Abstract
Congenital peritoneal encapsulation (CPE), a rare developmental anomaly in which the small intestines are enclosed within an accessory peritoneal sac, is an uncommon but important cause of abdominal pain and small bowel obstruction. With fewer than 60 reported cases, the demographics, imaging findings, and symptoms of CPE remain elusive, as most cases are diagnosed intraoperatively during treatment for bowel obstruction. We report a case of a 34-year-old male with no significant medical history who presented with acute abdominal pain and nausea. He was hemodynamically stable and afebrile, and CT imaging indicated a possible small bowel obstruction. Surgical intervention was required when his condition worsened, with large-volume emesis following oral contrast administration during a small bowel follow-through study. Explorative laparotomy revealed a peritoneal encapsulation of the entire small bowel as the cause of obstruction. Complete removal of the membrane was performed, and histopathology confirmed findings consistent with an intraperitoneal sac with reactive changes, supporting the diagnosis of CPE. This review highlights the challenges in diagnosing CPE due to its lack of associated comorbidities, minimal symptoms, and nonspecific imaging findings. A detailed discussion of 21 recent cases of CPE emphasizes patient demographics, presentation history, imaging, physical exam, intraoperative findings, and surgical management. This modern analysis underscores the importance of considering CPE in the differential diagnosis for unexplained abdominal pain or bowel obstruction, particularly when conventional diagnostic methods fail to identify a cause.
Introduction
Congenital peritoneal encapsulation (CPE) has sparked interest in the medical community as an exceedingly rare but significant cause of abdominal pain and small bowel obstruction. This congenital malformation involves the small intestine being enclosed, in varying lengths, within an accessory double-layered peritoneal sac. Embryologically, the malformation is explained as a defective return of the small bowel into the abdominal cavity in the 12th developmental week. Patients with CPE are often asymptomatic and are diagnosed only as an incidental finding. As such, less than 60 cases have been described in the literature, with an average presenting age of 40.8 years old and a 5:3 male predominance. 1 However, while the clinical presentation of small bowel obstruction secondary to CPE is rare, it remains an important potential cause of abdominal pain and small bowel obstruction due to the excellent prognosis it carries with appropriate identification and surgical treatment. 2
We report a 34-year-old male with no previous abdominal surgeries who presented to the emergency department with 3 days of abdominal pain, nausea, and vomiting. He reported some initial diarrhea that had subsequently stopped; he had not passed flatus or stool for 24 hours prior to arrival. His only past medical history included alcohol abuse. Review of his medical record noted that he had presented to the hospital twice the previous year with similar abdominal complaints. The patient’s abdomen was minimally distended and diffusely tender, with focal peritonitis in the right lower quadrant. He was hemodynamically stable. His laboratory evaluation on arrival demonstrated a mild leukocytosis with WBC of 10.4 10 × 3/μL and otherwise normal lab values with a lactic acid level of 0.7 mmol/L. CT imaging findings were suggestive of a bowel obstruction with concern for a closed loop obstruction. To better assess the degree of obstruction and to possibly provide therapeutic benefit, the patient underwent a modified small bowel follow-through per our hospital protocol, given that the patient was hemodynamically stable without peritoneal signs.
The patient, however, became diffusely peritonitic following the administration of oral contrast and had a large-volume emesis. Due to this acute clinical change, the decision was made to urgently take the patient to the operating room for an exploratory laparotomy. We opened the fascia in the midline and the underlying peritoneal layer; however, we were unable to palpate any loops of small bowel. We opened our laparotomy further to fully visualize the abdominal contents, and soon we identified a large intraperitoneal sac that completely encapsulated the small bowel (Image 1). There were 2 transition zones caused by fibrous bands that externally compressed the intestine at the ligament of Treitz and the other at the terminal ileum. We carefully resected the sac and released the adhesive bands. The small bowel immediately decompressed the intraluminal contents into the cecum. The mesentery was notably thickened and minimally mobile, making evisceration of the bowel difficult. However, the bowel was run twice with no enterotomies or serosal tears noted, and the abdomen was closed in standard fashion. Pathology revealed fibroadipose tissue with reactive fibrosis and mesothelial lining and vascular congestion, which were compatible with peritoneum. The patient recovered well and began having bowel function postoperative day (POD) 1. His diet was advanced, and he was discharged POD3. He remained asymptomatic since discharge and was doing well when he was seen in the clinic for his 2-week follow-up. Peritoneal encapsulation of the small bowel visualized intraoperatively. A notably constrictive band was found at the terminal ileum.
