Fibromuscular Dysplasia
Description
●The introduction:
➣Inflammatory and non-inflammatory arteriopathies are the two main categories of arterial disorders involving numerous aneurysms.
➣Diagnosis is frequently challenging, although treatment strategies vary significantly between these two.
➣FMD: a rare non-inflammatory, non-atherosclerotic arteriopathy that primarily affects women between the ages of 15 and 50. It frequently affects the renal arteries (≈70%) and can manifest as renovascular hypertension. Rarely, it can result in aneurysms, dissections, or ischaemic complications in uncommon locations.
➣This case is uncommon as the patient's diagnosis was complicated by the presence of minor intestinal annular ulcers and burst hepatic artery aneurysms.
● Requirements for Admission:
• Weight: 40 kg; height: 161 cm; weight loss: 10 kg throughout the previous year.
• BP: 177/120 mmHg, T: 36.6°C, P: 101/min.
• There are no cutaneous or neurological symptoms, although there is soreness in the right hypochondrium.
➣Labs
• WBC (neutrophils): 9300/μL.
•CRP (inflammatory response): 8.6 mg/dL.
•Hb: 9.7 g/dL (progressive anaemia).
• Infectious and autoimmune markers: QuantiFERON-TB → negative, ANA, and ANCA (MPO & PR3).
• 33 ng/mL/hr (↑↑) is the plasma renin.
•Aldosterone: 25.5 pg/mL (better than before).
➣ Imaging and Cardiac Evaluation
• Abdominal X-ray, or CXR: normal.
• ECG: LVH, or LV high-voltage pattern.
• Echo: no valvular disease, excellent LV function.
●Imaging Results:
➣Abdomen CT: intra-gallbladder haemorrhage and many hepatic artery aneurysms.
➣On CT angiography, there are many liver aneurysms (hepatic artery).
➣Follow-up imaging:
• Hepatic artery aneurysm rupture → coil embolisation was carried out.
•Angiography: tubular stenosis in the right renal artery (not the traditional "string of beads") and a minor aneurysm in the GEA.
• Coronary CT: left coronary diagonal branch aneurysm, 5 mm.
• No aneurysms were seen on head/neck imaging.
➣ Pathology Results
• A small bowel resection was carried out due to bleeding, obstruction, and annular ulcers.
• Histopathology: → Internal elastic lamina rupture; → Recanalisation following ischaemic ulceration, a vascular blockage.
→Collagen, elastic fibres, and smooth muscle proliferation results in thicker intima and a constricted lumen; there is no infiltration of inflammatory cells; and fibromuscular dysplasia (FMD), a non-inflammatory arteriopathy, is confirmed.
Protocol
● Challenges the Patient Faces
➣ The patient had fibromuscular dysplasia, a non-inflammatory blood vessel disease that can cause aneurysms and vascular complications;
➣ There were multiple aneurysms, which increased the risk of vessel rupture, thrombosis, and distal organ ischaemia;
➣ There was psychological distress and anxiety because of the diagnosis and prognosis;
➣ The patient had a complicated medication regimen and required close monitoring;
➣ There was uncertainty in management because the condition was uncommon and required a multidisciplinary approach.
● Detailed nursing interventions.
➣Comprehensive monitoring: ongoing evaluation for indications of aneurysm rupture or the advancement of vascular disease. This entails keeping an eye on cardiovascular parameters, neurological health, limb perfusion, vital signs, discomfort, and physical changes.
➣Medication management: giving out prescription drugs and educating patients on their uses, dosages, and adverse effects. Anticoagulants, antihypertensives, and painkillers receive particular care, guaranteeing rigorous adherence and close observation for negative side effects.
➣Psychological Support: Research-proven nursing techniques to alleviate anxiety, reassure, and counsel the patient and their family. To reduce anxiety and uncertainty, nurses provide information regarding the illness, prognosis, and course of treatment.
➣ Multidisciplinary Care Coordination: Nurses help cardiologists, radiologists, surgeons, and other medical specialists communicate with one another. Serve as liaisons to guarantee prompt therapy administration and investigations (such as imaging and lab testing).
➣Education for Patients and Their Families: Comprehensive instruction on how to adjust one's lifestyle (diet, exercise, avoiding triggers), take care of oneself, identify warning signals (sudden pain, neurological changes), and know when to seek emergency treatment. guidance on medication adherence and at-home blood pressure monitoring.
➣Prevention of Complications: Using procedures to avoid infection, thrombosis/embolism (e.g., limb elevation, careful mobility), and intravenous line management. monitoring for issues with medication, bed rest, or catheters.
➣ Holistic Support: Humane care via advocating for patient values and preferences in care planning, emotional support, and shared decision-making facilitation.
●Management Strategies
➣ Nursing teams adhered to an evidence-based procedure, regularly assessed patient outcomes, and modified treatments in light of clinical recommendations and observed outcomes.
➣Frequent multidisciplinary meetings guaranteed the quality of care and prompt treatment plan modifications where necessary.
➣To be knowledgeable and competent in advanced vascular situations, team members received continuous clinical training.
➣Patient reactions and results were meticulously recorded to inform future advancements in nursing care for cases like these.
Notes
For more details https://doi.org/10.1016/j.jccase.2015.05.005
