Rare variant of genetically confirmed tuberous sclerosis complex presenting with bilateral renal angiomyolipoma in Wünderlich syndrome.

A 28-year-old woman came for non-traumatic right flank pain with hypotension and right flank mass. She had multiple hyperpigmented skin papules located on the centre area of her face, and angiomas on her toes. She was anaemic and had a blood transfusion on top of aggressive fluid resuscitation. Abdominal CT showed bilaterally enlarged kidneys and fluid collection in the right perirenal space (haemorrhage). The consideration was an angiomyolipoma in spontaneous perinephric haemorrhage. We considered tuberous sclerosis complex (TSC) and did genetic testing. Results revealed mutations in the TSC2 gene, consistent with the diagnosis of TSC. No immediate surgical plans were considered at that time. She opted to be discharged against medical advice and was scheduled for a close outpatient follow-up. The patient followed up after 2 weeks, already on sirolimus 2 mg once daily. She reported improved overall well-being and a decrease in the flank mass size.

Resistant hypertension after shockwave lithotripsy: the rude awakening of an adrenal incidentaloma.

A 41-year-old woman presented to the hospital because of left flank pain. CT scan of the kidneys revealed left-sided calculi and an incidental right adrenal mass, no other symptoms noted. She then underwent shockwave lithotripsy (SWL). However, immediately postoperatively, she had elevated blood pressure and remained hypertensive despite having four different medications. How SWL could have increased blood pressure could not be identified. On endocrine consult 16 months after SWL, she was found to now exhibit signs and symptoms of Cushing's syndrome. Further workup revealed the adrenal incidentaloma to be cortisol-secreting. After undergoing right laparoscopic adrenalectomy, her blood pressure normalised, cortisol levels decreased and signs of Cushing's syndrome gradually improved. We hypothesise that the performance of the SWL could have triggered the adenoma to 'awaken' from being non-functioning to cortisol-producing since this was the only intervening event. Observations of other patients are needed to validate our hypothesis.

Hypermucoviscous capsular 1 (K1) serotype Klebsiella pneumoniae necrotising fasciitis and metastatic endophthalmitis.

A 48-year-old man presented with a non-healing wound on his left foot after stepping on a nail. He self-medicated with amoxicillin, but the wound progressed prompting consult. On examination, his left foot was diffusely swollen with surrounding erythema, areas of gangrene, foul-smelling purulent discharge and subcutaneous emphysema. He was managed as a case of necrotising fasciitis and underwent emergent amputation. Three days after amputation, he developed a sudden and progressive blurring of vision, swelling and conjunctival erythema, with purulent discharge and the presence of hypopyon on the left eye. He was then managed as a case of endophthalmitis of the left eye and underwent pars plana vitrectomy. All cultures (blood, tissue and vitreous fluid) grew pan-susceptible hypermucoviscous Klebsiella pneumoniae, with positive string tests and confirmed by multilocus gene sequencing and sequence type analysis. He gradually improved with intravenous antibiotics, but only regained light perception in the left eye.