Rare etiology of colonic intussusception involving an adult with emphysematous cystic enteropathy: A case report and review of literature

Abstract

BACKGROUND

Pneumatosis cystoides intestinalis (PCI), characterized by a collection of gas-filled cysts in the intestinal wall, is an uncommon but well-known condition in gastroenterology. Abdominal pain is the most frequent symptom associated with PCI. Intussusception represents a potential cause of recurrent abdominal pain or emergency presentation. However, the occurrence of colonic intussusception secondary to PCI is very unusual in adulthood.

CASE SUMMARY

A 52-year-old male, known with idiopathic PCI, presented seventeen months after initial diagnosis with a new right upper quadrant pain. A computed tomography-scan demonstrated a colonic intussusception at the hepatic flexure. PCI did not progress compared with initial investigation. The patient underwent an emergency right hemicolectomy.

CONCLUSION

Resection was recommended in this case because PCI proved to be persisting with no identified curable cause. Surgery allowed to address the underlying pathology, the potential relapse of intussusception, and the likely cause of recurrent abdominal pain, either invagination or PCI itself.

Key Words: Pneumatosis cystoides intestinalis, Colonic intussusception, Colonic obstruction, Colonoscopy, Case report

Core Tip: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by a collection of gas-filled cysts in the intestinal wall with various possible causes, such as constipation. Intussusception secondary to PCI in adult population has been rarely reported before. PCI may be a cause of intussusception similar to any tumor acting as a leading point. However, in the particular situation of intussusception caused by PCI, resection should be recommended to address this possible recurrence of abdominal pain, specifically if the underlying etiology of PCI is deemed irreversible.

INTRODUCTION

Pneumatosis cystoides intestinalis (PCI) refers specifically to the accumulation of gas in the submucosa and subserosa forming diffuse and extensive intraluminal cysts[1,2]. Even though it is not common, PCI is a known entity in gastroenterology, affecting 0.03% of the population[3]. There is a 3:1 male predominance with an age range between 30 and 50[2]. The number of reported cases of this condition is expected to increase because of the wider use of better diagnostic modalities such as computed tomography (CT) scan and colonoscopy[4]. It can occur anywhere throughout the gastrointestinal tract[1] with the colon being the most frequently affected site[1,2,5]. The descending colon is the most commonly involved part of the large bowel[2,3,6]. PCI is a benign condition that is usually managed conservatively[1,5]. Complications may, however, occur in 16.3% of cases, including obstruction and perforation[2].

Etiology and pathogenesis of PCI remain unclear[1,5,6]. In adults, PCI is usually benign and found incidentally on imaging or endoscopy[3]. Approximately 15% of cases are primary, and 85% are secondary[1,3,5]. Inflammation, physical damage of intestinal mucosa, nutritional imbalance, dysbacteriosis, gastrointestinal dysmotility, and immune dysfunction have been proposed as the underlying pathophysiological mechanisms[1]. Various predisposing factors have been associated with PCI: Abdominal surgery, pulmonary disease, scleroderma, diabetes, constipation, malnutrition, chemotherapy, and toxic exposure[1,5].

In adult population, colonic intussusception remains rare[7-10] and represents only 1% of intestinal obstruction[7,11,12]. Intussusceptions in adults generally have a well-defined pathological lead point, which is malignant in about 60% of cases[8]. Any benign lesion may, however, serve as the lead point causing the intussusception[8,10-12]. The occurrence of colonic intussusception secondary to PCI is very unusual in the adult population[2].

We present a case of right colonic intussusception, secondary to the known PCI of the ascending colon. Following a review of the literature, we discuss the management of this case and similar ones.

CASE PRESENTATION

Chief complaints

A 52-year-old male was brought to the emergency room by his mother with a 2 wk history of abdominal pain and bloating.

History of present illness

The patient presented with crampy pain on the right side of the abdomen. He reported bloating but no vomiting. He was constipated more than usual. However, he was still passing some gas. He had no rectal bleeding. He denied having fever, shortness of breath or thoracic pain.

