1. Multicystic Dysplastic Kidney Ayman Abou Mehrem, MD, CABP Neonatology Fellow September 28, 2010

14. Case presentation

24. Case presentation

25. Case presentation

33. Embryology Wein: Campbell-Walsh Urology, 9 th ed., 2007 Saunders, Chapter 114 – Renal Dysgenesis and Cystic Disease of the Kidney

34. Embryology Wein: Campbell-Walsh Urology, 9 th ed., 2007 Saunders, Chapter 114 – Renal Dysgenesis and Cystic Disease of the Kidney

37. Etiology Normal (A) and dysplastic kidney development (B) Winyard P, Chitty LS. Dysplastic kidneys. Semin Fetal Neonatal Med. 2008 Jun;13(3):142-51.

41. Avner, Ellis D.; Harmon, William E.; Niaudet, Patrick, Pediatric Nephrology, 5th Edition, 2004 Lippincott Williams & Wilkins, Chapter 6 – Syndromes and Malformations of the Urinary Tract. Potter (oligohydramnios) sequence Prune-belly syndrome Renal adysplasia Roberts syndrome Rokitansky-Mayer-Kuster-Hauser syndrome Rubella syndrome, congenital Short rib-polydactyly syndrome Smith-Lemli-Opitz syndrome Thalidomide embryopathy Trisomy 8, 9, 13, 18, 21, and 22 Tuberous sclerosis VATER (VACTERL) association von Hippel Lindau disease Zellweger and pseudo-Zellweger syndromes Fryns syndrome Glutaric aciduria, type II Goldenhar syndrome Hajdu-Cheney syndrome Ivemark syndrome Jeune syndrome Joubert syndrome Kaufman-McKusick syndrome Lenz microphthalmia syndrome Leprechaunism (Donohue) syndrome Limb body wall complex Marden-Walker syndrome Marfan syndrome Marshall-Smith syndrome Meckel-Gruber syndrome MURCS association Noonan syndrome OEIS complex Oral-facial-digital syndrome, types I and VI Pallister-Hall syndrome Pallister-Killian syndrome Acro-renal-mandibular syndrome Alagille syndrome (arteriohepatic dysplasia) Baller-Gerold syndrome Bardet-Biedl syndrome Beckwith-Wiedemann syndrome Branchio-oto-renal syndrome Campomelic dysplasia Carbohydrate deficient glycoprotein syndrome CHARGE association Chondrodysplasia punctata, nonrhizomelic Cloacal exstrophy Cornelia de Lange syndrome Diabetic mother, infant of Ectrodactyly-ectodermal dysplasia-clefting syndrome Fanconi anemia syndrome Fetal alcohol syndrome Fraser (cryptophthalmos) syndrome Syndromes Associated with Renal Dysplasia/Cystic Kidney

43. Pathology A typical multicystic kidney having the appearance of a bunch of grapes Wein: Campbell-Walsh Urology, 9 th ed., 2007 Saunders, Chapter 114 – Renal Dysgenesis and Cystic Disease of the Kidney

44. Pathology Nonfunctioning solid cystic dysplastic kidney Wein: Campbell-Walsh Urology, 9 th ed., 2007 Saunders, Chapter 114 – Renal Dysgenesis and Cystic Disease of the Kidney

45. Pathology Wein: Campbell-Walsh Urology, 9 th ed., 2007 Saunders, Chapter 114 – Renal Dysgenesis and Cystic Disease of the Kidney Hydronephrotic form of multicystic kidney

59. Postnatal diagnosis Wiener, Multicystic Dysplastic Kidney Imaging. Medscape 2010

72. Case presentation

73. Case presentation

74. Case presentation

75. Case presentation * * * Plt transfusion

76. Case presentation

78. Diagnosis ? Syndrome ?

80. THANK YOU