérance, Rue Saint-Nicolas 447-449, 4420 Liège, Belgium e-mail: [email protected] S. Colinet e-mail: [email protected] I. Paquot e-mail: [email protected]
N. Kalach Department of Pediatrics, Hôpital Saint-Vincent de Paul, Bd de Belfort, 59020 Lille, France e-mail: [email protected] D. Djeddi Department of Pediatrics, CHU Nord d’Amiens, Place Victor Pauchet, 80054 Amiens, France e-mail: [email protected]
J. Rebeuh Department of Pediatrics, CHU Strasbourg, Strasbourg, France e-mail: [email protected]
G. Le Henaff Department of Pediatrics, CHU de Nantes, Quai Moncousu 7, 44093 Nantes Cedex 1, France e-mail: [email protected]
F. Gottrand : L. Michaud Department of Pediatrics, CHRU Lille Hôpital Jeanne de Flandre, Av Oscar Lambret, 59037 Lille, France
L. Rebouissoux Department of Pediatrics, CHU Bordeaux Pellegrin , Place Amélie Raba-Léon, 33000 Bordeaux, France e-mail: [email protected]
F. Gottrand e-mail: [email protected] L. Michaud e-mail: [email protected]
V. Robert Department of Pediatrics, CHU PAU, Bd Hauterive, 64000 Pau, France e-mail: [email protected]
Eur J Pediatr
What is Known: • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.
Keywords Sigmoidoscopy . Sigmoidectomy . Endoscopic exsufflation . Children Abbreviations CIPO Chronic intestinal pseudo-obstruction
Introduction Sigmoid volvulus is a rare form of intestinal obstruction in children. It usually occurs in adult males after the age of 40 years [1, 2]. The diagnosis is based on clinical and radiological findings and seems more difficult to establish in children compared with adults; hence, a correct diagnosis can be easily missed or delayed [2, 3]. Morbidity and mortality rates can be high because of the risk of perforation and gangrene [1]. The present retrospective study was designed to record precisely the clinical presentation, treatment modalities, and outcome of children presenting with a sigmoid volvulus based on chart reviews of experienced pediatric gastroenterologists within the network of the French-speaking group of pediatric gastroenterology hepatology and nutrition (GFHGNP).
Patients and methods We studied retrospectively the charts of all the patients with sigmoid volvulus occurring during the last 11 years within the French-speaking group of pediatric gastroenterology, hepatology, and nutrition. Age, sex, medical history, symptoms and signs at diagnosis, medications, radiological features and other investigations, modalities of endoscopic and surgical procedures, recurrence rate, and complications were recorded.
Results Thirteen cases of sigmoid volvulus (six boys and seven girls) were reported during the period of 2001 to 2012. Their median age at diagnosis was 12.8 years (range, 15 months to 17 years). Associated diseases were observed in eight children (mental retardation [n=2], myopathy [n=2; with one child having
congenital myopathy associated with mental retardation], and chronic constipation [n=6; including one child with mental retardation]). Chronic intestinal pseudo-obstruction (CIPO) was suspected in two patients with severe constipation: one patient had recurrent episodes of volvulus of the cecum and transverse colon and the second child presented after repeated episodes of intestinal obstruction. In our series, no patient had Hirschsprung’s disease, anal stenosis, anal imperforation, intestinal malrotation, or omphalomesenteric abnormalities. Initial symptoms were abdominal pain (13/13), abdominal distension (11/13), nausea (7/13) and vomiting (7/13), constipation (3/13), or diarrhea (3/13). No patient presented with fever or melena. Clinical examination revealed abdominal tenderness (13/13), absence of stool in the rectum (6/13), muscular rigidity (3/13), and abdominal mass (1/13). Shock or signs of perforation were not observed in any of the patients. At the time of hospital admission, five children presented with recurrent abdominal symptoms for 3 days to 5 months. One child presented with recurrent symptoms of abdominal pain with an acute episode