Unusual complication of Nephrotic syndrome Muzamil Latief 2nd year Resident Nephrology GMC Secb’d

CASE DETAILS 25 years aged, resident of mahaboobnagar, daily labourer presented with pedal edema, abdominal distention x 20 days No H/o of hematuria, oliguria, abdominal pain, vomiting, dyspnea, fever

CASE DETAILS General physical examination : Moderately built, moderately nourished Pedal edema +,No pallor, icterus, lymphadenopathy PR: 80 bpm regular. BP : 140/ 80 mm Hg Systemic examination : WNL

INVESTIGATIONS Hb: 12.8 gm/dl. TLC: 6500. PLT: 1.5 lakhs. Serum creatinine : 1.0 mg/dl Serum albumin: 2.3 mg/dl. Total cholesterol :285, HDL : 57, LDL : 206, TG:170. CUE : Albumin+++,RBC : Nil. 24 hrs urine protein : 3.6gm HIV : NR, HbsAg : NR, HCV : NR Ultrasound abdomen : Both the kidneys :normal in size

Renal Biopsy Biopsy includes 13 glomeruli, the glomeruli are enlarged in size with thickened basement membrane which show spikes. Tubules are closely packed. Interstitium and vessels are unremarkable. IF IgG and C3 positive Impression : Membranous nephropathy Placed on ARB+MMF+steroids

Contd….. remission till April 2018 (24 hr TUP 330mg) subsequently lost to follow up

Contd….. September 2018: Right loin pain x 5 days, no radiation of pain . No H/o of burning micturition. No H/o of fever, vomiting. No H/o of oliguria, hematuria. No H/o trauma. No h/o stone disease O/E : WNL

Investigations Hb 11gm/dl TLC 7500 plat: 2.5lac S.creat 0.8mg/dl Prot 5.8gm/dl S. alb 2.7gm/dl CUE: alb :2+,PC 1-2, RBC NIL 24 HR TUP: 4.6 gm

Contd…… Ultrasound abdomen : Large perinephric collection surrounding the right kidney. CT KUB : Large subcapsular collection tracking along the right psoas and in posterior pararenal space. Pelvicalyceal system is normal, there was no leak from the pelvicalyceal sytem on contrast administration

CT films

Differential diagnosis Perinephric abscess :Bacterial vs TB Urinoma Perinephric hematoma Transudative Perinephric Collection

Urologist opinion was taken and planned for RGP followed by PCN

RGP

Contd….. There was no leak from the PCS into the perinephric collection and so a PCN was placed. clear fluid drained from the PCN tube About 500ml / day of fluid was draining from the PCN for 3 days Fluid analysis :

Contd…… Transudative fluid collection of Nephrotic state Perinephric fluid analysis DD Clear, colourless, No cells detected, creatinine 0.9, Amylase 76 Cultures : Sterile CBNAAT :negative Serum Creatinine 0.8 Urinoma Pseudocyst of Pancreas Transudative fluid collection of Nephrotic state

In our patient ….. Urinoma High fluid creatinine ✖ Transudative perirenal collection High fluid creatinine ✖ Communication between renal pyelocalceal system and perirenal space ✖ Usually bilateral ✖ Transudative fluid✔ nephrotic state✔

Diagnosis Nephrotic syndrome : Primary Glomerular disease : Membranous nephropathy in Relapse With Perirenal transudative fluid collection–Grade 3 - FLOATING KIDNEY

COURSE Patient has PCN in situ and started on Immunosuppression Steriods/MMF +ARB

DISSCUSSION

D/D of Perirenal collection Hematoma Urinoma Abscesses Lymphangiomatosis Xanthogranulomatous pyelonephritis Pancreatitis Pseudocyst of pancreas Transudative Perirenal Collection American Journal of Roentgenology. 2004;183: 1697-1702. 10.2214/ajr.183.6.01831697

Contd…. URINOMA TRANSUDATIVE PERIRENAL COLLECTION Usually bilateral unless a complication of renal vein thrombosis coexists Transudative fluid nephrotic state Resolution with resolution of NS Sparse literature Accumulation of urine in subcapsular or perinephric space Obstructive /nonobstructive High fluid creatinine Communication between renal pyelocalceal system and perirenal space

Contd…. Perirenal Fluid collections in Medical renal disease were ist called as “Renal sweat” it was described in 1999 by Nyassa et al (Los Angeles) It was thought as a sign of Renal Failure in 14% of patients - when present it was always bilateral and diagnosed on USG Nyassa et al American j of Roetntgenology 99

Contd…. The source of the fluid is debated and its etiology has been ascribed to sodium retention and veno-occlusive state On renal sonographic images, kidney sweat is seen as an anechoic lucent rim around the kidney CT/MRI may be needed Hira lal et al Abdom Radiol (2017)

Grading of Perirenal Collections consisted of a small rim or thin layer of Fluid ranging from 3 to 10 mm in thickness surrounding part of the renal parenchyma, mainly the anterior aspect of the kidney and its lower pole or completely surrounding the kidney in a circumferential pattern consisted of a moderate amount of Fluid ranging from 1 to 5 cm in thickness with evidence of strands and indentations of the renal parenchyma consisted of a large Fluid collection greater than 5 cm in thickness surrounding the kidney “Floating Kidney” Haddad et al Clinical Radiology (2001) 56: 979±983

Contd…. When a large volume of fluid surrounds the kidney it is called ‘‘floating kidney’’ The condition is usually bilateral The presence of renal failure is not mandatory for the development of ‘‘renal sweat” Hira lal et al Abdom Radiol (2017)

Contd… Unilateral Transudative Collection is a rare entity Only few case reports available A patient had nephrotic syndrome (Lupus), developed right renal vein thrombosis without evidence of perirenal fluid around the right kidney, but had unilateral left perirenal fluid Royal college of Radiology Clinical Radiology (2001) 56: 979±983

Contd…. Another case of membranous Nephropathy and right renal vein thrombosis associated with ipsilateral perirenal subcapsular fluid collection managed with PCN and subsequently on antiplatelets and spontaneous remission of MN Orofino et al 'ThE JOURNAL OF UrOLOGY July 1986.

Take Home message Floating Kidney is a rare entity It can present as bilateral, bilateral with varying severity and rarely unilateral It can be minimal “perirenal rim” to large volume “Floating Kidney” Diagnosis is established based on Imaging and Fluid analysis PCN drainage may be required in some cases and of course “Treat the cause”

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