Successful management of intussusception with total polyp clearance in Peutz-Jeghers syndrome using a combined endoscopic and surgical approach

Abstract

Intussusception due to small intestinal polyps in Peutz-Jeghers syndrome represents a significant clinical challenge. Neither pure surgical nor endoscopic approaches alone are effective in the long-term management of this problem. We describe a combined approach using both surgery and small bowel endoscopy in the management of this condition, which resulted in both immediate and long-term success. Although not new, we believe this approach remains relevant despite recent technological advancements in this area.

BACKGROUND

Intussusception caused by recurrent large polyps in the small intestine represents a clinical challenge. A surgical solution alone is inappropriate as it will result in multiple laparotomies, complicated by adhesions and small bowel resection, leading eventually to short bowel syndrome. Conversely, a non-surgical approach alone, such as per-oral endoscopy, would not be able to deal with the mechanical problem of intussusception. Combining surgical laparotomy with endoscopic treatment has previously been described in a few case reports to successfully minimise the long-term complications of this problem.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterised by intestinal polyps in association with mucocutaneous melanocytic macules at lips, oral and gingival mucosae.¹ Gastrointestinal polyps occur in 88–100% of patients with PJS, with the higher frequency in the jejunum, ileum and large intestine. The polyps are mostly hamartomatous and are often multiple.² They lead to frequent complications such as intussusception, obstruction and bleeding.

We report on a case of small bowel intussusception associated with multiple small bowel polyps in a patient with PJS managed successfully using a combined surgical and endoscopic approach.

CASE PRESENTATION

A 37-year-old Malay female with a known history of PJS presented to the surgical unit of this institution with a sudden onset of right iliac fossa pain. There was no history of vomiting or abdominal distension. The patient had previously had a laparotomy with small bowel resection in 1996 for obstruction secondary to polyps. Examination revealed a tender mass in the right iliac fossa, but the rest of the abdomen was soft on palpation. The patient was not febrile and the total white cell count was 12.7×10⁹/L.

INVESTIGATIONS

A colonoscopy revealed several pedunculated polyps, which were excised by snare polypectomy, but no mass lesion was seen. A CT scan of the abdomen subsequently revealed a target lesion or a doughnut sign in between the loops of small bowel consistent with an intussusception (fig 1). Further lesions were seen within the lumen of the small bowel as well, which was suggestive of polyps.

Figure 1.

Abdominal CT scan demonstrating intestinal intussusception (white arrow).

TREATMENT

As laparotomy was indicated for the persistent abdominal pain and intussusception, we decided to perform an on-table enteroscopy to examine the entire small intestine. An ileo-ileal intussusception invaginated over a large 3 cm polyp was found at laparotomy (figs 2 and 3) and this was manually reduced. A therapeutic gastroscope (GIF 160, Olympus, Tokyo, Japan) was then inserted into the small intestine via an enterotomy incision around the same area. The entire small intestine proximal and distal to the insertion site was examined with manual assistance. A further seven large broad-based polyps were identified in the duodenum and ileum. These were all successfully resected using a electrosurgical polypectomy snare (Microvasive Boston Scientific, Massachusetts, USA) and retrieved with polyp retrievers (Olympus) (fig 4). The invaginated small bowel was finally resected and anastamosed to facilitate removal of the 3 cm broad-based polyp that acted as the lead point. Histopathological examination revealed that all polyps were hamarmatous in nature.

Figure 2.

Laparotomy showing ileo-ileal intussusception.

Figure 3.

Large 3 cm polyp acting as lead point for intussusception.

Figure 4.

Endoscope with one of the retrieved hamartomatous small intestinal polyp.

OUTCOME AND FOLLOW-UP

Post-operative recovery was uneventful with no evidence of ileus. The patient was discharged 7 days after surgery. She remained well and asymptomatic for 20 months post-surgery but was subsequently lost to follow-up.

DISCUSSION

Traditional surgical management of intussusception caused by polyps in PJS had been in the form of a manual reduction during laparotomy, followed by surgical polypectomy and segmental bowel resection.² Polyps were identified via on-table transillumination and external palpation. Multiple enterotomies were often required to excise and extract polyps in different parts of the small intestine. However, as on-table manual identification has been shown to be unreliable at detecting up to 40% of intestinal polyps, many patients would re-present with further complications of missed polyps. This would inevitably result in further laparotomies and bowel resections. The long-term consequence of this pure surgical approach would include multiple adhesions and even short-bowel syndrome.³

The development of smaller and more flexible endoscopes in the last two decades have revolutionised the management of PJS complications¹. Intraoperative enteroscopy with manual assistance has the advantage of examining the entire length of bowel.² The endoscope is inserted via an enterotomy, and the operating surgeon assists in guiding and pushing the bowel over the endoscope, ensuring that the bowel is not torn or the mesentery not overstretched resulting in bleeding or infarction.⁴ Polyps that are identified can be excised using a snare with standard electro-cautery and the entire small bowel can be cleared of polyps in one sitting. This technique has indeed proved successful, with several published series in the literature now.^5–8 The reduced need for repeated intestinal resection results in the avoidance of complications mentioned previously and patients have also been shown to remain symptom free for a long period of time.

Recent developments in endoscopic technology with the double-balloon enteroscope (DBE) may soon render the combined endoscopic and surgical approach obsolete. This latest endoscopic technique allows entire examination of the small intestine via a per-oral route obviating the need for surgical enterotomies and on-table enteroscopies.⁹ The working channel of the DBE further enables therapeutic polypectomy, although retrieval of multiple lesions may prove cumbersome.¹⁰ However, DBE alone is clearly inadequate in dealing with polyps and intussusception, which was the problem in our patient. A recent report of laparoscopic-assisted DBE demonstrated more effective clearance of polyps in three patients with small bowel adhesions.¹¹ This is a definite advantage in patients with PJS, as many, including our patient, would have had previous intestinal surgery with resultant adhesions. The use of this approach in cases like ours remains to be proven.

Several reports in the literature have described using a laparoscopic approach in the management of intussusception with PJS. Cunninghan et al reported on a single case whereby intussusception in a 15-year-old female was resected laparoscopically with a temporary successful outcome.¹² More recently, Zanoni et al successfully treated an intussusception and removed three of the largest polyps through several enterotomies using a pure laparoscopic approach.¹³ However, in both cases, complete clearance of small intestinal polyps was not performed and it is likely that recurrence of obstruction due to residual hamartomas would be high.

Laparotomy to reduce or resect invaginated small bowel, followed by assisted pan-enteroscopy and total polyp clearance remains the best approach in our opinion. Similar to our experience, successful combined endoscopic and surgical treatment for intestinal intussusception in PJS were previously described in singular cases by at least three other groups.^6,14,15

Complications of a combined laparotomy and on-table enteroscopy can include prolonged ileus from extensive bowel handling, stretching and gaseous distension. However, none of the cases reported, including our patient, had developed any of these problems. Clinicians should also be wary of the potential risk of diathermic perforation and reactionary haemorrhage from snare resection. We conclude that a combined surgical laparotomy followed by an enterotomy and endoscopic polypectomy remains the best modality of treatment in managing intussusception in PJS with multiple small intestinal polyps. Despite newer developments in minimally invasive surgery and therapeutic endoscopy, this combined approach remains relevant and effective in current clinical practice.

LEARNING POINTS

• Intussusception caused by large hamartomas in the small intestine requires urgent management.

• Surgery alone is ineffective as it will result in repeated operations and subsequent complications over a patients’ lifetime.

• Combined laparotomy and total small bowel endoscopy allows for the effective management of both the intussusception and clearance of all polyps in one sitting.