of abdominal distension and vomiting, during which the diagnosis of recurrent sigmoid volvulus was established (Table 1). All patients underwent a plain abdominal X-ray, which showed the presence of dilated sigmoid loops and air-fluid levels (12/13) (Fig. 1). The Bcoffee bean^ sign was present in 6/13 patients. Colic distension was considered moderate in 2/13 and severe in 11/13 patients. Eight children underwent barium enema, which confirm the final diagnosis of sigmoid volvulus in 5/8 cases. A bird’s beak sign was observed in 3/8 patients. Barium enema was not used for sigmoid reduction in our series. In four patients, the final diagnosis of sigmoid volvulus was confirmed by abdominal CT scan. Anorectal manometry was normal in seven patients screened after an acute episode. Rectal biopsies performed in ten patients showed no abnormalities, with presence of ganglion cells. The median diagnostic delay (time from first reported symptoms) was 360 h and varied from 3 h to 150 days in recurrent forms. No gangrene or perforation was observed in our series. First-line management consisted in flexible sigmoidoscopy and exsufflation in 12/13 patients, which was performed under general anesthesia in eight patients. With the patient in left lateral position, the flexible endoscope was advanced to the point of volvulus. A successful deflation was accompanied by a large amount of release of gas and liquid stool. Exsufflation was successful in these 12 patients, and no complications occurred. Endoscopic biopsies were performed in two patients. Just after exsufflation, a rectal tube was placed in four patients. Six patients presented recurrence of the sigmoid volvulus 3 days to 3 months after the first episode three of whom experienced more than one recurrence (maximum of three recurrences), and three of whom had a rectal tube. Sigmoid resection was performed in 11 patients for the prevention of recurrence, with primary anastomosis in 9/11 patients (Fig. 2).
Eur J Pediatr Table 1
Diagnosis of recurrent sigmoid volvulus
Patients
Age (year)
Associated malformations
Mode of presentation
Treatment
Recurrence
Surgical treatment
1 2 3 4
9 14 14 10
No No Constipation Constipation, POIC?
Acute Acute Acute Acute
Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation
2 1 3 3
5 6 7
15 15 1
Acute and recurrent Acute Recurrent
Endoscopic exsufflation Endoscopic exsufflation Surgical
8 9 10 11 12
17 15 16 10 14
No No Mental retardation, myopathy, POIC? Constipation Constipation No Myopathy Constipation
Acute Acute Acute Acute and recurrent Acute and recurrent
Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation
No No Cecal and transverse colon volvulus 1 No No No 1
Sigmoidectomy Sigmoidectomy Sigmoidectomy Sigmoidectomy with colostomy Sigmoidectomy Sigmoidectomy Sigmoidectomy
13
12
Mental retardation, constipation
Recurrent
Endoscopic exsufflation
No
Sigmoidectomy Sigmoidectomy Sigmoidectomy No Sigmoidectomy with colostomy No
Two patients underwent a colostomy. Histological studies were performed in five of the 11 operated patients and were normal in all cases. First-line sigmoidectomy was performed in the youngest (15 months of age), as he presented with multiple early recurrences of the volvulus despite a first spontaneous reduction while he was on total parenteral nutrition and received frequent rectal disimpaction. During the mean follow-up (from the time of diagnosis) of 16 months (range, 2 months to 4 years), five patients remained symptomatic: two patients had chronic constipation, with persistent colic dilatation in one of them. Two children presented during episodes of intestinal obstruction (multiple episodes in one child with a suspected CIPO). One child who underwent first-line sigmoidectomy presented with several postoperative
complications during follow-up: cecal volvulus 8 months later, colorectal anastomotic stenosis 9 months after surgery, and a transverse colon volvulus 18 months later. At the last followup, eight patients remain asymptomatic.
