Uncommon Ileo-Cecal Intussusception in Adults: A Case Report of Diagnosis and Management

Abstract

Patient: Male, 36-year-old

Final Diagnosis: Idiopathic ileo-cecal intussusception • short bowel syndrome

Symptoms: Abdominal pain, diarrhea, vomiting

Clinical Procedure: —

Specialty: Critical Care Medicine • Gastroenterology and Hepatology • Surgery

Objective: Rare disease

Background

This report describes the case of a 36-year-old man presenting with severe abdominal pain and small-bowel obstruction due to idiopathic ileo-cecal intussusception. Intussusception refers to the telescoping or folding of a segment of bowel into the section distal to it. It is one of the main differentials of small-bowel obstruction in the pediatric population, but remains exceedingly rare in adults. Intussusception in adults is generally associated with organic masses of the digestive tract, especially tumors, which act as lead points that drag the proximal bowel into a more distal segment. It is extremely uncommon to find intussusception in an adult patient with no obvious underlying bowel pathology.

Case Report

We report a case of ileo-cecal intussusception in an adult patient that yielded no underlying bowel anomaly on surgical exploration and pathology. The patient, a 36-year-old man, presented with severe abdominal pain and was diagnosed with intussusception through computed tomography (CT) scanning. Surgical resection and anastomoses of the bowel were subsequently performed when medical therapy failed. The patient experienced diarrhea and vitamin-deficient neuropathy a month after surgery, which was diagnosed as short bowel syndrome, and which resolved with appropriate medical care.

Conclusions

Intussusception, while rare in adult patients, is possible even without underlying bowel disease. However, a thorough surgical exploration and proper pathology analysis are essential to confidently exclude other gut anomalies. Clinicians should be aware of possible complications like short bowel syndrome if the surgical approach is used and a significant amount of intestine is resected.

Introduction

Intestinal intussusception refers to the invagination or folding of a proximal segment of the bowel into a distal segment [1]. Typically, this involves the small intestine, although sometimes the large bowel may be involved [1]. It frequently results in small-bowel obstruction, and sometimes other emergency complications like strangulation, perforation, and peritonitis [1,2]. This condition is much more prevalent in children than adults, in whom it accounts for less than 5% of bowel obstructions [2,3]. In adults, more than 90% of cases are due to an anatomical lead point, most frequently a tumoral mass that drags the proximal segment into the distal one, causing the invagination [3–5].

Intussusception is not a common differential when evaluating abdominal pain in adults [5,6]. Unlike the acute picture often seen in pediatric cases, symptoms are generally a bit more chronic and intermittent in adults [5–7]. Among adult invaginations, truly idiopathic cases are even more rare; thorough investigation is required to eliminate not only obvious anatomical lesions like tumors, but also more subtle causes like Crohn’s or ulcerative colitis [6,7]. Repeat CT imaging and intestinal biopsy are often required to definitively classify an adult intussusception as having no true underlying cause [7].

Because of the rarity of this condition in adults and the paucity of pathognomonic signs, presentations are often misdiagnosed in favor of more common differentials like appendicitis, bowel obstruction, abdominal hernias, renal or biliary colic, torsion, and diverticular disease [8,9]. This is potentiated by the fact that many presentations of adult intussusception are opposed to the classic acute presentation seen in infants, with many having chronic abdominal pain [6] or simply diarrhea [8]. It is thus essential for clinicians to maintain a high index of suspicion for intussusception in all adults with both acute and chronic gastrointestinal concerns, request imaging as early as possible, and consider the diagnosis even after no organic lesions are found in the abdominal cavity. We describe the case of a 36-year-old man who presented with severe abdominal pain and small-bowel obstruction, who was subsequently found to have idiopathic ileo-cecal intussusception, and had transient short bowel syndrome a month after surgical resection of the terminal ileum and colon.

Case Report

Our patient was a 36-year-old man who presented with 72 hours of severe abdominal pain, predominating in the right iliac fossa and exacerbated in the 3 hours prior to arrival. He had experienced several episodes of lower right-quadrant abdominal pain prior to this, with the last one being a month prior to presentation; but none of the previous episodes had been this severe, and they had all resolved with over-the-counter antispasmodics. He had no other relevant medical, surgical, drug, or family history.

