Acute ileo-ileal intussusception following a Swenson procedure in a resource-limited setting: a case report

Abstract

Introduction and importance:

Unexpected postoperative complications associated with the Swenson procedure for the surgical management of Hirschsprung’s disease are rarely documented, and postoperative intussusception following this technique is seldom reported. We present a case of acute ileo-ileal intussusception occurring after the Swenson procedure.

Case presentation:

The patient was a 12-month-old infant who, 16 days after undergoing a Swenson resection, presented with rectal bleeding and abdominal distension. A plain abdominal X-ray demonstrated features of intestinal obstruction, while a longitudinal ultrasound scan revealed a “sandwich” sign, leading to a suspected diagnosis of intussusception. A laparotomy was subsequently performed; intraoperative findings of ileal necrosis necessitated ileal resection and anastomosis. The patient’s postoperative course was favorable, and he was discharged on the seventh day.

Clinical discussion:

This unusual case suggests the existence of a functional mechanism, even in the absence of an identified anatomical cause. According to Swenson, post-surgical intestinal intussusception is uncommon but should be taken into account when making a differential diagnosis for any mechanical intestinal blockage that develops following surgery.

Conclusion:

Any child who has undergone a Swenson procedure and subsequently presents with rectal bleeding and other signs of intestinal obstruction should undergo advanced diagnostic evaluation to exclude intussusception. The necessity of establishing a postoperative follow-up protocol in resource-limited settings is evident.

Introduction

Postoperative intussusception is a rare complication, especially following surgical procedures used to treat Hirschsprung’s disease^[1,2^]. However, it can occur following various types of pediatric surgery^[2^]. The reported incidence among pediatric patients undergoing laparotomy ranges from 0.01% to 0.25%^[3–5^].

Hirschsprung’s disease is a congenital condition characterized by aganglionosis affecting a segment of the colon, with proximal dilation manifesting as congenital megacolon. Several surgical techniques have been developed for its management; however, the Swenson procedure remains one of the gold standards in the surgical treatment of this condition^[6,7^]. However, this technique is associated with a range of postoperative complications, including pelvic abscesses, fistulas, pelvic nerve damage, stricture of the coloanal anastomosis leading to constipation, necrotizing ulcerative enterocolitis, fecal incontinence, and sphincter dysfunction^[8^].

HIGHLIGHTS

• Rigorous and prolonged monitoring following surgery for Hirschsprung’s disease, extending beyond the first postoperative week, is essential.

• An expanded differential diagnosis should be considered in the presence of obstructive symptoms, ensuring that intussusception is not overlooked despite its rarity.

• Urgent abdominal imaging, particularly ultrasound interpreted alongside clinical symptoms in resource-limited settings, is recommended to rapidly identify intussusception.

• Early surgical intervention can improve prognosis.

Currently, there is limited literature from resource-limited settings documenting acute postoperative intussusception as a rare complication of the Swenson surgical lowering technique^[9,10^].

This technique, developed by Swenson, involves excising the aganglionic segment and restoring intestinal continuity and is commonly used in the treatment of Hirschsprung’s disease, a congenital intestinal aganglionosis that frequently leads to neonatal intestinal obstruction^[9,11,12^]. We describe a rare case of acute intussusception occurring after a Swenson procedure, with the aim of discussing potential underlying mechanisms and providing healthcare professionals with better insight into this unusual complication.

This study has been reported in line with the revised Surgical Case Report (SCARE) statement^[13^].

Case presentation

A 12-month-old boy, who had been suffering from abdominal distension and constipation since birth, was referred to us. The child underwent a Swenson procedure after a barium enema revealed a funnel-shaped appearance (Fig. 1); an intraoperative biopsy and histopathological analysis confirmed the diagnosis of Hirschsprung’s disease. The initial postoperative period proceeded smoothly.

Figure 1.

(A) A barium enema reveals a funnel-shaped appearance (yellow arrow) in Hirschsprung’s disease, indicating a narrowed, aganglionic segment, an intermediate zone with a few ganglia, and a healthy segment in a patient who underwent a Swenson procedure. (B) Image showing an air-fluid level identified on an unenhanced abdominal X-ray obtained for suspected acute postoperative intussusception.