Fig. 1 X-ray of the abdomen showing a massively dilated bowel
Fig. 2 Intraoperative finding of a dilated sigmoid colon
Discussion Sigmoid volvulus is very rare and poorly described in children [1–4]. Herein, we report one of the largest pediatric series of patients with sigmoid volvulus. The present study report demonstrated clearly that the clinical diagnosis of this condition may be significantly delayed, although clinical symptoms are often suggestive of this condition [1, 3]. This is the first pediatric series showing, that endoscopic exsufflation is an efficient and safe treatment option, confirming data from adult patients. We did not observe a single complication related to this procedure in children. In adults, sigmoid volvulus is more
Eur J Pediatr
common in males after the fourth decade with a large geographic variation. A previous pediatric study indicated a median age at diagnosis of 7 years, which is in keeping with our findings, with male predominance (male/female ratio, 3.5:1), which is in contrast with the results of our series [2]. As in adults, the etiology of this condition is not clear in children; however, volvulus tends to occur in the setting of a long sigmoid colon with an elongated mesentery and narrow mesenteric attachment [5–7]. Several underlying diseases can predispose to sigmoid volvulus: Hirschsprung’s disease, omphalomesenteric abnormalities, intestinal malrotation, anal stenosis, chronic constipation, surgical adherence [1], prune belly syndrome, and mental retardation [2]. In our series, no patient had Hirschsprung’s disease; however, two patients had mental retardation, and CIPO was suspected in two patients but was not confirmed by histological data. As reported in the present series, the most prevalent symptoms were abdominal pain with abdominal distension and vomiting. Clinical examination showed a tympanic and distended abdomen, whereas rectal examination revealed an empty rectum. The association of these symptoms is very suggestive of sigmoid volvulus and should prompt its diagnosis in a child with predisposing condition (i.e., constipation) and dilated sigmoid on X-ray. Additional radiological signs as the coffee bean sign (i.e., an area of transparency that resembles the shape of a coffee bean) and air-fluid levels are frequently described [8]. As in adult patients, barium enema and, especially, abdominal CT are useful in the final diagnosis of sigmoid volvulus in children, as confirmed in our series. The bird’s beak configuration of the twisted colon is characteristic and increases the diagnostic accuracy by 20–30 % [5, 7]. Barium enema can also be used for volvulus reduction, with a success rate of 68–79 % with an early recurrence rate [11–35 %] and risk of perforation in cases of ischemia [2, 9]. The best management of acute sigmoid volvulus remains controversial. Our results suggest that nonsurgical reduction should be attempted first in children in the absence of signs of complications [1]. Sigmoidoscopic reduction of sigmoid volvulus has a success rate of 70–90 % in adults [5–7]. In the present series, sigmoidoscopic exsufflation and reduction were performed successfully in 12 patients. In addition, the endoscopic procedure has the advantage of allowing the visualization of the mucosa [5–7, 10]. In the absence of complications, endoscopic decompression is an emergency procedure that allows the stabilization of the patient and provides sufficient time to prepare the patient safely for definitive surgery. The use of flexible sigmoidoscopy has reduced the risk of iatrogenic perforations reported in the past when using rigid sigmoidoscopy to less than 3 % [2, 5, 6]. In our experience, the placement of a rectal tube for 24 to 72 h helped stabilize the patient further and prevent an
early relapse [5, 7, 11]. However, in our series, the recurrence rate was high (50–90 %), which was in keeping with findings from adult patients [5, 6, 10]. Thus, definitive elective sigmoid resection is strongly recommended during the initial hospital admission for most patients [5, 12]. Our study has several limitations. This is a retrospective study with only few centers that have responded and recall bias which may underestimate the frequency of this complication in children.
Conclusion Volvulus of the sigmoid is rare in children, and its diagnosis is often missed or delayed despite a suggestive clinical presentation. Sigmoidoscopic reduction is a successful first-line treatment option for patients without any signs of complications. Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.
Author’s contributions Dr Rebeuh has contributed to this work and has submitted 4 patients in this series. Dr Gottrand and Dr Michaud have contributed to this work and have submitted two patients in this series. Dr Kalach has contributed to this work and has submitted two patients in this series. Dr Djeddi has contributed to this work and submitted one patient in this series. Dr Le Henaff has contributed to this work and has submitted one patient in this series. Dr Rebouissoux has contributed to this work and has submitted one patient in this series. Dr Robert has contributed to this work and has submitted one patient in this series. Dr Colinet and Dr Paquot have contributed to this work and submitted one patient in this series. Dr Colinet have collected all the data and written this article in collaboration with Dr Michaud and Dr Gottrand.