History of past illness

The patient was seen seventeen months earlier for gastroenterology consultation because of chronic constipation, which was mild but lasting for few years with stools varying from 2 to 3 times per week. He denied having abdominal pain at this time. Because the patient was over 50, he underwent investigation to rule out a mechanical cause of constipation. He was diagnosed with intestinal pneumatosis by abdominal CT scan (Figure 1) and endoscopy (Figure 2). There was no evidence of other colonic disease or neoplasia. The patient was prescribed polyethylene glycol (PEG 3350) once to twice daily. No further investigation or treatment was planned.

Figure 1  Coronal reconstruction of an enhanced abdominal computed tomography with lung window showing multiple air-filled cysts along the intestinal wall in the ascending colon (box).

Figure 2 Colonoscopy showing numerous soft polypoid masses with normal overlying mucosa located in the ascending colon. A: Caecum; B: Ascending colon.

Personal and family history

This 52-year-old patient had a congenital mild cerebral palsy with mild mental retardation. He was living with his parents. He was active and a manual worker, with no mobility problems. He was also known to have dyslipidemia, hypertension, diabetes, and atrial fibrillation. His medication consisted of warfarin, pantoprazole, spironolactone, sitagliptin, atorvastatin, perindopril, and bisoprolol. He had no history of smoking or alcohol consumption. He was never operated. Family history was found not relevant to his clinical condition. He has an older brother who is in good health without intellectual deficit.

Physical examination

Upon admission, the patient was awake, well oriented and collaborative. He had right upper quadrant pain without defense or rebound tenderness. His vital signs were stable with blood pressure of 120/85 mmHg, heart rate of 90, and temperature of 36.5 °C.

Laboratory examinations

Hemoglobin level, white cell count, coagulation parameters, renal and liver function tests were all within the normal limits. C-reactive protein was 25 mg/L (Normal: 0.0-5.0 mg/L).

Imaging examinations

An enhanced abdominal computed tomography showed a colocolic intussusception near the hepatic flexure over a short segment (Figure 3). The PCI was still present and showed the same right-side distribution in comparison with previous imaging (Figure 3). There was no evidence of perforation or small bowel distention.

Figure 3 Abdominal computed tomography at the time of presentation compared with an antecedent abdominal computed tomography. A: Abdominal computed tomography (CT) showing a colonic intussusception over a short segment at the level of the hepatic flexure of the colon. Mesenteric fat is entrapped between the colonic walls (arrow); B: Abdominal CT showing the normal continuity of the colon at the same level seventeen months earlier.

FINAL DIAGNOSIS

Right colonic intussusception secondary to PCI.

TREATMENT

The patient was observed after discontinuing and reverting the anticoagulation, considering that there was no evidence of perforation or bowel ischemia, and patient still passing flatus. The right upper quadrant pain persisted, although mildly, during the observation period. The patient remained stable and no intervention became necessary to address the intussusception itself. Since the patient had persisting abdominal pain, he was scheduled to the operating room 3 d after initial consultation. A median laparotomy was undertaken. At exploration, the intussuscepted segment of the distal ascending colon was identified. While manipulating the colon during surgery, the intussusception was already reduced. A right hemicolectomy, including the proximal transverse colon involved with PCI, was performed. The resected specimen was opened, and the presence of parietal cysts was revealed (Figure 4). The final pathologic examination confirmed the diagnosis of PCI.

Figure 4 Surgical specimen of right hemicolectomy. A: The resected specimen showing polypoid lesions with normal mucosa and cystic structures; B: A submucosal cyst was opened demonstrating a spongy consistency (box).

OUTCOME AND FOLLOW-UP

The postoperative period was uneventful. Normal diet was resumed after 3 d. The patient was discharged from the hospital after 6 d. After eight months, the patient was eating normally and resumed his usual activities. Constipation has much improved. No further colonic follow-up is planned.

IMAGING EXAMINATIONS

An enhanced abdominal computed tomography showed a colonic intussusception near the hepatic flexure over a short segment (Figure 3). The PCI was still present and showed the same right-side distribution in comparison with previous imaging (Figure 3). There was no evidence of perforation or small bowel distention.

