Midgut Volvulus with Necrosis: A Complication of Endometriosis

Abstract

Background:

Deeply infiltrating endometriosis (DIE) can involve the bowel leading to necrosis with septic shock, a rare complication occurring in 5% of endometriosis patients. This case report aims to illustrate a life-threatening complication of endometriosis, emphasizing the need for strong clinical acumen in atypical cases of endometriosis. This also highlights the need for improved noninvasive diagnostic modalities to enable earlier detection.

Case:

A 33-year-old woman with a presumed diagnosis of Crohn’s disease presented to the emergency department in septic shock following six months of chronic gastrointestinal symptoms. She had previously undergone colonoscopy with biopsy showing terminal ileitis. Due to signs of peritonitis and systemic infection, she was urgently taken to the operating room and underwent an exploratory laparotomy that revealed midgut volvulus and extensive hemorrhagic necrosis. She underwent resection of necrotic bowel and cecum with end jejunostomy. Final pathology demonstrated DIE involving the bowel wall and mesentery, with no histologic evidence of Crohn’s disease. Her postoperative course was complicated by prolonged intensive care unit (ICU) admission, cardiac arrest, and short bowel syndrome requiring nutritional support. Post operatively a pelvic magnetic resonance imaging (MRI) confirmed stage IV endometriosis with infiltration into the rectosigmoid. She was discharged in stable condition with multidisciplinary follow-up involving colorectal surgery, gynecology, and reproductive endocrinology.

Conclusion:

This case illustrates a rare and severe presentation of intestinal endometriosis mimicking Crohn’s disease, progressing to life-threatening bowel necrosis. It underscores the diagnostic challenges of endometriosis and the critical role of multidisciplinary care in optimizing outcomes for complex cases.

INTRODUCTION

Deep infiltrating endometriosis is defined by invasion of endometrial glands and stroma >5 mm beneath the peritoneum, affecting pelvic structures and, less commonly, other structures.¹ Bowel endometriosis frequently involves the rectosigmoid, rare cases may affect the ileum, appendix, or cecum.^2,3 These presentations may mimic inflammatory bowel disease (IBD), especially Crohn’s disease, causing diagnostic challenges.³

We report the case of a young woman with chronic gastrointestinal symptoms and biopsy-proven ileitis, presumed to be Crohn’s disease. The patient later presented to the emergency department (ED) in septic shock with acute abdomen due to midgut volvulus and extensive small bowel necrosis. Emergent surgical intervention revealed deeply infiltrating endometriosis.

This case emphasizes the diagnostic complexity of deeply infiltrating endometriosis when presenting with predominantly gastrointestinal symptoms, and highlights the importance of maintaining a broad differential diagnosis in women with recurrent bowel obstruction. Endometriosis can present with a wide range of symptoms including dysmenorrhea, pelvic pain, dyspareunia, dyschezia, and cyclical rectal bleeding. When the bowel is involved, symptoms mimic gastrointestinal disorders such as IBD or irritable bowel syndrome (IBS), making the diagnostic process challenging.^4,5

Diagnosing endometriosis requires pathology confirmation, often through laparoscopy. Although imaging may support suspicion, surgical assessment remains the gold standard.⁶

CASE PRESENTATION

Patient History and Initial Presentation

A 33-year-old nulligravid woman presented with a one-year history of 10-kg unintentional weight loss and chronic gastrointestinal symptoms (postprandial abdominal pain, bloating, distention, borborygmi, and constipation). She had multiple hospitalizations for partial small bowel obstructions, managed conservatively. Serial computed tomography (CT) scans (Figures 1A and 1B) demonstrated a transition point in the terminal ileum with proximal small bowel dilatation. Previous colonoscopy in the nonacute setting demonstrated patchy erythema in terminal ileum with a normal-appearing colon. Biopsies showed ileitis with ulceration and crypt distortion, but no granulomas. Based on pathology and symptomology, a theoretical diagnosis of Crohn’s disease was made, and empirical budesonide was started. The patient had no prior gynecologic symptoms concerning for endometriosis. Notably, she did not report cyclical pelvic pain or gastrointestinal symptoms, dyspareunia, or dyschezia. She had a history of dysmenorrhea since menarche, which was managed by combined oral contraceptive pill use and levonorgestrel intrauterine device. Pelvic examination at the time of gynecologic evaluation for dysmenorrhea was unremarkable. Her abdominal pain was postprandial in nature and not temporally associated with menses.