References 1. Atamanalp SS, Yildirgan MI, Basoglu M, Kantarci M, Yilmaz I (2004) Sigmoid colon volvulus in children: review of 19 cases. Pediatr Surg Int 20:492–495 2. Feldman D (2000) The coffee bean sign. Radiology 216:178–179 3. Ismail A (1997) Recurrent colonic volvulus in children. J Pediatr Surg 32:1739–1742 4. Lal SK, Morgenstern R, Vinjirayer EP, Matin R (2006) Sigmoid volvulus an update. Gastrointest Endosc Clin N Am 16:175–187 5. Mellor MF, Drake DG (1994) Colonic volvulus in children: value of barium enema for diagnosis and treatment in 14 children. AJR Am J Roentgenol 162:1157–1159 6. Oren D, Atamanalp SS, Aydinli B, Yildirgan I, Basoglu M, Polat Y, Onbas O (2007) An algorithm for the management of sigmoid colon volvulus and the safety of primary resection: experience with 827 cases. Dis Colon Rectum 50:489–497 7. Osiro SB, Cunningham D, Shoja MM, Tubbs S, Gielecki J, Loukas M (2012) The twisted colon: a review of sigmoid volvulus. Am Surg 78:271–279
Eur J Pediatr 8. Puneet, Khanna R, Gangopadhyay AN, Shahoo SP, Khanna AK (2000) Sigmoid volvulus in childhood: report of six cases. Pediatr Surg Int 16:132–133 9. Raveenthiran V, Madiba TE, Atamanalp SS et al (2010) Volvulus of the sigmoid colon. Colorectal Dis 12:e1–e17 10. Salas S, Angel CA, Salas N, Murillo C, Swischuk L (2000) Sigmoid volvulus in children and adolescents. J Am Coll Surg 190:717–723
11. Smith SD, Golladay ES, Wagner C, Seibert JJ (1990) Sigmoid volvulus in childhood. South Med J 83(7):778– 781 12. Turan M, Sen M, Karadayi K, Koyuncu A, Topcu O, Yildirir C, Duman M (2004) Our sigmoid colon volvulus experience and benefits of colonoscope in detortion process. Rev Esp Enferm Dig 96:32–35
SHORT COMMUNICATION
Presentation and endoscopic management of sigmoid volvulus in children Stéphanie Colinet & Julie Rebeuh & Frederic Gottrand & Nicolas Kalach & Isabelle Paquot & Djamal Djeddi & Gaelle Le Henaff & Laurent Rebouissoux & Virginie Robert & Laurent Michaud & on behalf of the French-speaking Pediatric Hepatology Gastroenterology and Nutrition Group (GFHGNP)
Received: 3 August 2014 / Revised: 20 November 2014 / Accepted: 9 January 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract The aim of the present study was to evaluate clinical presentation and management of sigmoid volvulus in children, focusing on endoscopic reduction. In this retrospective multicenter study, we reviewed the charts of 13 patients with sigmoid volvulus. We recorded clinical symptoms, diagnostic methods, endoscopic or surgical therapy, and outcome. The children (seven girls, six boys) had a median age of 12.8 years (range, 15 months to 17 years) at initial presentation. Eight patients had associated diseases (e.g., chronic constipation, mental retardation, or myopathy). The initial symptoms were abdominal pain (13/13), abdominal distension (11/13), and vomiting (7/13), which were associated with abdominal tenderness in all patients.
Abdominal X-ray showed dilated sigmoid loops and air-fluid levels in all patients. Endoscopic reduction by exsufflation was successful without any complications in 12 patients, whereas the youngest patient underwent a first-line sigmoidectomy. Recurrence occurred in 7/12 patients after endoscopic exsufflation. Finally, 11 patients underwent a sigmoidectomy. Conclusion: Although rare in children, sigmoid volvulus should be advocated when abdominal pain is associated with dilated sigmoid loops. Sigmoidoscopic exsufflation can be considered as the first-line management in the absence of perforation. However, sigmoidectomy is often required for prevention of recurrence.
