Case 1: KW Lee, KF Chan; Case 2: KF Kwan, KL Siu

(A) Case 1

CW is a 4 years old boy, admitted for severe headache and vomiting for several days. Physical examination showed left facial nerve palsy (lower motor neuron lesion) and his blood pressure was 220/150 mmHg. Cardiovascular and respiratory examinations were uneventful. Abdominal examination did not reveal any mass and bruit. Fundoscopic examination showed papilloedema. He was managed as malignant hypertension and subsequently his blood pressure was controlled with three antihypertensive drugs.

Investigations includes renal function test, autoimmune markers, serum renin and angiotensin, thyroid function, cortisol, 24 hours urine for catecholamine metabolites, ultrasound of abdomen with renal Doppler study, DTPA scan with captopril, and MIBG scan were all normal. Echocardiogram did not show any coarctation of aorta. Renal arteriogram was then performed (Figures 1 & 2).

Figure 1 Arteriogram of right renal artery.

Figure 2 Arteriogram of left renal artery.

Question

1. What are the features shown in the renal arteriogram?
2. What is the diagnosis?
3. What is the prognosis?

(B) Case 2

A full term baby girl with birth weight of 2.88 kg was admitted on Day 28 for poor weight gain, skin lesions over limbs and perinanal area. She was discharged on Day 3 with normal physical examination. On admission, she weighed 2.86 kg.

Physical examination revealed (Figures 3 - 6):

Question

What is the most likely diagnosis?

Figure 3 Abdominal distension.

Figure 4 Multiple skin lesions like that on her left thigh.

Figure 5 Skin lesion on the finger.

Figure 6 Skin lesion on the perianal area.

Answer to "Clinical Quiz" on Pages 223-224

N.B. The Editors invite contributions of illustrative clinical cases or materials to this section of the journal.