The pain during this episode was colicky, non-radiating, and associated with 2 episodes of vomiting greenish material and 3 episodes of mucoid, bloody diarrhea, for which he had received antiparasitic agents on the first day of onset.

Physical examination revealed a sausage-like mass extending from the right hypochondrium into the epigastrium, with positive Dance’s sign – a retracted right iliac fossa with bulging in the right hypochondrium. There was generalized tenderness, guarding, and rebound tenderness, but the abdomen was not distended. A digital rectal examination was remarkable only for slightly bloody, brown, liquid stools. Initial vitals were: blood pressure 134/83 mmHg, pulse 77 beats per minute, temperature 37.2°C, and oxygen saturation 98% on room air.

Laboratory results from initial blood tests were unremarkable, except for a slightly accelerated erythrocyte sedimentation rate (ESR) (Table 1), which made infectious diarrhea and appendicitis unlikely. Abdominal CT scanning was performed the same day the patient presented to the hospital, as the differentials at this point were volvulus causing obstruction, and an intestinal tumor or intussusception, which would not be clearly visualized with ultrasound or plain films. The CT revealed severe gastric distension (Figure 1) and an undifferentiated mass in the right iliac fossa (Figure 2).

Table 1.

Laboratory results from initial blood tests.

Laboratory test	Value	Normal range
Hemoglobin, g/dL	13.1	13–17
Hematocrit, %	42.6	40–50
White blood cells, 103/mcL	8.6	4–10
Neutrophils, %	68.4	50–70
Platelets, 103/mcL	229	150–350
Na, mmol/L	138	135–145
K, mmol/L	3.71	3.5–5
Cl, mmol/L	100	98–107
Creatinine, mg/dL	0.89	0.7–1.2
Urea, mg/dl	27	17–48
SGOT, UI/L	26	<40
SGPT, UI/L	13	<40
CRP	3.15	<5
ESR	17	<10

Figure 1.

Gastric distension (arrow key) indicative of severe bowel obstruction.

Figure 2.

Sausage-shaped right iliac fossa mass (arrow) representing the intussuscepted bowel.

Medical treatment was attempted with a nasogastric tube for decompression and adequate fluid and electrolyte resuscitation. The tube yielded about 600 ml of greenish secretions in the first 24 hours. The patient was admitted and kept NPO (nothing by mouth) while receiving intravenous antibiotics and analgesia. Symptoms persisted in the first 24 hours, with pain worsening and the abdomen beginning to distend. The patient was transferred to the operating room after failure of medical therapy.

Given the worsening pain and failure of medical decompression, a median laparotomy was performed under general anesthesia. Upon exploration, there was moderate ascites, several inflamed mesenteric lymph nodes, and a large ileo-cecal invagination with ongoing necrosis of the ascending colon. Reduction of the intussusception was impossible. Ileo-cecal resection was performed, with removal of about 60 cm of the ileum as well as the entire ascending colon. An isoperistaltic, latero-lateral anastomosis between the terminal ileum and the transverse colon was done and sutured with absorbable thread. The breach in the mesocolon was next repaired, then the entire abdominal cavity was thoroughly explored for other lesions and a possible lead point, but the rest of the abdomen was found to be normal.

The patient remained stable postoperatively, with only mild pain even on the first day after surgery; there was 200 cc of serous fluid in the drain and 165 cc of greenish fluid drained by the nasogastric tube. Flatus was passed on the third day and the nasogastric tube was removed. A soft liquid diet was started on the fourth day and was well tolerated. There was fever on the fifth day, which was subsequently found to be linked to infusion phlebitis on his left forearm. As he was already adequately tolerating oral intake, he was switched to oral pain medication, fluids, and antibiotics, and alcohol dressings were started. The fever resolved by day 7, and the drain was removed. He was discharged on the eighth postoperative day.

