Multicystic kidney dysplasia

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Classification according to ICD-10
Q61.4 Kidney Dysplasia - Multicystic
ICD-10 online (WHO version 2019)

The Multicystic renal dysplasia (MCDK) is a spontaneously occurring, usually unilateral occurring congenital developmental disorder ( malformation ) of a kidney with multiple large cysts instead of the parenchyma and complete loss of function. Often no pelvic calyx system is detectable.

Synonyms are: aplastic cystic kidney; Multicystic renal dysplasia; Renal dysplasia, diffuse; MDK; RMD; Renal multicystic dysplasia ( English multicystic dysplastic kidney; MCDK )

classification

Formally , a distinction can be made

  • Unilateral MCDK
  • Bilateral MCDK, incompatible with life, without dialysis Death usually within 8 weeks

According to the American Academy of Pediatrics - classification of cystic kidney diseases, multicystic kidney dysplasia is classified as a non-genetic or non-hereditary disease.

pathology

The formation of cysts is based on a disturbed interaction between ureter and kidney anlage, so that the nephrons are not properly formed. The healthy opposite side hypertrophies compensatory .

distribution

The frequency of the one-sided form is given as 1 in 4,300 live births .

The malformation usually occurs sporadically, but there are also more frequent familial occurrences with autosomal dominant inheritance.

Multicystic kidney dysplasia is the most common abdominal tumor in the newborn . In almost 40% there are also other malformations such as ureteral stenosis in 3–12%, vesicorenal reflux in 5–20%, or benign cystic dysplasia of the testicle .

root cause

The cause lies in a complete obstruction of the ureter in the 8th – 10th centuries . Week of pregnancy . If the transfer takes place later, a mixed form of multicystic-dysplastic kidney develops , followed by severe subpelvic stenosis (ureteral stenosis) even later. The reason for this inhibited formation of the ureteral bud and the metanephrogenic tissue is unclear. Viral infections are discussed.

As with some syndromes with renal dysplasia can mutations on EYA1 - SIX1 - PAX2 - or HNF1B - gene , gestational diabetes or some medications during pregnancy, especially antiepileptics , play a role.

In the context of syndromes

MCKD is found in some syndromes:

Clinical manifestations

Clinical criteria are:

The malformation is usually symptom-free and is often diagnosed intrauterine during a routine examination around the 20th week or later as part of routine diagnostics, e.g. B. discovered in a urinary tract infection by sonography . The contralateral kidney is already enlarged to compensate for a quarter to a half at birth, in most cases the compensatory increase in size occurs during the first years of life.

In rare cases, renal hypertension , proteinuria, or even kidney failure can develop. If there is a birth with bilateral MCDK, typical signs of the oligohydramnios sequence are found .

diagnosis

The diagnosis is made by sonographic evidence of a cystic, chambered tumor in the kidney box.

A micturition cystourethrogram is indicated to differentiate massive hydronephrosis and to rule out vesicoureteral reflux . The lack of function can be confirmed in kidney scintigraphy , magnetic resonance imaging or computed tomography with administration of contrast medium .

Differential diagnosis

To be differentiated are the medullary sponge kidney , multilocular cystic nephroma and massive hydronephrosis .

therapy

In the case of unilateral dysplasia, no treatment is usually required. Regular checks for any urinary tract infections, development of renal insufficiency or renal anemia are indicated.

The nephrectomy , which has long been recommended because of the risk of hypertension, tumor formation and trauma, is only performed if there is a suspicion of cystic Wilms' tumor, if it occupies space, has increased size or if hypertension is manifest.

Repeated amniotic fluid replenishment may be an option during pregnancy with bilateral MCDK.

Prospect of healing

The prognosis is good, most kidneys shrink within the first few years of life, complications are rare, so there is no general indication for surgery.

Web links

Individual evidence

  1. ↑ a b c d e f g Urology textbook
  2. ↑ a b c d kidney dysplasia, multicystic. In: Orphanet (Rare Disease Database).
  3. ↑ a b c d Medscape Multicystic Renal Dysplasia
  4. ↑ FC Sitzmann: Malformations of the kidneys and lower urinary tract. In: Dual Series: Pediatrics . Springer, 2007, p. 412 ff., ISBN 978-3-13-125333-0 / Online ISBN 978-3-13-190563-5 , doi: 10.1055 / b-0034-10819
  5. ↑ Renal dysplasia, multicystic, unilateral form. In: Orphanet (Rare Disease Database).
  6. ↑ Renal dysplasia, multicystic, bilateral form. In: Orphanet (Rare Disease Database).
  7. ↑ a b c A. Sigel, R.-H. Ringert (Ed.): Pediatric urology. 2nd edition, Springer, Berlin 2001, ISBN 3-662-08081-8 (print) / ISBN 978-3-662-08080-1 (e-book)
  8. Jump up ↑ KI Glassberg, FD Stephens, RL Lebowitz, V. Braren, JW Duckett, EC Jacobs, LR King, AD Perlmutter: Renal dysgenesis and cystic disease of the kidney: a report of the Committee on Terminology, Nomenclature and Classification, Section on Urology , American Academy of Pediatrics. In: The Journal of Urology . Volume 138, No. 4 Pt 2, October 1987, pp. 1085-1092, PMID 3309374 (review).
  9. ↑ a b c Entry on Multizystische_Nierendysplasie in Flexikon , a Wiki of the DocCheck company
  10. ↑ a b c d e W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 . Vol. II
  11. ↑ KO Kagan, M Schmidt, U Kuhn, R Kimmig: Recurrent amniotic fluid fillings for prolongation of pregnancy in bilateral multicystic kidneys In: Zentralblatt Gynäkologie , Vol. 126, 2004, Congress Abstract, P1_2, doi: 10.1055 / s-2004-829794
  12. ↑ F. Kirchmair, J. Gellermann, KS Heling, C. Tennstedt: The unilateral multicystic renal dysplasia (MCDK) - long-term course after prenatal diagnosis . In: Perspectives of Surgery in the 21st Century, 117th Congress of the German Society for Surgery, 2. – 6. May 2000, Berlin , vol. 2000, p. 545, doi: 10.1007 / 978-3-642-59573-8_209 , ISBN 978-3-540-41127-7