Figure 1.

Contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrating high-grade small bowel obstruction. (A) Sagittal view showing markedly dilated proximal small bowel loops with a transition point in the distal ileum. (B) Axial view demonstrating severe small bowel dilation with air-fluid levels and mesenteric congestion, concerning for evolving ischemia.

She did not seek further gynecologic evaluation until one month before her ED admission. At that visit, she endorsed dysmenorrhea, but was mainly complaining of new onset hirsutism and acne, raising concern for polycystic ovarian syndrome. Endometriosis was not suspected at the time given the absence of active classic gynecological complaints and predominance of gastrointestinal symptomology.

With this working diagnosis, an elective outpatient ileocecectomy for her presumed Crohn’s was scheduled. In the interim, she presented to the ED in septic shock: heart rate in 140s, systolic blood pressure in 60s, lactate of 8.4 mmol/L, and pH of 7.04.

Emergent Surgical Intervention

Due to the patient’s signs of sepsis and peritonitis, a CT scan of the abdomen and pelvis revealed a high-grade small bowel obstruction with bowel dilation, new ascites, abnormal wall thickening and edema of dilated small bowel loops. She was taken to the operating room for an emergent exploratory laparotomy. Intraoperatively, a midgut volvulus was identified around an inflamed terminal ileum, with hemorrhagic necrosis involving distal small bowel. The mesentery was severely engorged with a developing hematoma and areas of microperforation but no gross evidence of Crohn’s disease.

The necrotic bowel was resected from the ileocecal valve proximally to 90 cm of the ligament of Treitz distally, including the cecum, and appendix; the proximal ascending colon was preserved. The remaining small bowel measured 90 cm, and an end jejunostomy was created. The colon appeared grossly normal. Pelvic anatomy was not notable on gross inspection during the emergent operation, which was focused on management of bowel necrosis and hemodynamic instability.

Final pathology revealed deeply infiltrating endometriosis involving the terminal ileum, cecum, and appendix, confirming hemorrhagic necrosis of the small bowel, reactive mesenteric lymphadenopathy, and no histologic features of Crohn’s disease.

Postoperative Course

Recovery was complicated by cardiac arrest from pulseless electrical activity (PEA) requiring advanced cardiac life support (ACLS). Hemodynamic instability was secondary to hemoperitoneum from mesenteric bleeding due to venous congestion and hematoma. The patient experienced ileus, and short bowel syndrome with malnutrition necessitating total parenteral nutrition (TPN). She required prolonged intensive care before stabilization and eventual discharge to home with follow-up through colorectal and gynecologic surgery.

Postdischarge, the minimally invasive gynecologic surgery (MIGS) team proceeded with pelvic magnetic resonance imaging (MRI) (Figures 2A and 2B), confirming stage IV deeply infiltrating endometriosis with peritoneal thickening. Obliterated posterior cul-de sac, thickening of the rectovaginal septum, bilateral uterosacral ligament involvement and tethering of the ovaries without adnexal masses were also noted.

Figure 2.

Pelvic magnetic resonance imaging (MRI) demonstrating stage IV deeply infiltrating endometriosis. (A) Sagittal view showing thickening of the posterior peritoneum and rectovaginal septum with obliteration of the posterior cul-de sac. (B) Axial view demonstrating bilateral uterosacral ligament involvement and ovarian tethering, consistent with advanced endometriotic disease, without discrete adnexal masses.

Following confirmation of endometriosis, she was referred to reproductive endocrinology and infertility (REI) for long-term disease management and fertility preservation.

Fertility Preservation and Surgical Planning

Given the need for further resection and ostomy reversal, oocyte preservation was recommended prior to the operative intervention. Multidisciplinary surgical planning was arranged between colorectal and MIGS team for ostomy reversal and definitive endometriosis excision.

