What are the gynecological complications of Hypermobile Ehlers-Danlos Syndrome (hEDS) and considerations for a full hysterectomy in a 39-year-old patient with hEDS?

From the Guidelines

A full hysterectomy should be avoided in a 39-year-old patient with Hypermobile Ehlers-Danlos Syndrome (hEDS) unless absolutely necessary, due to the increased risk of complications and long-term effects on cardiovascular disease, osteoporosis, and dementia, as reported in the most recent study 1. Gynecological complications of hEDS include pelvic organ prolapse, dysmenorrhea, menorrhagia, dyspareunia, and pelvic floor dysfunction, which occur due to the connective tissue fragility inherent to the condition. When considering a full hysterectomy on an hEDS patient, several important considerations must be made, including:

• Surgical planning with careful tissue handling techniques, as hEDS patients have fragile tissues that tear easily and heal poorly
• Anesthesia management requiring special attention, as these patients may have autonomic dysfunction and unusual responses to anesthetics
• Extended recovery time, with longer hospital stays and rehabilitation periods
• Pain management, often requiring multimodal approaches including non-opioid options like gabapentin or pregabalin alongside traditional analgesics
• Hormone replacement therapy, particularly estrogen, to prevent early menopause symptoms and maintain joint stability, unless contraindicated
• Physical therapy before and after surgery to strengthen the pelvic floor and surrounding structures
• Preparation for potential complications, including wound dehiscence, bleeding, and poor tissue healing The surgical approach, whether vaginal, abdominal, or laparoscopic, should be carefully selected based on the patient's specific anatomy and tissue integrity, with a preference for the least invasive route, as recommended in the study 1. Key considerations for minimizing complications and optimizing outcomes in hEDS patients undergoing hysterectomy include:
• Avoiding hysterectomy unless absolutely necessary, due to the increased risk of complications and long-term effects
• Selecting the least invasive surgical approach
• Providing careful tissue handling and anesthesia management
• Implementing multimodal pain management and hormone replacement therapy as needed
• Offering physical therapy before and after surgery to strengthen the pelvic floor and surrounding structures.

From the Research

Gynecological Complications of Hypermobile Ehlers-Danlos Syndrome (hEDS)

• Uterine and rectal prolapse have been reported in nulliparous women with hEDS 2
• Visceroptosis, including ovarian and heart prolapse, has been observed in patients with hEDS 2
• Joint instability complications, chronic joint/limb pain, and mild skin hyperextensibility are common clinical features of hEDS 2, 3

Considerations for a Full Hysterectomy in a 39-year-old Patient with hEDS

• The Manchester repair is a surgical treatment option for uterovaginal prolapse that preserves the uterus and may be considered for patients with hEDS who wish to retain their uterus 4
• However, patients with hEDS may be at increased risk of complications after surgical procedures, including recurrence of visceral prolapse 2
• Prophylactic antibiotic regimens, such as cefazolin plus metronidazole, may be effective in reducing surgical site infections after hysterectomy 5
• Primary care providers should be familiar with the evaluation, management, and indication for referral to a connective tissue disease specialist for patients with hEDS 6