What is the estimated frequency of a case with severe pelvic adhesions, probable deep endometriosis, chronic constipation, Ehlers-Danlos syndrome (EDS), and multiple prior surgeries in the population undergoing surgery for endometriosis or pelvic adhesions?

Rarity of This Clinical Presentation

This constellation of severe pelvic adhesions, probable deep endometriosis, chronic constipation, Ehlers-Danlos syndrome, and multiple prior surgeries represents an uncommon but well-recognized clinical scenario, occurring in approximately 1-2% of the general population when considering deep endometriosis prevalence, with additional complexity from EDS present in roughly 1 in 5,000 individuals. 1, 2

Individual Component Frequencies

Deep Endometriosis Prevalence

• Deep endometriosis (defined as lesions >5 mm beneath the peritoneal surface) affects approximately 1-2% of women in their reproductive years 2
• Among surgically treated endometriosis patients, 51.2% have deeply infiltrating lesions, with 43.3% involving uterosacral ligaments and 18.4% involving intestinal structures 3
• Deep endometriosis is strongly associated with severe pelvic pain (>95% of cases) and likely contributes to infertility 2

Ehlers-Danlos Syndrome Frequency

• Hypermobile EDS (hEDS) and Hypermobile Spectrum Disorders represent approximately one-third of patients referred to tertiary neurogastroenterology clinics 1
• Vascular EDS is much rarer, with prevalence of 1/50,000 to 1/200,000 1, 4
• hEDS patients commonly present with gastrointestinal symptoms including abdominal pain (especially postprandial), constipation, and autonomic dysregulation 1

Combined Presentation Complexity

Adhesive Disease Burden

• The risk of small bowel obstruction is highest following colorectal, oncologic gynecological, or pediatric surgery 1
• One in ten patients develops at least one episode of small bowel obstruction within 3 years after colectomy 1
• Patients with deep endometriosis frequently develop severe adhesions after surgery, though approximately 50% still conceive spontaneously 2

Multiple Prior Surgeries Impact

• Patients with deep endometriotic lesions have significantly more often undergone previous operations for endometriosis (p<0.01) compared to those without deep disease 3
• High rates of prior pelvic surgery (especially hysterectomy) are documented in women with chronic pelvic pain syndromes 1
• The presence of multiple prior surgeries increases adhesion complexity, with matted adhesions occurring in 67% of patients with previous surgery versus band adhesions 5

Clinical Context and Recognition

Why This Combination Appears Rare

• The intersection of EDS with deep endometriosis and severe adhesive disease creates a particularly challenging clinical scenario that requires multidisciplinary expertise 1, 2
• Many patients experience diagnostic delays due to poor knowledge within the medical community about these overlapping conditions 1
• The combination of visceral hypersensitivity from EDS, chronic pain from endometriosis, and mechanical issues from adhesions creates a complex pain presentation that may be misattributed 1

Important Clinical Pitfalls

• EDS patients may have visceral hypersensitivity that mimics dysmotility rather than true small bowel dysmotility, making surgical decision-making more complex 1
• The presence of EDS should prompt extreme caution with escalating invasiveness of nutrition support or repeat surgeries, especially in pain-predominant presentations 1
• Surgical complications in EDS patients can be severe, including delayed bowel perforations and fistula formation, requiring experienced surgical teams 1, 2

Prognostic Factors

• Patients with deep endometriosis and pelvic pain as surgical indication (OR 3.9) and totally obliterated rectovaginal pouch (OR 4.0) are independent predictors of deep disease 3
• Recurrence of deep endometriosis after complete excision is rare, with excellent pain relief in most patients 2
• However, recurrence of adhesive small bowel obstruction occurs in 12% at 1 year and 20% at 5 years after non-operative treatment 1

Population-Specific Estimates

While precise epidemiological data for this exact combination is not available in the literature, the clinical scenario represents:

• A rare subset within the 1-2% of women with deep endometriosis 2
• Further narrowed by the 1/5,000 prevalence of hEDS 1
• Complicated by the high surgical burden that characterizes both conditions independently 1, 3

This makes the complete presentation likely to occur in fewer than 1 in 10,000 women in the general population, though it may be encountered more frequently in tertiary referral centers specializing in complex pelvic pain and EDS management 1, 2.