Renal agenesis in a child with ipsilateral hemihypertrophy.
Hemihypertrophy is a clinical condition defined as an asymmetric enlargement of one side of the body. Inguinal hernias, renal cysts, cryptorchidism, ectasias of collecting tubes, medullary sponge kidney and horse-shoe kidney are examples of abnormalities associated with hemihypertrophy that have been described in the literature. We report here a 17.2-year-old patient with a left hemihypertrophy and renal agenesis with contralateral right compensatory renal hypertrophy together with normal renal function and blood pressure in the absence of proteinuria. He also presented with faint macular hyperpigmented skin lesions on his left upper arm, forehead and abdomen. To the best of our knowledge, this is the first report of renal agenesis and hemihypertrophy in the same patient. Hemihypertrophy can be seen as a component of Klippel-Trenaunay-Weber syndrome. The other components of this syndrome are varicose veins, skin naevus and arteriovenous malformations. Although renal agenesis, hemihypertrophy and hyperpigmented macular skin lesions (skin naevuses) may be incidental findings, together they may form a variant of Klippel-Trenaunay-Weber syndrome, as in our case.
Oren
Nisa Cem
NC
Department of Radiology, GATA Medical Faculty, Ankara, Turkey. drcemoren@hotmail.com
Vurucu
Sebahattin
S
Karaman
Bülent
B
Ors
Fatih
F
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000293:Adolescent; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis*; D008297:Male; D009506:Nevus / Q000175:diagnosis; D012878:Skin Neoplasms / Q000175:diagnosis
Clin Orthop Relat Res. 1979 Oct;(144):198-211
119599
Br J Radiol. 1988 Sep;61(729):851-3
3052686
J Bone Joint Surg Am. 1997 Nov;79(11):1731-8
9384434
Am J Roentgenol Radium Ther. 1946 Nov;56(5):616-23
20275162
Best Pract Res Clin Endocrinol Metab. 2008 Jun;22(3):403-14
18538282
J Genet. 2009 Aug;88(2):239-43
19700863
Pediatrics. 1965 Sep;36(3):434-48
5318604
Radiology. 2002 Aug;224(2):375-8
12147831
Arch Dis Child. 2006 Dec;91(12):995-9
16857697
Hematol Oncol Clin North Am. 1995 Dec;9(6):1253-65
8591963
J Med Genet. 1999 Nov;36(11):837-42
10544228
Birth Defects Orig Artic Ser. 1979;15(5B):291-6
118782
Adv Hum Genet. 1989;18:181-303, 373-6
2658495
J Genet Hum. 1964 Sep;13:223-32
14231762
AJR Am J Roentgenol. 1996 Oct;167(4):989-95
8819399
Radiology. 2008 Mar;246(3):974-9
18309020
J Bone Joint Surg Am. 1964 Sep;46:1197-202
14214353
Eur J Pediatr. 1995 Dec;154(12):958-68
8801103