Coccygeal polypoid eccrine nevus associated with imperforate anus and unilateral multicystic kidney
Multicystic kidney disease (MCKD) is a developmental disorder of the kidney, in which the normal renal parenchyma is replaced by multiple, non-communicating cysts of varying size.
Bilateral multicystic kidney disease occurs in about 10-20% of cases and is a lethal condition.
It is worth noting that the often-quoted rate of vesicoureteral reflux up to 43% from the Multicystic Kidney Registry is prone to selection bias as only 15% of patients in the registry had a voiding cystogram.[sup.30]
Risk of Wilms' tumour with multicystic kidney disease: a systematic review.
Risk of hypertension with multicystic kidney disease: a systematic review.
Risk ofWilms' tumour with multicystic kidney
disease: a systematic review.
In particular, genitourinary malformations such as dysplastic and multicystic kidneys
, hydronephrosis, hydroureter, ureterocele, duplicated renal collecting system, and absent bladder are common as extensive injury and fibrosis of the uroepithelium can lead to obstructive uropathies .
Other conditions that may be associated with DiGeorge are atopic disorders (asthma and eczema) rheumatoid arthritis, autoimmune thyroiditis, renal abnormalities (such as multicystic kidneys
and Vesicoureteral reflux).
Table 2: Type of Anomaly (n=62) Type of Anomaly Frequency Hydrocephalus 16 Anencephaly 14 Meningomyelocele 4 Encephalocele 6 CNS Anomalies, [49 (79%)] Hydroceph+meningomylocele 4 Anenceph+spinabifida 2Isolated Microcephalous 1Anomalies, [57(91.9%)] Acrania 1 Dandy walker malformation 1 Polyscystic kidneys 2 Renal Anomalies, [4 (6.5%)] Dysplastic Multicystic kidneys
2 Omphalocele 1 GIT Anomalies, [2 (3.2%)] Gastroschisis 1 Skeletal Anomalies, [2 (3.2%)] Achondroplasia 2 Mickel Gruber syndrome 2 Hydroceph, Omphalocele 1Complex Anomalies, [5 (8.1%)] Esophageal atresia, Renal anomlies 1 Facial abnormalities, Talipes, Esophageal 1 atresia, Renal agenesis
The findings included large multicystic kidneys
For example, multicystic kidneys
erroneously called MCDKs were proven to be examples of GCKD instead.