Case 40

HPI

27y M w hx inguinal hernia presented for pressure in his abdomen and bilateral flank pain. No fever or urinary complaints. No hematuria. + nausea and vomiting. Normal bowel movements. No back or testicular pain.

ROS

No fever, no chills, no HA, no visual changes, no SOB, no CP, no joint pain, no muscle aches, no rashes, no dysuria. + abdominal pain, + abdominal distension 

PE

Vitals: 97.6, 69, 19, 175/116, 100 RA

CONSTITUTIONAL: WDWN, NAD
ENT: MMM
CARDIOVASCULAR: RRR, cap refill <2 seconds
RESPIRATORY: Breath sounds clear, no distress present, no wheezing rales, rhonchi or tachypnea
GI/ABDOMEN: + abdominal distension and diffuse TTP. No r/g. + b/l CVAT
GENITOURINARY: Rt testicle >10 cm non reducible hernia, nontender. L side WNL
HEME/LYMPH: No adenopathy, no palpable or tender nodes.

As an extremely dedicated ED provider, you perform a thorough renal US as part of your exam. See the videos below.

 

Questions

  1. What is the most likely differential given the US findings?
  2. What other pathological findings are associated with these findings?
  3. What are the main categories of this pathology?
  4. What is the definitive treatment for this disease?
  5. What might ED management include?

Answers

  1. Polycystic kidney disease, likely autosomal dominant
  2. Hypertension, berry aneurysms, AKI/CKD, nephrolithiasis, hepatic and pancreatic cysts, valvular abnormalities, abdominal wall hernias
  3. Autosomal dominant – usually begins to manifest >18 years old, better prognosis
    Autosomal recessive – less common, manifests in early childhood, worse prognosis
  4. Renal transplant
  5. Manage HTN, renal consult, renal function tests, pain management

Also, here's the CT scan from the case: