INTRODUCTION

Sigmoid volvulus (SV) is a rare cause of intestinal obstruction in children¹. Consequently, most published studies on this topic are case reports^2-6. These reports highlight an association of SV with chronic constipation (10%) and Hirschsprung’s disease (HD) (10%)⁷.

Anorectal anomalies, such as imperforate anus, represent a condition that can trigger constipation and has also been associated with SV⁸, although they are less commonly reported in the literature.

We report the case of a 6-year-old boy with chronic constipation due to an imperforate anus and poor adherence to outpatient treatment, who developed a severe sigmoid volvulus requiring Hartmann’s procedure. The patient showed significant clinical improvement following surgery.

The main objective of this report is to contribute to knowledge about a rare condition in children.

METHODS

RESULTS

Case report

A 6-year-old patient, who had undergone cutback anoplasty 5 years prior due to an imperforate anus, was under regular outpatient follow-up with no other comorbidities. During follow-up, the patient presented chronic constipation and required ambulatory fecal impaction removal in the surgical center 17 times.

During the most recent outpatient visit, a significant fecal impaction was again identified, requiring surgical removal. Following the procedure, the patient remained hemodynamically stable, but experienced prostration, abdominal pain, poor oral intake tolerance, and diffuse abdominal tenderness on palpation, without signs of peritonitis. An abdominal X-ray was performed (figure 1), and the patient was started on a regimen of antibiotics, cessation of oral intake, basal maintenance fluids, and optimized analgesia.

Figure 1 : Abdominal X-ray with no evident abnormalities 

Although hemodynamically stable, the patient continued to report intermittent abdominal pain. On physical examination, the abdomen was tense, with no rebound tenderness. Given these findings, a follow-up upright abdominal X-ray was ordered (figures 2 and 3), as the initial supine bedside X-ray showed no significant abnormalities.

Figure 2 : The plain film suggests the presence of a sigmoid volvulus 

Figure 3 : The plain film suggests the presence of a sigmoid volvulus 

The follow-up imaging revealed the “coffee bean” sign, leading to a sigmoid volvulus diagnosis. Surgical intervention was deemed necessary, and an exploratory laparotomy was performed. This confirmed the presence of a volvulus with three complete twists, accompanied by necrosis of the entire sigmoid colon, though without perforation (figure 4). The volvulus was successfully untwisted, and the necrotic sigmoid segment was resected. The distal rectal stump was amputated, and a Brooke colostomy was created. The procedure was completed without complications.

Figure 4 : Twisted sigmoid colon with segmental necrosis 

Following surgery, the patient required brief vasoactive support with norepinephrine and continued intravenous antibiotics. The clinical course was favorable, with the patient progressing to clinical stabilization and improvement. Upon discharge, the patient was in good general condition, afebrile, tolerating oral intake well, with a soft, non-tender abdomen on palpation and normal bowel sounds.

DISCUSSION

Sigmoid volvulus is a rare but significant condition that can lead to acute intestinal obstruction. While commonly seen in adults, particularly those with risk factors such as chronic constipation or advanced age, its occurrence in children is infrequent. Since 1940, fewer than 100 cases of sigmoid volvulus in pediatric patients have been reported in the literature, highlighting its unusual nature in this population. Among these, only three cases have been described in association with anorectal malformations, further underscoring the complexity and rarity of this clinical presentation⁹.

The clinical presentation typically includes abdominal pain, distension, and constipation, often associated with colonic transit disturbances, as seen in our case. Radiological imaging, such as the classic “coffee bean” sign observed in abdominal X-rays, is essential for early diagnosis, as demonstrated in this case^9,10 This finding aligns with the literature, which suggests that early identification of the sign can reduce the risk of severe complications, such as intestinal perforation^9,11.

The therapeutic approach to sigmoid volvulus depends on the extent of necrosis and the patient’s clinical status. Although endoscopic detorsion is considered the initial treatment option for uncomplicated cases, surgical intervention was indicated in this patient due to the presence of extensive sigmoid colon necrosis^11,12. Exploratory laparotomy was performed, successfully removing the affected segment and creating a Brooke colostomy13, which is the standard approach for cases with extensive necrosis. This decision was made based on the fact that a definitive colostomy provides better outcomes in cases of extensive necrosis, particularly when the viability of the colon cannot be safely restored¹⁴.

Postoperatively, the patient required brief vasopressor support for hemodynamic stabilization, which was effective and short-lived, as the overall clinical course was satisfactorily managed with antibiotics and appropriate post-surgical care. Although this case had a favorable outcome, it is important to note that complications such as sepsis or multi-organ failure may occur, especially in older patients or those with significant comorbidities^9,11.

The main clinical lesson from this case is the importance of rapid and accurate evaluation in patients presenting with signs and symptoms of acute intestinal obstruction, as early diagnosis can improve prognosis and reduce complications. Additionally, careful selection of the therapeutic approach, based on the severity of necrosis and the patient’s clinical condition, is crucial for treatment success.

CONCLUSION

Sigmoid volvulus should be considered in the differential diagnosis of pediatric patients presenting with acute abdominal obstruction, especially those with underlying conditions such as anorectal malformations. Early diagnosis and timely surgical intervention are crucial to prevent morbidity and mortality. This case adds to the limited literature on sigmoid volvulus in children and highlights the challenges in managing such rare and complex cases.