What is the best approach to manage the care of a 45-year-old female patient with a history of bladder prolapse, visceral pseudo-aneurysm of the pancreaticoduodenal artery, umbilical hernia, poor scar healing with recurrent infection, myopia, and claw toes requiring osteotomy, and a family history of cardiovascular disease?

Clinical Approach to Managing a 45-Year-Old Female with Multiple Connective Tissue Manifestations

This patient's constellation of findings—bladder prolapse requiring surgical repair, visceral pseudo-aneurysm of the pancreaticoduodenal artery, umbilical hernia, poor wound healing with recurrent infections, myopia, and claw toes requiring osteotomy—strongly suggests an underlying connective tissue disorder that must be systematically evaluated and managed through a multidisciplinary team approach to prevent life-threatening complications.

Immediate Priority: Rule Out Hereditary Connective Tissue Disorder

The combination of visceral artery aneurysm, multiple hernias, poor wound healing, and skeletal abnormalities raises critical concern for vascular Ehlers-Danlos syndrome (vEDS) or other hereditary connective tissue disorders. 1

Key Diagnostic Steps:

• Obtain genetic testing immediately for COL3A1 mutations (vEDS), fibrillin-1 mutations (Marfan syndrome), and other collagen disorders, as these patients face 25% risk of major arterial or organ rupture by age 40 1
• Perform comprehensive vascular imaging including CT angiography of the entire aorta, cerebral vessels, and visceral arteries to identify additional aneurysms, as pancreaticoduodenal artery aneurysms are rare (2% of visceral aneurysms) and often associated with celiac artery stenosis or systemic arteriopathy 2, 3
• Assess for celiac artery disease (occlusion or >70% stenosis), present in 66% of pancreaticoduodenal artery aneurysms, which may require concomitant hepatic revascularization during any intervention 3

Cardiovascular Risk Stratification and Management

Given the family history of maternal cardiovascular death at age 69 and the patient's vascular pathology, aggressive cardiovascular risk modification is mandatory. 1

Blood Pressure Management:

• Target blood pressure <130/80 mmHg using beta-blockers as first-line agents, as they reduce arterial wall shear stress and may slow aneurysm progression 4, 5
• Add ACE inhibitors or ARBs if needed to achieve target, as renin-angiotensin system alteration shows robust evidence in aneurysm management 5

Lipid Management:

• Initiate high-intensity statin therapy (atorvastatin 40-80 mg or rosuvastatin 20-40 mg) for patients with vascular disease and atherosclerosis 1
• Target LDL-cholesterol <1.8 mmol/L (<70 mg/dl) given imaging evidence of vascular disease 1

Surgical Risk Assessment and Perioperative Planning

Any future surgical procedures in this patient carry extremely high risk due to poor wound healing and potential underlying connective tissue disorder. 1

Critical Surgical Considerations:

• All surgical decisions must be made by a multidisciplinary team including vascular surgery, genetics, anesthesiology, and wound care specialists 1
• Avoid elective surgery whenever possible until genetic diagnosis is established, as patients with vEDS have catastrophic complication rates including spontaneous arterial rupture, organ rupture, and wound dehiscence 1
• If surgery is unavoidable, use meticulous tissue handling, avoid tension on tissues, consider prophylactic mesh reinforcement for any fascial closure, and plan for prolonged wound healing 1

Wound Healing Optimization:

• Screen for nutritional deficiencies (vitamin C, zinc, protein) that impair collagen synthesis and wound healing 1
• Optimize glycemic control if diabetes is present, targeting HbA1c <7% 1
• Consider prophylactic antibiotics for any invasive procedures given history of recurrent wound infections 1

Management of Existing Vascular Pathology

The pancreaticoduodenal artery pseudo-aneurysm requires ongoing surveillance regardless of prior repair, as reintervention rates are high (28-47%). 3, 6

Surveillance Protocol:

• Obtain CT angiography every 6-12 months to monitor for aneurysm recurrence or new aneurysm formation 3, 6
• Maintain strict blood pressure control to minimize rupture risk, which occurs independent of aneurysm size (mean rupture size 16.4 mm vs intact size 19.4 mm, p=0.95) 3
• Educate patient on rupture symptoms: sudden severe abdominal pain, hematemesis, melena, or hematochezia requiring immediate emergency evaluation 2, 6

If New Aneurysms Develop:

• Endovascular coil embolization is preferred initial treatment over open surgery, though reintervention rates remain high 3, 6
• Assess need for hepatic revascularization if celiac occlusion is present, though not mandatory in all cases (61% managed without bypass) 3

Functional Assessment and Quality of Life Optimization

Use a four-domain framework to comprehensively assess and optimize this patient's care beyond medical management. 1

Medical Domain:

• Coordinate care between primary care, genetics, vascular surgery, and orthopedics 1
• Reconcile all medications at each visit to avoid polypharmacy and drug interactions 1
• Screen for depression and anxiety using validated tools (PHQ-2), as chronic multimorbidity increases risk 1

Physical Functioning Domain:

• Refer to physical therapy for management of claw toe deformity and optimization of mobility 1
• Prescribe low-impact aerobic exercise 30-60 minutes, 3-4 days per week if blood pressure is controlled, avoiding heavy lifting or Valsalva maneuvers that increase arterial wall stress 4

Mind and Emotion Domain:

• Screen for anxiety and depression at each visit, as 15-20% of patients with vascular disease develop anxiety and 33% develop depression 4
• Consider cognitive behavioral therapy (14 sessions over 4 months) if anxiety or depression is identified 4

Social and Physical Environment Domain:

• Assess medication affordability and leverage formulary options to minimize out-of-pocket costs 1
• Evaluate need for advance care planning given high-risk vascular pathology and potential for catastrophic complications 1
• Ensure adequate social support for managing complex medical regimen 1

Lifestyle Modifications and Risk Reduction

Smoking cessation is absolutely critical, as smoking is directly linked to aneurysm development, growth, and rupture 4, 5

• Provide intensive smoking cessation counseling with pharmacotherapy (varenicline or combination nicotine replacement) if patient smokes 4
• Counsel on heart-healthy diet low in saturated fat to reduce cardiovascular risk 1, 4
• Avoid NSAIDs (ibuprofen, naproxen) which may impair wound healing and increase bleeding risk in patients with vascular pathology 1

Common Pitfalls to Avoid

• Never assume isolated findings—this pattern demands systematic evaluation for connective tissue disorder 1
• Do not delay genetic testing while pursuing other workup, as diagnosis fundamentally changes surgical risk stratification 1
• Avoid "watchful waiting" without structured surveillance protocol for vascular pathology, as rupture risk is independent of size 3
• Do not perform elective surgery without multidisciplinary team evaluation in patients with poor wound healing and multiple hernias 1
• Never prescribe medications without checking formulary status to avoid financial toxicity and non-adherence 1