Summary of 21 Case Reports of Congenital Peritoneal Encapsulation (CPE) Published From 2019 to 2024. Some Boxes are Omitted due to Missing or Not Applicable Information.
Pathology
CPE is gaining attention as a rare but significant cause of bowel obstruction. It involves the encasement of the small bowel, in varying lengths, in a sack of peritoneum, due to the presence of an accessory peritoneal membrane between the omentum and mesocolon. 1 The cause of CPE remains poorly understood. The condition was first described in 1868 on study of a cadaver by Cleland, who described the membrane’s continuity with the ascending and descending colon laterally, transverse mesocolon superiorly, and the parietal peritoneum inferiorly. 3 The foundation of the most accepted theory is built on the identical histology of the accessory peritoneal sac with peritoneum. It is thought that the small bowel is anomalously coated with the yolk sac as it migrates back into the fetus, giving rise to an additional peritoneal membrane. 4 It is proposed that, during physiological herniation in the 12th week, sufficient traction occurs between the midgut’s peritoneal lining and the caudal duodenum that the peritoneal lining accompanies the small bowel into the abdomen, instead of remaining within the umbilical stump. 1 This anomalous return creates membrane openings around the duodenal-jejunal flexure and at the ileocecal junction. Our patient’s anatomy supports this postulation, as the accessory membrane was found to have transition zones in both areas.
The accessory peritoneal membrane morphologically and histologically imitates peritoneum. 1 Histology of the sac reveals fibroadipose tissue and mesothelial lining, which otherwise characterizes normal peritoneal tissue. This finding helps to differentiate from similar conditions, including abdominal cocoon and encapsulating peritoneal sclerosis (EPS). In contrast to CPE, these acquired encapsulating pathologies are histologically identified with dense fibrous connective tissue and chronic inflammatory cell infiltration. 5 Abdominal cocoon often occurs without an identifiable insult, whereas EPS involves more extensive fibrosis and calcification and is most often incited by peritoneal dialysis. 1
Clinical Presentation
The typical demographic of those diagnosed with CPE is a young male, supported by a mean presenting age of 40.8 (32-46) years old and a 5:3 male predominance. 1 However, Frerichs et al discovered CPE in a 6-day-old neonate, the youngest patient reported to date. 6 Most patients have noncontributory medical history and no previous abdominal surgeries. 1 CPE has been described to co-exist with familiar congenital anomalies, including incomplete situs inversus and epigastric hernia, 7 malrotation,6,8 and Meckel’s diverticulum. 9 These findings further support the embryological origin of CPE. Our patient did not have any congenital anomalies, nor did he have any pertinent medical history or prior abdominal surgeries.
Most cases of CPE are diagnosed incidentally, often when undergoing workup for another pathology or posthumously. 1 Those who become symptomatic commonly present with signs of intestinal obstruction, including colicky abdominal pain and constipation, which describe most patient presentations (Table 1). Symptoms can be acute, as described by the severe onset of abdominal pain reported by Chowdhury et al in a healthy 28-year-old male. 10 Patients who present acutely are often misdiagnosed. Both Davis et al and Tojal et al described patients who were recently seen at the hospital before their presenting complaint and were diagnosed with gastroparesis and idiopathic abdominal pain, respectively.8,11 Other reports have described a chronic history of intermittent symptoms, suggesting the role of CPE in longstanding constipation and abdominal pain.12–15 Our patient was seen 2 separate times in the year before his admission, the first being for vague epigastric pain for which he was diagnosed with viral gastroenteritis, and his second being diffuse abdominal pain with nausea and vomiting and idiopathic hypotension.