Communicated by Jaan Toelen S. Colinet (*) : I. Paquot Department of Pediatrics, CHC Liège Clinique de L’Espérance, Rue Saint-Nicolas 447-449, 4420 Liège, Belgium e-mail: [email protected] S. Colinet e-mail: [email protected] I. Paquot e-mail: [email protected]
N. Kalach Department of Pediatrics, Hôpital Saint-Vincent de Paul, Bd de Belfort, 59020 Lille, France e-mail: [email protected] D. Djeddi Department of Pediatrics, CHU Nord d’Amiens, Place Victor Pauchet, 80054 Amiens, France e-mail: [email protected]
J. Rebeuh Department of Pediatrics, CHU Strasbourg, Strasbourg, France e-mail: [email protected]
G. Le Henaff Department of Pediatrics, CHU de Nantes, Quai Moncousu 7, 44093 Nantes Cedex 1, France e-mail: [email protected]
F. Gottrand : L. Michaud Department of Pediatrics, CHRU Lille Hôpital Jeanne de Flandre, Av Oscar Lambret, 59037 Lille, France
L. Rebouissoux Department of Pediatrics, CHU Bordeaux Pellegrin , Place Amélie Raba-Léon, 33000 Bordeaux, France e-mail: [email protected]
F. Gottrand e-mail: [email protected] L. Michaud e-mail: [email protected]
V. Robert Department of Pediatrics, CHU PAU, Bd Hauterive, 64000 Pau, France e-mail: [email protected]
Eur J Pediatr
What is Known: • Sigmoid volvulus is uncommon in childhood. • Diagnosis is often missed or delayed. What is New: • This is the first pediatric series showing that endoscopic exsufflation is an efficient and safe treatment option. • Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.
Keywords Sigmoidoscopy . Sigmoidectomy . Endoscopic exsufflation . Children Abbreviations CIPO Chronic intestinal pseudo-obstruction
Introduction Sigmoid volvulus is a rare form of intestinal obstruction in children. It usually occurs in adult males after the age of 40 years [1, 2]. The diagnosis is based on clinical and radiological findings and seems more difficult to establish in children compared with adults; hence, a correct diagnosis can be easily missed or delayed [2, 3]. Morbidity and mortality rates can be high because of the risk of perforation and gangrene [1]. The present retrospective study was designed to record precisely the clinical presentation, treatment modalities, and outcome of children presenting with a sigmoid volvulus based on chart reviews of experienced pediatric gastroenterologists within the network of the French-speaking group of pediatric gastroenterology hepatology and nutrition (GFHGNP).
Patients and methods We studied retrospectively the charts of all the patients with sigmoid volvulus occurring during the last 11 years within the French-speaking group of pediatric gastroenterology, hepatology, and nutrition. Age, sex, medical history, symptoms and signs at diagnosis, medications, radiological features and other investigations, modalities of endoscopic and surgical procedures, recurrence rate, and complications were recorded.
Results Thirteen cases of sigmoid volvulus (six boys and seven girls) were reported during the period of 2001 to 2012. Their median age at diagnosis was 12.8 years (range, 15 months to 17 years). Associated diseases were observed in eight children (mental retardation [n=2], myopathy [n=2; with one child having
congenital myopathy associated with mental retardation], and chronic constipation [n=6; including one child with mental retardation]). Chronic intestinal pseudo-obstruction (CIPO) was suspected in two patients with severe constipation: one patient had recurrent episodes of volvulus of the cecum and transverse colon and the second child presented after repeated episodes of intestinal obstruction. In our series, no patient had Hirschsprung’s disease, anal stenosis, anal imperforation, intestinal malrotation, or omphalomesenteric abnormalities. Initial symptoms were abdominal pain (13/13), abdominal distension (11/13), nausea (7/13) and vomiting (7/13), constipation (3/13), or diarrhea (3/13). No patient presented with fever or melena. Clinical examination revealed abdominal tenderness (13/13), absence of stool in the rectum (6/13), muscular rigidity (3/13), and abdominal mass (1/13). Shock or signs of perforation were not observed in any of the patients. At the time of hospital admission, five children