Role of CT scan in diagnosis of renal lymphangiectasia: our single-center experience.

BACKGROUND: Renal lymphangiectasia is rarely reported benign renal disorder of lymphatic malformation. Though found incidentally; it presents with nonspecific symptoms and shows characteristic findings in radiological imaging studies. AIM: Here, we report eight patients with symptoms, laboratory and imaging findings compatible with renal lymphangiectasia. This report describes clinical and laboratory characteristics, treatment, Imaging findings and outcome of a series of patients with renal lymphangiectasia and reviews the literature. METHODS AND MATERIAL: Eight patients (mean age 45 years, male:female ratio 3:1) from 1st January 2011 to 30th June 2016; showing renal lymphangiectasia as incidental finding on CT IVP were included in the series. Imaging and laboratory findings were reviewed. Two out of eight patients (25%) underwent aspiration of collection and laboratory findings confirmed the diagnosis of renal lymphangiectasia. Four out of eight patients (50%) did not undergo aspiration of fluid and were offered conservative treatment. Two out of eight patients (25%) were donors for renal transplantation who were managed conservatively. RESULTS: Renal lymphangiectasia was diagnosed on CT IVP. In each case, where aspiration of collection fluid was offered, the laboratory diagnosis of renal lymphangiectasia was confirmed and patients were managed conservatively. However, large collection in one patient was relieved by percutaneous aspiration. CONCLUSIONS: Renal lymphangiectasia can be diagnosed with CT scan and confirmed by laboratory tests. As it may be confused with other cystic lesions of kidney; proper diagnosis and exclusion of other differentials can be effectively offered by CT scan IVP, which can avoid unnecessary invasive treatment options.

The long-term outcomes after staged repair of exstrophy-epispadias complex.

INTRODUCTION: Classic bladder exstrophy (BE) is a rare malformation of the genito-urinary tract affecting 1:50,000 to 1:100,000 live births. The surgical reconstruction of the BE-epispadias complex is challenging for the most experienced pediatric urologists, surgeons, and orthopedists. PURPOSE: To assess the success of staged reconstruction of the BE and long-term effects on the upper urinary tract, renal function, and continence. MATERIALS AND METHODS: This is retrospective study; between 1994 and 2013, 30 patients with BE have undergone stage 1 repair at the institute. Eighteen male patients have been operated for epispadias repair and thirteen patients have undergone Guy Leadbetter bladder neck reconstruction. Three patients required augmentation cystoplasty one child is continent after epispadias repair only and one child attained continence after single-stage repair. RESULTS: Hence, out of 17 patients, 14 are socially continent, four patients require clean intermittent self-catheterization for bladder emptying. Four patients, who are coming for regular follow-up, are awaiting continence procedure. Two patients who underwent augmentation cystoplasty are on hemodialysis for renal failure and one child has altered renal function. CONCLUSION: In our experience, the modern staged repair offers a low risk of renal scarring with acceptable continence opportunity with acceptable cosmetic appearance of external genitalia in the males and females.

Continent cutaneous diversion and external genitalia reconstruction in a child with severe variety urogenital sinus and ambiguous genitalia.

The diagnosis and management of a child with ambiguous genitalia and severe variety of urogenital sinus with a high vesico-vaginal confluence is challenging. This 4-year-old female child had solitary right kidney with ectopic ureter opening in high variety of urogenital sinus with hypo-plastic urinary bladder and incontinence. We describe genitourinary reconstruction with complete functional rehabilitation in this child. This complex problem was managed with continent urinary diversion with Penn pouch and refashioning of external genitalia, rendering continence and near normal female external genitalia. The child and parents are happy with continence and aesthetically normal external genitalia.

Triad of bladder agenesis with solitary kidney and ectopic ureter.

The bladder agenesis is an extremely rare congenital genitourinary anomaly; only 60 cases have been reported in the English literature and only 19 of these were noted in viable neonates.[1] Our case represents the 20th live birth with bladder agenesis. The triad of bladder agenesis with solitary kidney and ectopic ureter is seldom compatible with life due to associated anomalies.[23] Successful treatment and the long-term prognosis are usually poor because of the associated abnormalities. In our case, function of left solitary kidney was good and child did not have associated life-threatening disorder. Hence child was successfully managed with continent urinary diversion with good quality of life.