The diagnosis of CPE prior to surgery is challenging, as initial workup is unrevealing. Patients present with stable vitals and nonspecific laboratory values. Reported findings include mild leukocytosis,8,16–18 increased CRP, 8 and/or elevated lactate. 18 In 1 case, increasing lactate levels with an unclear diagnosis was the reason to undergo laparoscopic exploration. 16 In most cases, the abdominal findings on physical exam are nonspecific. Reported patients have presented with a variety of abdominal findings, with abdominal tenderness being the most common. This pain is typically in the periumbilical region but can also present in the left upper and lower quadrants, as described in the patients reported by Robbins et al and Toma et al.9,19 Less commonly, patients demonstrate signs of peritonitis. 1
There have been efforts to identify specific physical exam findings that may be indicative of CPE. Naraynsingh et al suggested 2 clinical signs to help preoperatively diagnose a patient with peritoneal encapsulation: (1) fixed asymmetrical distention of the abdomen on physical exam and/or (2) difference in consistency of the abdomen to palpation, with firmer areas signifying the presence of the dense accessory peritoneal sac. 4 Mohamed et al identified the former of these 2 findings and described the presence of an asymmetrical mass located at the epigastric and central abdominal regions, adding evidence for their suspicion of peritoneal encapsulation. 20 Our patient’s initial workup, however, was negative for any physical findings suggestive of CPE, and was only notable for diffuse abdominal tenderness with focal peritonitis in the right lower quadrant.
Imaging
CT imaging is regarded as the most sensitive tool to CPE. It is helpful to categorize imaging findings as direct, indirect, or normal. Direct findings display the small intestine wrapped in a serpentine-like pattern within an accessory membranous capsule. Mitrousias et al coined this finding as the “helix sign.”
21
Mohamed et al and Hajimirzai et al both identified this sign on preoperative CT imaging and included peritoneal encapsulation in their differential prior to surgery.13,20 In the case of our patient, CT imaging performed on 2 separate occasions prior to his presentation did not show a helix sign. Retrospective review of his axial CT imaging, however, showed an abnormal clustering of small bowel (Image 2). This finding resembles the “cauliflower sign,” characterized by serpentine dilatation of the small bowel loop in a fixed U-shaped cluster, encased by a visible thick membrane. The radiologic term was originally coined by Sieck et al on barium contrast study and first characterized on CT scan by Ibrallulah et al in 2016.22,23 It is often used as an adjunct of the diagnosis of the similar, yet different, conditions of encapsulating peritoneal sclerosis and abdominal cocoon.
23
Our patient’s CT finding is better termed “cauliflower-like,” as there was no visible evidence of a membrane surrounding the small bowel. Axial CT image demonstrated abnormal small bowel clustering, resembling the “cauliflower sign” with dilated, bunched loops. The accessory membrane was not seen, making the findings “cauliflower-like.”
Other radiology reports include comments concerning the localization of the small intestine to the left upper quadrant14,16,24,25 or right upper quadrant in the setting of malrotation. 8 The relationship of the peritoneal sac to surrounding structures is important to exclude other mimicking etiologies, such as a paraduodenal hernias. The sac containing the small intestines is anterior to mesenteric vasculature and can subsequently cause splaying of these vessels. 20 Sometimes, only the accessory sac is identified, but other pathologies are considered given the rarity of CPE. Abuzaina et al and Renko et al both suspected that the sac resembled an internal hernia.12,18 The radiologist in the case of Suseeharan et al suspected a transmesenteric internal hernia or abdominal cocoon, the latter of which was also included in the differential of Mohamed et al.20,24
The accessory membrane can also be missed radiographically. Five of the case reports commented that the accessory sac was identified retrospectively on imaging (Table 1). While not specific to CPE, indirect findings encompass anatomic deviations that suggest a congenital anomaly and can help point to a diagnosis of CPE. Davis et al reported that the duodenum was anterior to the superior mesenteric artery. 11 Giaracuni et al found that the transverse colon was ptosic and located in the pelvis, which resulted in an abnormal course of the middle colic vessels. 17 Frerichs et al argued that their patient’s anatomy was not explained by malrotation, given atypical positioning of the duodeno-jejunal junction and jejunum. 16
Most published imaging reports of CPE, however, report expected anatomy, but no accessory sac. This is especially true for those affected by CPE who experience recurrent episodes of vague abdominal pain, as imaging is often unremarkable. 1 In the absence of any anatomical variations, the most common imaging finding is a small bowel obstruction, a symptomatic sequela of CPE. Most reported CT findings are significant for dilated bowel loops, signifying obstruction (Table 1). CT imaging is considered as an adjunct in diagnosis, as it is more often unrevealing to CPE.