presented with recurrent abdominal symptoms for 3 days to 5 months. One child presented with recurrent symptoms of abdominal pain with an acute episode of abdominal distension and vomiting, during which the diagnosis of recurrent sigmoid volvulus was established (Table 1). All patients underwent a plain abdominal X-ray, which showed the presence of dilated sigmoid loops and air-fluid levels (12/13) (Fig. 1). The Bcoffee bean^ sign was present in 6/13 patients. Colic distension was considered moderate in 2/13 and severe in 11/13 patients. Eight children underwent barium enema, which confirm the final diagnosis of sigmoid volvulus in 5/8 cases. A bird’s beak sign was observed in 3/8 patients. Barium enema was not used for sigmoid reduction in our series. In four patients, the final diagnosis of sigmoid volvulus was confirmed by abdominal CT scan. Anorectal manometry was normal in seven patients screened after an acute episode. Rectal biopsies performed in ten patients showed no abnormalities, with presence of ganglion cells. The median diagnostic delay (time from first reported symptoms) was 360 h and varied from 3 h to 150 days in recurrent forms. No gangrene or perforation was observed in our series. First-line management consisted in flexible sigmoidoscopy and exsufflation in 12/13 patients, which was performed under general anesthesia in eight patients. With the patient in left lateral position, the flexible endoscope was advanced to the point of volvulus. A successful deflation was accompanied by a large amount of release of gas and liquid stool. Exsufflation was successful in these 12 patients, and no complications occurred. Endoscopic biopsies were performed in two patients. Just after exsufflation, a rectal tube was placed in four patients. Six patients presented recurrence of the sigmoid volvulus 3 days to 3 months after the first episode three of whom experienced more than one recurrence (maximum of three recurrences), and three of whom had a rectal tube. Sigmoid resection was performed in 11 patients for the prevention of recurrence, with primary anastomosis in 9/11 patients (Fig. 2).
Eur J Pediatr Table 1
Diagnosis of recurrent sigmoid volvulus
Patients
Age (year)
Associated malformations
Mode of presentation
Treatment
Recurrence
Surgical treatment
1 2 3 4
9 14 14 10
No No Constipation Constipation, POIC?
Acute Acute Acute Acute
Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation
2 1 3 3
5 6 7
15 15 1
Acute and recurrent Acute Recurrent
Endoscopic exsufflation Endoscopic exsufflation Surgical
8 9 10 11 12
17 15 16 10 14
No No Mental retardation, myopathy, POIC? Constipation Constipation No Myopathy Constipation
Acute Acute Acute Acute and recurrent Acute and recurrent
Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation Endoscopic exsufflation
No No Cecal and transverse colon volvulus 1 No No No 1
Sigmoidectomy Sigmoidectomy Sigmoidectomy Sigmoidectomy with colostomy Sigmoidectomy Sigmoidectomy Sigmoidectomy
13
12
Mental retardation, constipation
Recurrent
Endoscopic exsufflation
No
Sigmoidectomy Sigmoidectomy Sigmoidectomy No Sigmoidectomy with colostomy No
Two patients underwent a colostomy. Histological studies were performed in five of the 11 operated patients and were normal in all cases. First-line sigmoidectomy was performed in the youngest (15 months of age), as he presented with multiple early recurrences of the volvulus despite a first spontaneous reduction while he was on total parenteral nutrition and received frequent rectal disimpaction. During the mean follow-up (from the time of diagnosis) of 16 months (range, 2 months to 4 years), five patients remained symptomatic: two patients had chronic constipation, with persistent colic dilatation in one of them. Two children presented during episodes of intestinal obstruction (multiple episodes in one child with a suspected CIPO). One child who underwent first-line sigmoidectomy presented with several postoperative
complications during follow-up: cecal volvulus 8 months later, colorectal anastomotic stenosis 9 months after surgery, and a transverse colon volvulus 18 months later. At the last followup, eight patients remain asymptomatic.