The entire surgical specimen was sent to pathology. Macroscopy confirmed an invagination of 20 cm of the terminal ileum into the cecum, with infarcted, necrotic bowel walls in both the ileum and colon. The mucosa of both the ileum and colon were normal, with extensive hemorrhage in the submucosa and edema of the chorion and muscular layers. The appendix and ileal submucosa were congested, with a polymorphic inflammatory infiltrate. The lymph nodes were hyperplasic with sinus edema. This confirmed that there was no underlying bowel disease prior to the invagination, confirming this as a case of idiopathic adult intussusception.

The patient remained symptom-free after discharge and was seen twice in the surgery clinic after discharge, with no acute concerns during either visit. However, a month after the surgery, he presented for his follow-up visit with a 1-week history of crampy abdominal pain, foamy diarrhea, and paresthesia of the abdomen and lower limbs. Examination revealed moderate dehydration and peripheral sensory neuropathy (hyperesthesia and dysesthesia) of the lower abdomen and lower limbs. He was referred to the gastroenterologist. The Gastroenterology team diagnosed him with short bowel syndrome following ileal resection, causing the diarrhea and vitamin-deficient peripheral neuropathy. His symptoms resolved with appropriate medical therapy (oral loperamide, hydration, and vitamin supplements) and diet modification after 1 month of treatment. He has since been followed up in the Gastroenterology Department and has remained symptom-free, with no further episodes of recurrent abdominal pain, diarrhea, or signs of malabsorption.

Discussion

This report confirms that healthy adults with no underlying bowel diseases can present with intussusception. Unlike with pediatric invaginations, where most cases are idiopathic, adult intussusceptions are almost always caused by an organic lesion of the bowel, called a lead point [1–5]. A large percentage of these lesions are malignant, with about 66% of colonic intussusceptions and about 30% of enteric invaginations linked to malignancy [10,11]. Primary lesions tend to predominate in the colon and in older patients, while metastases tend to be more frequent in younger patients with small-bowel lesions [11]. Our patient did present with an ileo-cecal invagination, suggesting possible small-bowel disease; however, his ileum was found to be normal on pathology. A small percentage of adult intussusceptions (5–16%) can be idiopathic, although causes like pancreatic disease, inflammatory bowel disease, and extrinsic modifiers of gut motility like cannabis use, chronic opioid use, and genetic syndromes like cystic fibrosis have been described as potential causes of what would seem at first glance to be idiopathic intussusception [8–12]. However, after careful history-taking, a normal surgical exploration and normal bowel pathology, this patient was a true case of idiopathic intussusception in an adult who had been previously fit and well with no known gastroenterological or systemic disease.

Adult intussusception is usually very different from the classic pediatric triad of abdominal pain, currant-jelly stool, and a palpable abdominal mass [2–7]. Unlike the acute presentation in children, the literature frequently describes adult invaginations with chronic, intermittent abdominal pain and non-specific signs like nausea, gastrointestinal bleeding, and constipation [11,12]. Rarely, the classic sausage-shaped abdominal mass is found on physical examination [13], and the patient might present acutely with bowel obstruction [14,15]. However, abdominal pain is by far the most common symptom, found in more than 90% of cases [14]. Our patient presented acutely with abdominal pain and bloody diarrhea, and there were signs of peritonism and a sausage-shaped mass on physical exam.

Though intussusception is generally diagnosed preoperatively, the diagnosis is sometimes uncertain until intraoperative assessment [15]. For the preoperative diagnosis, laboratory investigations are generally normal, unless linked to complications like bowel ischemia or peritonitis [16]. The criterion standard for preoperative diagnosis of invagination is abdominal CT, although other imaging methods have been used with varying efficacy: ultrasound, plain radiographs, and barium enemas [2,3,17]. The classical pathognomonic sign of a heterogenous target lesion, also called Dance’s sign, consisting of an outer intussuscipiens and a central intussusceptum, is frequently seen with CT, but can also be observed with ultrasound and plain radiographs, especially in children [3,17]. Other radiological findings include those linked to bowel ischemia or other complications like edema, mural thickening, and bowel dilatation [3,18]. These are most readily seen with CT, which is why it is the preferred preoperative diagnostic modality, especially in adults [16–18]. In this case, bowel obstruction and a right iliac fossa pathology were evident from clinical examination. The patient was thus sent directly for CT, as ultrasound and plain films are generally unhelpful in visualizing bowel masses. The diagnosis of intussusception was thus confirmed preoperatively in this patient with CT scanning of the abdomen.