DISCUSSION

We present a patient with PCI who developed an intussusception at the right hepatic flexure of the colon. The PCI was known for seventeen months before the intussusception. The PCI was attributed to constipation, which was chronic but mild. In this case with no other etiologic factors identified, PCI could have been considered as primary. As there was no identified cause and also the absence of symptoms, the patient was initially managed conservatively, as recommended[1].

Abdominal pain remains the most frequent symptom associated with PCI[2]. However, pain and bloating at presentation were new symptoms in this case. The patient had persisting abdominal pain during the days before intervention. Even if the pain could not be attributed entirely to the intussusception without any doubt, it remains the most probable cause for the new onset of symptoms, in our opinion. Because there was no change in the distribution and severity of the disease, we considered that the seventeen-month history of PCI, probably secondary to chronic constipation, and which never regressed, was irreversible.

The patient did not show any signs of deterioration, but had decreased pain and ongoing passage of flatus, while fasting. We thus chose to wait for reversal of anticoagulation before surgical intervention, however remaining ready to proceed earlier in case of worsening. We decided to proceed with a right hemicolectomy, which included the part of the colon involved with PCI, even if the underlying pathologic process was already known, benign, and potentially reversible. We did not proceed with further investigation since the cause of the colonic intussusception was already known and benign. We do not present a particular surgical approach laparoscopic hemicolectomy could have also been contemplated. Should the pain be attributable to the intussusception or PCI itself, the surgical approach addressed the underlying pathology and potential relapse of abdominal pain and/or invagination.

The situation, as in the present case, may be underreported, but it certainly remains a very rare occurrence. We retrieved only six adult cases of colonic intussusception associated with PCI (Table 1)[4,13-17]. No case had other lesions that could potentially serve as the lead point. Two thirds occurred in the ascending colon. None of the earlier reported cases involved the left colon, even though this is the most commonly affected part of the colon with PCI[6], probably due to the smaller lumen of this segment. Two thirds have been reduced either during enema exams[4,16,17] or colonoscopy[13]. In two cases, no surgery was carried out[4,16]. However, follow-up is either not mentioned or too short to draw conclusion about the necessity of surgery. Question deserves to be kept in mind, as to whether recurrence of abdominal pain in patients could be associated with episodes of intussusception, since pain is the most frequent symptom[2]. Constipation itself might be involved in colonic intussusception[18] but its contribution in this case remains difficult to assess. Because of the presence of a lesion in the colon, even benign, surgical intervention remained indicated. In the present case, the intussusception was spontaneously reduced at the time of surgical intervention, confirming the possible hypothesis that the new onset of pain was caused by the episodes of intussusception.

Table 1 Reported cases of colonic intussusception in adults associated with pneumatosis cystoides intestinalis.

Ref.	Age	Sex	Background	Site	Treatment	Remarks
Fujiya et al[4]	29	Male	Steven-Johnson syndrome, COPD with bronchiectasis	Right transverse	Reduction with gastrographin enema. No surgery	Follow-up not specified
Ahrar et al[6]	29	Male	None	Ascending	Reduction with barium enema. Deferred right hemicolectomy
Nagata et al[13]	23	Male	None	Ascending	Reduction with colonoscopy. Laparoscopy-assisted partial ascending colectomy one month later
Wang et al[14]	55	Male	Diabetes	Left transverse	Partial colectomy with stoma and mucous fistula. Subsequent reanastomosis
Itazaki et al[15]	20	Male	Glomerulonephritis. Corticosteroid therapy	Ascending	Right hemicolectomy
Stern et al[16]	32	Male	None	Ascending	Reduction with barium and air enema. No surgery	No recurrence at 2 months

COPD: Chronic obstructive pulmonary disease.

CONCLUSION

In conclusion, PCI is an uncommon condition that general surgeons should be aware of. It may present with intussusception, particularly on the right side. Once intussusception is diagnosed, we recommend resection, particularly in potentially irreversible and persisting PCI. This management allowed to address the underlying pathology, the potential relapse of intussusception, and a likely cause of recurrent abdominal pain, that may be either invagination or PCI itself.