Diagnosis
CPE is definitively diagnosed intraoperatively upon the gross visualization of a translucent sac encapsulating the small intestines and confirmed by histology. This intraoperative discovery has led to reports of incidental CPE. Thomassen et al identified CPE when their patient was undergoing a laparoscopy for a perforated duodenal ulcer, while another patient was undergoing repair for diverticular colonic stenosis.17,26 More recently, incidental CPE has been found in 2 different patients in Syria and China undergoing surgical resection for metastatic colon cancer.25,27 Both authors noted that the cancerous infiltrative lesions, which were found in nearby structures, spared the small intestine, suggesting the protective role of the accessory membrane from metastasis. Other incidental reports include posthumous diagnoses found among autopsy findings, with the cause of death being unrelated to CPE, such as in the case of Cioffi et al. 28
Surgery
Surgical management involves the excision of the accessory sac and adhesiolysis of the sac’s fixation points and any identifiable bands. Excision of the sac should be undertaken in the setting of incidental intraoperative diagnosis, due to its predisposition to small bowel occlusion. 1 While removal of the sac has been traditionally performed via a laparotomy, there have been recent arguments for a laparoscopic approach to minimize postoperative complications. Giaracuni et al, Dey et al, and Del Giudice et al successfully removed the accessory sac via laparoscopy. The authors argued that the created pneumoperitoneum can aid in identification of the accessory sac and should not be a reason to convert to laparotomy.14,16,17
Given that the literature describes 3 successful laparoscopic resections, a surgeon with extensive laparoscopic or robotic experience could undertake a minimally invasive approach to successfully manage a patient affected by CPE. However, in our case, the decision to perform an open exploration was predicated on the fact that the patient developed peritonitis in the setting of a bowel obstruction. Giaracuni et al, who chose laparoscopic intervention, suggested that patients who present with bowel obstruction secondary to CPE may not be candidates for laparoscopy due to the decreased amount of pneumoperitoneum, making it more difficult to remove the accessory sac. 17
Although congenital anomalies have been loosely associated with CPE, no consistent anomaly has been identified. Of the latest 21 cases, 5 were associated with malrotation, 1 with epigastric hernia, and another with Meckel’s diverticulum.5–7,9,10,24 Surgeons should maintain a high index of suspicion for congenital anomalies when conducting a thorough inspection of the abdomen during abdominal exploration. If indicated, the surgeon could also address these congenital anomalies at the time of surgery, such as the completion of Ladd’s procedure in the case of malrotation reported by Frerichs et al. 6
In those presenting with obstruction, a common cause is the presence of congenital bands or adhesions, which are found intraoperatively. These bands are typically present at the terminal ileum, as was evident in the case of Mohamed et al. 20 Intraoperatively, our patient was found to have 2 constrictive bands, with that at the terminal ileum being the most obstructive. The contribution of this band to the patient’s obstruction was evident when the bowel decompressed upon its release. Additionally, Robbins et al described a unique case in which a mesodiverticular band tethered a Meckel’s diverticulum to the accessory peritoneal membrane and was thought to be contributing to their patient’s obstruction. 9 McMahon et al described a band-like effect on the transverse colon from the membrane’s opening for entry of the superior mesenteric artery. 15 There have also been reports of aberrant vessels being present in these bands.15,16,18 Other findings directly related to the accessory membrane itself include rotated mesentery, which was reported in the patients of Suseeharan et al, Ajibola et al, Tojal et al, and Chowdhury et al, whereas volvulus was described in the reports of Davis et al and Frerichs et al.6,10,11,24,28 Davis et al further described that the volvulus contributed to stenosis of the superior mesenteric vein (SMV) and likely the development of the SMV thrombus that was identified on preoperative imaging. 11 Additionally, a rare cause of obstruction as a sequela of CPE has been described by Ajibola et al, who reported a severe case in which gangrenous bowel loops were identified extruding from a defect in the accessory membrane. 5
Outcome
Given that patients present either asymptomatically or symptomatically and often have no contributing medical history, physical exam findings, or notable CT findings, the diagnosis of CPE is clinically difficult to achieve prior to surgery. Among patients who have been surgically treated, postoperative outcomes have been excellent with high survival rates.1,2 Additionally, no reports of recurrence have been reported, although more insight is needed regarding long-term outcomes, as the longest follow-up period is 7 years. 1 More research is also needed to identify patients at risk for becoming symptomatic and what factors may trigger obstruction to occur.
Conclusion
Our case report and findings from the most recent twenty-one cases add to the multi-faceted and vague presentations of CPE, illustrating the lack of consistent comorbidities, symptomatology, presentation, and imaging findings that may lead a clinician to this rare diagnosis. Clinicians should keep a wide differential that includes CPE when treating a patient with no identifiable cause of longstanding abdominal pain or acute bowel obstruction.
Footnotes
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