Unlike pediatric invaginations, adult intussusception is rarely managed medically, owing to the increased prevalence of underlying bowel abnormalities that tend to make spontaneous reduction unlikely [12]. Air and barium enemas are rarely successful for similar reasons, although some reports advocate for the attempt, especially when there is a concern for surgical complications like short-gut syndrome [7,19]. There is a large body of evidence against attempted reduction in adults, however, due to risks of perforation, systemic seeding of gut flora or tumor cells, and increased surgical complications of an edematous manipulated bowel [18,20]. As our patient already had signs of peritonism and bowel ischemia, preoperative reduction was not attempted.

With regards to operative management, laparoscopic reduction is feasible in adults with both small- [21,22] and large-bowel obstructions [22,23]. Laparotomy is the preferred approach when there is significant distension or when multiple complications are present [20]. Ileo-cecal and colocolic intussusception in adult patients is highly suggestive of malignancy, and most studies recommend a colon cancer resection regardless of pathology [7–12]. Given that our patient had significant bowel distension, signs of ischemia, and peritonism, a laparotomic colon cancer resection was performed, as this would permit better visualization of the cavity, wider resection margins, and better peritoneal lavage.

Resections of large sections of the small intestine can cause many short- and long-term complications, of which short-gut syndrome is potentially one of the most serious [12,24]. Short-gut syndrome is a rare malabsorption disorder caused by insufficient functional bowel. It commonly presents with diarrhea, fatigue, weight loss, and signs of malabsorption (anemia, spasms or vitamin-deficient neuropathy) [24,25]. It can be congenital or acquired, with nearly all acquired cases linked to surgical resection of the small intestine [24]. Studies report that it generally occurs when there is less than 2 m (6.6 ft) of working small bowel, where it is likely to progress to intestinal failure [25]. However, even longer functional intestinal lengths may be associated with short bowel syndrome, but it is usually transient and resolves with adequate medical therapy as the remaining bowel adapts [25]. Our patient presented with subacute transient diarrhea and micronutrient deficiency that was diagnosed as short bowel syndrome and resolved in a few weeks with loperamide and vitamin supplementation, and has been followed up in Gastroenterology with no further complications since the surgery.

Adult idiopathic intussusceptions can present in a variety of ways. Acute presentations with obstruction in otherwise young, healthy adults as described in this report have been previously documented [5], but more often symptoms are intermittent and chronic [7], and found in adults with some co-morbidities [6]. Plain films and abdominal ultrasounds can help to eliminate differentials, but definitive preoperative diagnosis is invariably by CT [5–7], as was done in this case. Surgical management is generally the mainstay of treatment [5,7] as in our case, although medical management was tried in a case where co-morbidities made surgery too risky, and worked [6]. Prognosis after management is generally excellent, as the main indicator of poor outcome in adult invaginations is usually the lead point rather than the intussusception itself [5]. Our patient’s good outcome was similar to the other cases of idiopathic ileo-cecal intussusception described in the literature [5–7].

Conclusions

Although intussusceptions are rare in adults, it is important to include them in the differentials of acute abdomen and bowel obstruction, especially when physical examination points towards it, as in this case. Symptoms can be non-specific. Abdominal CT remains the best diagnostic tool preoperatively, but abdominal ultrasound and plain abdominal films can be useful to exclude differentials. Adult intussusception is rarely managed medically, and surgery is eventually required in nearly all cases. Most occurrences in adults are linked to organic lead points like malignancies or systemic disease, yet some cases are ultimately found to be idiopathic, as was this case. However, it is extremely important to thoroughly examine the abdominal cavity during surgery and send specimens for pathology review, to be certain no underlying anomaly exists. Further investigations into anything that could potentially impair bowel motility like recreational drug use, chronic opioid consumption, or genetic disease are important considerations in seemingly idiopathic intussusception. If a large segment of bowel is removed during surgery, it is important to be aware of possible long-term complications like short bowel syndrome. Follow-up of patients after surgery in both the short and long term is essential to make these diagnoses in a timely manner.