His medical history indicates that he had previously suffered from tonsillitis, which was treated by the pediatric team; however, the etiology was unknown, and it was reported that a traditional practitioner had examined his throat before his consultation for Hirschsprung’s disease.

There is no reported family history of genetic disorders. He is the eldest of his siblings, with a father employed as a teacher and a mother employed as a farmer. No significant social history was identified. The supplementary medical history notes a respiratory condition characterized by rhinorrhea.

On the sixteenth postoperative day following Swenson’s procedure, the patient presented with acute abdominal distension, bilious vomiting, persistent crying, and rectal bleeding. Clinical examination revealed a deterioration in the patient’s general condition, marked abdominal distension, periumbilical tenderness, and visible peristaltic waves. A digital rectal examination demonstrated an empty rectal ampulla, with blood noted on the examining finger.

Intestinal intussusception was suspected. To exclude volvulus, an emergency abdominal ultrasound was performed, revealing a “target” sign. An abdominal X-ray without prior preparation (ASP) demonstrated air-fluid levels consistent with intestinal obstruction (Fig. 1). The complete blood count was within normal limits; the patient was blood type B, Rh-positive; and bleeding and clotting times were normal. Based on these clinical and laboratory findings, a water-soluble enema was not performed. Instead, an exploratory laparotomy was undertaken by the pediatric surgeon, assisted by two surgical residents.

Intraoperative findings revealed extensive necrosis of the ileal loop and an ileo-ileal intussusception measuring approximately 12 cm in length (Fig. 2). Manual reduction was not feasible due to ileal necrosis and failure of the loop patency test. Consequently, approximately 12 cm of the ileum was resected, and a terminal-to-terminal ileo-ileal anastomosis was performed. To rule out skip-segment Hirschsprung disease or other causes, the excised invaginated part of the ileum was sent to a laboratory.

Figure 2.

(A) Intraoperative finding of acute intussusception occurring 7 days after a Swenson procedure to lower the healthy colon, performed to treat Hirschsprung’s disease. (B) Resection of the intussuscepted ileum, which was sent for histological examination to rule out ultra-long or skip-segment forms of Hirschsprung’s disease.

No anatomical abnormalities such as Meckel’s diverticulum, mesenteric lymphadenopathy, or a tumor were identified. The abdominal cavity was closed in a layered fashion. Postoperative management included dietary restriction until the passage of flatus resumed and parenteral nutrition consisting of a 10% glucose solution administered at 20 mL/kg, alternating with Ringer’s lactate and 0.9% saline.

By the second day following the second procedure, bowel function and passage of gas had been restored, and the child was discharged from the hospital on the seventh day; the parents reported being satisfied with the care they received.

Discussion

This case report presents, for the first time, a rarely documented postoperative complication in a resource-constrained environment following lymph node dissection and direct coloanal anastomosis using the surgical technique outlined by Swenson^[1^] for Hirschsprung’s disease, with the mid-term postoperative period characterized by acute postoperative intussusception.

This postoperative complication following a Swenson procedure is rarely reported in the current literature^[5,14^]. Its postoperative incidence for conditions other than Hirschsprung’s disease can reach 5–15% of all postoperative intestinal obstructions^[5,14^], requiring early diagnosis and prompt emergency surgery^[3,15^] to establish a favorable prognosis, as we observed in our patient.

During repeat surgery, no macroscopic aganglionosis was found, so an intraoperative biopsy was omitted, given local resource limits. Prior histology lacked a clear aganglionic origin, suggesting aganglionosis as a factor in intussusception^[5,6,16^]. This complication is atypical in Hirschsprung’s disease, making the case unusual and noteworthy for the discussion of etiology.

Multiple explanations may elucidate the intussusception in this instance, with the most plausible explanation being issues associated with residual motility; even after excision of the aganglionic segment of the intestine, the normally innervated section may still demonstrate motor dysfunction^[6,16,17^]. This can cause dyskinesia in certain areas and disrupt the coordination of contractions^[18^], eventually causing the upper intestine to collapse into the lower intestine.

