1. PERINATAL DIAGNOSED UNILATERAL MULTICYCTIC DYSPLASTIC KIDNEY A.HELLARA*, A. AYAT*, A. ACHOUR**, K.SALHI*,S.JERBI**, H. SOUA*, A. NOURI***, H.A. HAMZA**, M.M.SOUISSI****, H.BENHAMMOUDA*, M.T SFAR* Department of Pediatrics ** Department of Radiology *** Department of PediatricSurgey **** Department of Gynecology Hammamet, 26-28 April 2012 5th Arab Radiology Congress

2. CLUB DES PÉDIATRES DU CENTRE ET DU SUD 27 Juin 2009 INTRODUCTION Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney Multicyctic dysplastic kidney (MCDK) : the most frequently diagnosed congenital cyctic kidney malformation in children. Incidence :1/4300 NewBorn. Arises in the embryo in the stage of development of the grown up kidney(metanaphros). The bilateral affection is rare and usually lethal in utero.

3. To study the diagnostic aspects Clarify the epidemiological profile Determine the medium and long term evolutionary aspects of unilateral MCDK, as well as those of associated kidney anomalies. OBJECTIFS Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

4. Retrospective survey concerning 18 cases of unilateral MCDK collected in the department of pediatrics in the hospital “Tahar Sfar” of Mahdia in Tunisia between 1994 and 2009: *15 cases were diagnosed by prenatal kidney ultrasonography (USG) *3 cases were diagnosed after birth: 2 cases by the discovery of an abdominal mass, 1 case during the exploration of urinary infection. Searching for associated malformative uropathy was systematic. These patients were followed up at the paediatric consultation, they had renal analysis, and kidney ultrasonography (USG) each 6 months the first 2 years then each year until total disappearance of the mass. MATERIALS AND METHODS (1/2) Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

5. In the diseased kidney: Many non-communicating cysts of variable sizes. The largest cysts are located at the periphery. No renal parenchyma can be identified. MATERIALS AND METHODS (2/2) POSTNATAL RENAL ULTRASONOGRAPHY UNILATERAL MULTICYCTIC DYSPLASTIC KIDNEY Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

6. RESULTS: SEX DISTRIBUTION

7. Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney RESULTS: LOCALISATION

8. Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney Associated kidney malformations: 61% of the cases. RESULTS: CONTROLATERAL KIDNEY MALFORMATIONS(50%)

9. Ophthalmological abnormalities (1 case): exophthalmia and strabismus. Genital malformations (3 cases): phimosis and tight foreskins, testicular ectopia, and bilateral scrotal inguinal hernia. Facial dysmorphia (3cases): retrognathism, cleft lip, nasal collapse. Orthopedic malformations (1case): congenital talipes equinovarus, syndactyly Urinary tract infection was observed in all 6 cases with an associated malformative uropathy: 5 VUR and 1 refluxing megaureter. RESULTS :EXTRA-RENAL MALFORMATIONS Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

10. Conservative treatment :14 cases (77%) Nephroctomy: 4 cases (22%) * 1 case at the age of 3 years and 4 months due to an increasing size of the dysplastic kidney. * Associated to an anti reflux procedure in 3 cases, because of high grade VUR. Treatment of the associated uropathy: 6 cases (33%) Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney RESULTS: TREATEMENT

11. Ultrasonographic follow up has shown complete spontaneous involution of the affected kidney in 4 cases at an average age of 3 year old. With an average decline of 7 years: * kidney compensatory hypertrophy : all cases. * No complications were identified: such as hypertension, kidney failure, kidney degeneration. * A boy died at the age of 3 years old due to a septic shock. Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney RESULTS: EVOLUTION

12. The Multicyctic dysplastic kidney (MCDK) : non hereditary cyctic kidney abnormality which is usually unilateral. Its predominantly occurs in boys: 58 to 70% of the cases. Left kidney : 51 to 70% of the cases. Controlateral kidney abnormalities :7 to 50% of the cases, vesico-ureteral reflux is the most associated uropathy. The course of this disease is often benign. The occurrence of hyper tension is rare (5.4 %). Malignant degeneration is exceptional (the odds of Wilms tumor is estimated at 0.03 to 0.1%) The prognosis is directly related to the existence of abnormalities in the controlateral kidney. Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney DISCUSSION(1/4)

13. The appropriate management of the MCDK remains controversial:  Therapeutic abstention: * Can be justified by the low incidence of short term complications and usual spontaneous involution. * In the isolated unilateral MCDK, using antibiotics to prevent urinary infection is useless. It’s indicated if there is high grade vesico-uretral reflux or a mega-ureter. *The follow up of a MCDK is based on: physical examination, kidney ultrasonography in the first years, renal and urinary analysis every 5 years, and elementary nephro-protection measures.  Surgery can be indicated : * Symptomatic forms ( compression) * Occurrence of complications ( pain, hypertension, infection) * Diagnostic dubiety * The discovery of a controlateral uropathy threatening the functional kidney. * The increase of the size of the kidney or the absence of regression at the kidney ultrasonography. DISCUSSION(2/4) Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

14. Antenatal diagnosed multicyctic dysplastic kidney Unilateral form Bilateral form (non viable) -searching for associated malformations - Karyotype -therapeutic interruption of pregnancy Presence of associated malformations Absence of associated malformations Depending on : -Karyotype, types of malformations Regular follow up of the pregnancy post-natal exploration Discussing a potential therapeutic interruption of pregnancy DISCUSSION(4/4) Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

15. Unilateral Multicyctic dysplastic kidney (post-natal therapeutic attitude) Patent : -symptomatic forms -Diagnostic dubiety clinical and ultrasonographic follow up -no size decrease -occurrence of complications : hypertension +++ Discussing surgery Latent surgery - Size decrease - absence of complications -Therapeutic abstention - Ultrasonographic follow up untill involution - Life clinical and biological follow up: Blood pressure per year/ micoalbuminuria and creatinine each 5 years/ urine culture if urinary infection is suspected Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney

16. Prenatal diagnosis of multicyctic dysplastic kidney (MCDK)+++ Guiding the postnatal management of this disease. Conservative treatment is recommended. The therapeutic attitude and the long term prognosis depend on the different evolutive modes and associated malformative uropathy. It is important then to search for these malformative uropathies and to guaranty a life regular follow up. Perinatal Diagnosed Unilateral Multicyctic Dysplastic kidney CONCLUSION