Definitive Surgery

Five months after the initial operation, the patient underwent joint robotic-assisted resection of the end ileostomy with isoperistaltic ileocolonic anastomosis, bilateral salpingectomy, and excision of deeply infiltrating endometriosis. Intraoperatively, a stricture was noted on the ascending colon, it was resected, and a side-to-side ileum-to-transverse colon anastomosis was created. The MIGS team identified extensive pelvic adhesive disease, a subcentimeter left ovarian endometrioma (ruptured intraoperatively), and bilateral hydrosalpinxes with tubal endometriosis. Dissection involved the posterior cul-de sac, uterosacral ligaments, and rectosigmoid colon. Two fibrotic lesions in the cul-de sac were suspected to represent endometriosis but left in place due to concern for bowel function, specifically the rectosigmoid. The left ovarian cyst bed was fulgurated.

The patient tolerated the procedure well, was successfully weaned off TPN, and continues to recover. Hormonal suppression was planned for ongoing management of residual endometriosis.

DISCUSSION

This case demonstrates the severe complications and diagnostic challenges that can arise when deeply infiltrating endometriosis presents with atypical, nongynecologic symtpoms and mimics primary gastrointestinal pathology. This patient had deeply infiltrating endometriosis extended to the gastrointestinal track, closely mimicking Crohn’s disease and ultimately resulting in life-threatening bowel obstruction and necrosis. Her acute abdominal symptoms reinforce the importance of considering endometriosis in the differential diagnosis for women with persistent or unexplained gastrointestinal complaints.⁷

This case illustrates that advanced endometriosis can present without classic symptoms such as dyspareunia, dyschezia, cyclical pain, or infertility. Although the patient had a history of dysmenorrhea controlled by hormonal therapy, she remained symptom-free for years before presenting with nonspecific complaints of dysmenorrhea, hirsutism, and acne. Endometriosis was not suspected, as her symptoms and biopsy-proven ileitis suggested Crohn’s disease. However, emergent surgery revealed extensive bowel endometriosis with midgut volvulus and mesenteric hemorrhage. Volvulus, although rare, is a recognized complication of bowel endometriosis due to adhesive disease or transmural invasion, carrying risks of torsion, ischemia, and necrosis.^2,8

Endometriosis of the terminal ileum, appendix, or cecum is rare, affecting <5% of intestinal cases.^3,9 Presentations can be mistaken for IBD and in the absence of granulomas on biopsy, as in this patient, the diagnosis of Crohn’s disease remains presumptive. Additionally, nonspecific ileitis is seen secondary to endometriotic inflammation.¹

Several key considerations emerge from this case. First, the pathophysiology of endometriosis has unpredictable clinical progression. Disease burden does not always correlate to symptoms; minimal or nonspecific gynecological symptomology may still harbor extensive disease. Endometriosis is not limited to chronic pelvic pain but could affect multiple organ systems and lead to complications outside the reproductive tract.¹⁰

Second, endometriosis remains difficult to diagnose preoperatively, particularly in patients without classic gynecologic symptoms and with objective gastrointestinal findings. The current gold standard remains with histology. Noninvasive advances in transvaginal ultrasound and MRI show promise in detecting deep infiltrating endometriosis, especially with bowel or bladder involvement, but sensitivity remains limited and operator-dependent.^11,12 High-resolution MRI with rectal contrast or targeted molecular biomarkers are focuses of active research but are not standard practice.^13–15 Improved access to and utilization of existing imaging modalities, such as expert tansvaginal ultrasound and pelvic MRI, along with continued research into adjunctive noninvasive diagnostic tools, may facilitate recognition of atypical presentations.

Finally, long-term care requires a multidisciplinary approach including surgical intervention, symptom control, and fertility preservation when desired. The patient benefited from coordinated care from the colorectal surgery, MIGS, and REI teams. REI involvement was critical for oocyte cryopreservation prior to further surgical intervention.

This case represents a rare and life-threatening complication of deeply infiltrating endometriosis initially thought to be Crohn’s disease. Despite gastrointestinal-focused symptomology and evaluation, the true etiology was discovered intraoperatively. This underscores the importance of multidisciplinary awareness and timely utilization of appropriate diagnostic tools in recognizing rare and atypical presentations of endometriosis. It also highlights the benefits of a coordinated, patient-centered approach including emergent surgical resection, diagnostic imaging, fertility counseling, and a joint robotic surgery for fertility sparing treatment.