renal hypoplasia
description
renal hypoplasia signifies small kidneys or kidneys with a reduction in the number of nephrons. it may be segmental, unilateral or bilateral. unilateral hypoplasia is associated with hypertension in the first decade of life. bilateral hypoplasia is associated with chronic renal failure and end stage disease during the first decade of life. polyuria and polydypsia are common symptoms. a rare bilateral form called oligomeganephronia is associated with a reduced number of markedly hypertrophied nephrons. at about 5 weeks after conception and throughout gestation until 32 to 36 weeks the ampulla or actively growing part of the ureteral bud undergoes multiple divisions and induces the development of nephrons and establishes communication with them. the metanephric blastema gives origin to the nephrons. differentiation, elongation and maturation of the components of the nephron (glomeruli, proximal and distal convoluted tubules and henle’s loop) continue even after birth. renal hypoplasia can result from any insult that interferes with this process. it has also been shown to result from acquired cortical loss in children with vesicoureteral reflux (vur). back pressure of urine exerted through later generations of nephrons (which are attached in direct alignment with collecting tubules unlike earlier nephrons attached at acute angles) can become distended into cysts and thus halt further nephron induction.
diagnosis
segmental renal hypoplasia may be suspected by asymmetry of one kidney (abnormal kidney shape using renal pelvis as a reference point). unilateral hypoplasia may present with obvious differences in size of the two kidneys. renal biometry can be used to document small kidney size, unilaterally or bilaterally. renal length is measured from upper to lower renal pole in longitudinal section parallel to the long axis of the aorta. thickness, width and perimeter are measured in transverse section at the level of the renal pelvis or at the largest transverse section. respiratory movements can help define cleavage planes between kidney and surrounding structures. measurements of kidney perimeter/abdominal perimeter can also be helpful. unilateral or bilateral cysts formed as a result of back pressure can also be associated with renal dysplasia or hypoplasia. with unilateral hypoplasia, contralateral renal anomalies, including renal agenesis, should be excluded. genital and other organ system malformations can also be associated findings.
differential diagnosis
if oligohydramnios is present, a single hypoplastic kidney may be missed but the prognosis most likely would be the same as for bilateral renal agenesis. low contrast between the renal parenchyma and adrenal glands, spleen, liver and bowel may influence accuracy of measurements. movement with respiration can help define cleavage planes. normal size or large kidneys related to other malformations (e.g. vur) can be microscopically and functionally hypoplastic postnatally.
sonographic features
small to normal size kidney(s) below 5th centile for gestational age (biometry available for length, width, thickness, volume)
can be below 5th centile for kidney / abdominal perimeter ratio based on gestational age
other renal malformations may include unilateral renal agenesis, duplication defects, dysplasia, vesicoureteral reflux (vur)
if cortical loss is secondary to other renal malformations or reflux, the kidneys may be enlarged prenatally
amniotic fluid volume usually normal but may be increased or decreased
genital and other organ system malformations may be present
associated syndromes
- 13 ring
- 49 – xxxxx
- 4p-
- 4q – partial duplication
- acro-dysgenital
- alagille (arteriohepatic dysplasia)
- brachmann de-lange
- cerebro-ocular-facial-skeletal
- cerebro-renal-digital bendon type
- neural tube defects
- oculo-renal
- otocephaly
- potter’s
- renal adysplasia (hereditary renal adysplasia, hra familial bilateral renal agenesis)
- renal-genital-ear
- rokitansky (von mayer-rokitansky-kiister, mrk anomaly)
- sirenomelia
- townes-brocks
- twin-reversed arterial perfusion (trap)
- type i or saldino-noonan
- urorectal septum malformation
- vater
- vitamin a congeners
references
berhman re in: nelson textbook of pediatrics w.b. saunders co: philadelphia/london/toronto, p1350-51
nyberg d, mahony b, pretorius d in: diagnostic ultrasound of fetal anomalies vol ii mosby year book: st. louis, p433, p437
sheih cp, lu wt, liao yj, liang ww, li yh renal hypoplasia, gartner’s duct cyst and imperforated hemivagina: report of a case j formos med assoc 93:531-3
wax jr, prabhakar g, giraldez ra, hutchins gm, stetten g, blakemore kj unilateral renal hypoplasia and contralateral renal agenesis: a new association with 45,x/46,xy mosaicism am j perinatol 11:184-6
romero r, pilu g, jeanty p, ghidini a. hobbins j in: prenatal diagnosis of congenital anomalies appleton & lange: norwalk, p255-59
fleischer r, hall t, goodman r in: human malformations and related anomalies, vol ii oxford u press: oxford, new york, p262