Additionally, since several rare variants of Hirschsprung’s disease exist, the skip-segment form must be considered in the differential diagnosis, particularly in the context of intestinal intussusception following a Swenson procedure^[19,20^]. This is especially relevant given that histological examination confirmed the presence of healthy colonic tissue during the procedure, although tissue samples were not obtained from all loops.

We think that a fixed surgical anastomosis could result in motility issues or form the “head” of an intussusception^[21^] or that deep dissection in the pelvis during the abdominal phase of a Swenson procedure could damage the nerve plexuses responsible for rectal motility and continence disorders^[17,21^]. However, in our patient, neither the urodynamic study nor the clinical examination indicated pelvic nerve damage because the clinical presentation was dominated by signs of intussusception, because no anorectal manometry or electromyography (EMG) of the anal sphincter had been performed, and because there were insufficient resources to confirm pelvic nerve lesions, as documented in the literature^[6,21,22^].

The literature shows that undiagnosed intussusception causes necrosis and perforation^[23,24^]. Early diagnosis ensured a favorable prognosis; rectal bleeding and the ultrasound “target” sign were essential diagnostic tools in this case. This case suggests that, in clinical practice, it is necessary to establish a protocol for clinical and ultrasound follow-up in resource-limited settings for children who have undergone surgery for Hirschsprung’s disease to prevent complications that may arise regardless of the surgical technique used or the clinical presentation of Hirschsprung’s disease encountered.

Surgical options for Hirschsprung’s disease include the Swenson procedure (direct coloanal anastomosis after resection), the Soave procedure (colon lowered into a rectal muscular sleeve), and the Duhamel procedure (retro-rectal anastomosis)^[6,7^]. Modern approaches encompass transanal variants, laparoscopic and hybrid techniques, and, experimentally, robot-assisted surgery. All aim to excise the aganglionic segment and restore bowel continuity, although complications vary^[7,22,23^]. In this case, the Swenson procedure was chosen based on available resources, while ileal resection addressed the severity of necrosis.

The strength of this study lies in its documentation of a serious complication rarely observed following the lowering of a healthy colonic segment during the Swenson procedure in children with Hirschsprung’s disease in a resource-limited setting. Its principal limitation is that it describes a single case of a rare occurrence; therefore, the conclusions cannot be generalized. The implementation of a structured follow-up protocol tailored to children in resource-limited settings may enhance the long-term outcomes of those who undergo surgery for Hirschsprung’s disease.

This example has instructional significance because it highlights the need to expand the range of postoperative problems associated with surgery for Hirschsprung’s disease. Intussusception is uncommon, but it should be taken into consideration in any instance of intestinal blockage in a child who has had Swenson surgery, regardless of how much time has passed.

Because this case report is unique, it has limitations, as the results cannot be replicated. Nevertheless, it emphasizes the importance of conducting a long-term follow-up study of patients who have undergone surgery for Hirschsprung’s disease in resource-limited settings to lower the incidence of medium- and long-term postoperative complications.

Conclusion

According to Swenson, postoperative intussusception is an uncommon but dangerous surgical complication that is rarely documented after Hirschsprung’s disease surgery. The need for careful clinical surveillance and timely action is highlighted by this example. This further emphasizes that the diagnosis should not be ruled out in the absence of an anatomical entry point.

This case suggests that any child who has undergone a Swenson procedure and exhibits rectal bleeding and intestinal obstruction should be evaluated in clinical practice to rule out intussusception. To maximize clinical outcomes, early detection and management are crucial, as demonstrated by the case discussed below.

Ethical approval

Not applicable.

Consent

Written informed consent was obtained from the patient’s parents/legal guardian for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

This work did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contributions

Conception: M.B.D. and A.M.; Design: M.B.D. and M.M.J.; Administrative support: A.M.D.; Literature search: H.B. and J.F.; Manuscript preparation: M.B.D.; Manuscript editing: M.B.D., B.G., and A.M.; Manuscript review: all authors; Supervision: A.M.; Final approval of the manuscript: all authors.

Conflicts of interest disclosure

The authors declare that they have no conflict of interest regarding the publication of this case report.

Research registration unique identifying number (UIN)

Not applicable.

Guarantor

Matabishi Bandeke Destin.

Provenance and peer review

This article was not commissioned and was peer reviewed.

Data availability statement

Not applicable.