Fig. 1 X-ray of the abdomen showing a massively dilated bowel
Fig. 2 Intraoperative finding of a dilated sigmoid colon
Discussion Sigmoid volvulus is very rare and poorly described in children [1–4]. Herein, we report one of the largest pediatric series of patients with sigmoid volvulus. The present study report demonstrated clearly that the clinical diagnosis of this condition may be significantly delayed, although clinical symptoms are often suggestive of this condition [1, 3]. This is the first pediatric series showing, that endoscopic exsufflation is an efficient and safe treatment option, confirming data from adult patients. We did not observe a single complication related to this procedure in children. In adults, sigmoid volvulus is more
Eur J Pediatr
common in males after the fourth decade with a large geographic variation. A previous pediatric study indicated a median age at diagnosis of 7 years, which is in keeping with our findings, with male predominance (male/female ratio, 3.5:1), which is in contrast with the results of our series [2]. As in adults, the etiology of this condition is not clear in children; however, volvulus tends to occur in the setting of a long sigmoid colon with an elongated mesentery and narrow mesenteric attachment [5–7]. Several underlying diseases can predispose to sigmoid volvulus: Hirschsprung’s disease, omphalomesenteric abnormalities, intestinal malrotation, anal stenosis, chronic constipation, surgical adherence [1], prune belly syndrome, and mental retardation [2]. In our series, no patient had Hirschsprung’s disease; however, two patients had mental retardation, and CIPO was suspected in two patients but was not confirmed by histological data. As reported in the present series, the most prevalent symptoms were abdominal pain with abdominal distension and vomiting. Clinical examination showed a tympanic and distended abdomen, whereas rectal examination revealed an empty rectum. The association of these symptoms is very suggestive of sigmoid volvulus and should prompt its diagnosis in a child with predisposing condition (i.e., constipation) and dilated sigmoid on X-ray. Additional radiological signs as the coffee bean sign (i.e., an area of transparency that resembles the shape of a coffee bean) and air-fluid levels are frequently described [8]. As in adult patients, barium enema and, especially, abdominal CT are useful in the final diagnosis of sigmoid volvulus in children, as confirmed in our series. The bird’s beak configuration of the twisted colon is characteristic and increases the diagnostic accuracy by 20–30 % [5, 7]. Barium enema can also be used for volvulus reduction, with a success rate of 68–79 % with an early recurrence rate [11–35 %] and risk of perforation in cases of ischemia [2, 9]. The best management of acute sigmoid volvulus remains controversial. Our results suggest that nonsurgical reduction should be attempted first in children in the absence of signs of complications [1]. Sigmoidoscopic reduction of sigmoid volvulus has a success rate of 70–90 % in adults [5–7]. In the present series, sigmoidoscopic exsufflation and reduction were performed successfully in 12 patients. In addition, the endoscopic procedure has the advantage of allowing the visualization of the mucosa [5–7, 10]. In the absence of complications, endoscopic decompression is an emergency procedure that allows the stabilization of the patient and provides sufficient time to prepare the patient safely for definitive surgery. The use of flexible sigmoidoscopy has reduced the risk of iatrogenic perforations reported in the past when using rigid sigmoidoscopy to less than 3 % [2, 5, 6]. In our experience, the placement of a rectal tube for 24 to 72 h helped stabilize the patient further and prevent an
early relapse [5, 7, 11]. However, in our series, the recurrence rate was high (50–90 %), which was in keeping with findings from adult patients [5, 6, 10]. Thus, definitive elective sigmoid resection is strongly recommended during the initial hospital admission for most patients [5, 12]. Our study has several limitations. This is a retrospective study with only few centers that have responded and recall bias which may underestimate the frequency of this complication in children.
Conclusion Volvulus of the sigmoid is rare in children, and its diagnosis is often missed or delayed despite a suggestive clinical presentation. Sigmoidoscopic reduction is a successful first-line treatment option for patients without any signs of complications. Elective sigmoid resection with primary anastomosis is often required to prevent recurrence.
Author’s contributions Dr Rebeuh has contributed to this work and has submitted 4 patients in this series. Dr Gottrand and Dr Michaud have contributed to this work and have submitted two patients in this series. Dr Kalach has contributed to this work and has submitted two patients in this series. Dr Djeddi has contributed to this work and submitted one patient in this series. Dr Le Henaff has contributed to this work and has submitted one patient in this series. Dr Rebouissoux has contributed to this work and has submitted one patient in this series. Dr Robert has contributed to this work and has submitted one patient in this series. Dr Colinet and Dr Paquot have contributed to this work and submitted one patient in this series. Dr Colinet have collected all the data and written this article in collaboration with Dr Michaud and Dr Gottrand.
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