Table 3.
Natural history numerical data collected on bilateral renal agenesis patients.
| First author (year) | Study characteristics | Isolated vs. multiple | Livebirth | Stillbirth/abortion/TOP | Age at death |
|---|---|---|---|---|---|
| Slickers (2008) [11] | To explore potential relations between renal anomaly and maternal BMI, smoking, alcohol, and caffeine exposures Infants with BRA or hypoplasia n = 80 cases; 71 isolated BRA; 5 BRH,4 cases of 1 hypoplastic kidney and one absent kidney; 5 cases excluded for maternal reasons – 75 total | Not reported | n = 49/75 (65%) | n = 26/75 (35%) stillbirth/induced abortion | n = 3 (4%) Survival at interview = 10.6 months mean |
| Davis (2010) [12] | Investigating maternal diabetes and link to RA n = 89 deliveries n = 22 BRA; 59 unilateral; eight unclear laterality | Not reported | 83/89 | n = 6 fetal deaths/89 | |
| Kumar (2014) [25] | Identify renal anomalies associated with poor outcome n = 587 total babies noted w anomaly n = 136 with renal anomalies n = ll with absent kidney reported in text n = 8 with renal agenesis (from Table 2 - unspecified unilateral vs. bilateral) | n = 43 with multiple anomalies from total (not delineated for RA) | n = 64 liveborn babies with renal anomalies Of 8 with RA, n = 0 liveborn | Of all renal anomalies: n = 12T0P n = 60 stillborn Of eight infants with RA: n = 5TOP;n = 3IUFD | |
| Kumar (2013) [13] | To follow antenatal and postnatal course of pregnancies with anomalies to understand prognostication and improve management n = 523 eligible pregnancies with follow up n = 4 bilateral renal agenesis dx< 20 weeks' gestation n = 8 bilateral renal agenesis dx> 20 weeks' gestation | Not reported | n = 0 | n = 12 | |
| Nagase (201l) [23] | Analyzed prognosis of OS cases to clarify differences according to delivery method n = 47 cases with OS Excluded pre-viable cases, n = 32 cases of OS Two cases not prenatally diagnosed (emergency transfer cases) | Of 32 cases with OS, n = 19 with associated anomalies | n = 25 liveborn Of these: n = 5 of labor induction n = 17 for natural labor group n = 2 unidentified prenatally | TOP before 22nd week (previa ble) = 15 IUFD c-section n = 2 Induced labor group n = 4 for IUFD Natural labor group n = l IUFD | Labor induced – 5 born alive, alive an average 54 min Natural labor group n = 17 born alive, alive average of 5 h 41 min |
| Wang (2011) [14] | Conduct 25-year survival analysis of children with defects n = 57,002 children w birth defects from ∼6 million live births n = 1946 cases of RA or dysgenesis | Not reported | n = 1946 liveborn | n = 693 deaths 7 days survival P: 71 1 month survival P: 69 1 year survival P: 66 5 year survival P: 64.8 | |
| Ulkumen (2015) [22] | To evaluate outcomes in pregnancy with early onset OH n = 54 pregnancies w oligo or anhydramnios Of these, n = 5 bilateral renal agenesis | Thirteen of total 54 weeks/associated anomalies | n = 39/54TOP | Not reported | |
| Game (2005) [24] | To assess prenatal detection of renal anomalies n = 257 cases of b/l renal agenesis n = 201 with prenatal diagnosis | n = 17 chromosomal associations | n = 201 Prenatal diagnosis n = 158/257 TOP (61%) | ||
| Stojanovic(201l)[15] | To identify prevalence and outcome at 1 year of life for severe renal anomalies n = 985 cases with congenital renal anomaly 1989–2009 Of those, n = 230 with severe renal congenital anomalies n = 85 with BRA | n = 20 liveborn | Two spontaneous abortions 4 still births 59 TOP | Early neonatal death-19 0 survival at 1 year of age | |
| Mehler (201l) [17] | Evaluate prenatal/perinatal variables to predict survival and outcomes for the ROH population n = 36 liveborn infants (30 M, 6 F) with ROH 10 weeks renal dysplasia/agenesis (n = 2 b/l agenesis; n = 6 b/l dysplasia; n = 2 unilateral renal agenesis + contralateral multicystic dysplasia) | n = l of BRA with MMC | Total = 36 n = 2 BRA liveborn | Not reported | 7/36 died within 48 h – rep failure Of 2 BRA: Died Dl; died D5 |
| Melo (2012) [18] | Studying mortality in 524 newborns with renal and urinary tract anomalies between 1996 and 2006 n = 24 RA/dysplasia; of these five were unilateral (left) | n = 13 multiple malformations n = 3 w/other urinary anomalies n = 2 genetic (Fraser, limb defect) | Thirteen with multiple malformations died in neonatal period (54% mortality) | ||
| Wiesel (2005) [4] | To evaluate prevalence of prenatal ultrasound diagnoses for renal malformations in 20 registries n = 1130 infants with renal malformation diagnosis n = 95 with bilateral renal agenesis or dysgenesis | n = 8 multiple malformations. (5/8 TOP) n = 7 chromosomal syndrome (7/7TOP) n = 7 nonchromal syndrome (5/7 TOP) | n = 12 livebirth | In total: n = 58/95 TOP n = 24/95 stillborn | |
| Damen-Elias (2005) [21] | To determine long-term prognosis of antenatally detected renal anomalies to improve counseling Congenital renal and urinary anomalies = 402 Structural kidney disease n = 151 n = 33 BRA | n = 10 with additional malformations (six extra-renal) | n = 15 liveborn | n = 33 deaths (27% mortality of 121 deaths) Of the 33 deaths: n = 16TOP n = 2 stillbirths | n = 15 neonatal deaths (< 28 days) |
| Grijseels (201l) [19] | Evaluate outcomes of fetuses weeks/OH from renal cause n = 71 pregnancies evaluated n = 36 diagnosis renal dysplasia – (agenesis–cystic kidneys) 15 with agenesis; 12 b/l cystic, 4 combo Of 15 with agenesis 10 were bilateral | 23/71 total had associated anomalies 12/15 with agenesis had associated anomalies | 25/71 total were liveborn n = 6/15 with agenesis were liveborn | n = 29/36 w/dysplasia TOP n = l IUFD n = 5 neonatal death | Postnatal course of liveborn: 3 died 1st day (no resuscitation), 2 died >1 day; 3 infants required MV n = l living patient |
| Robbins (2007) [16] | Investigating mortality, hospital length of stay and charges related to birth defects Total n = 60,952 infants n = 1259 hospitalizations related to renal agenesis | Not reported | Not reported | n = 344 in hospital death (27.3%) | Not reported |
| Spiro (2015) [20] | Evaluate outcomes of pts with ROH n = 40,621 pregnancies n = 42 with renal agenesis, 41 BRA n = 16 prenatal intervention (not specified which but chorionic villous sampling, amniocentesis, or amnioinfusion) | 21/42 associated with anomalies Of 30 BRA nonsurvivors: 16 with associated anomalies | N=ll/42 livebirth (n = 2 prenatal intervention) (n = l unilateral agenesis) | Of 31 nonsurvivors with BRA: 6 IUFD; 25 abortion 8 neonatal deaths (n = 14 prenatal interventions) | One postnatal follow-up – unilateral renal agenesis |
b/l= Bilateral, BRA= bilateral renal agenesis, BRH = bilateral renal hypoplasia, MV= mechanical ventilation, OH = oligohydramnios, OS = oligohydramnios sequence, Pt or Pts = patient or patients, RA = renal agenesis, ROH = renal oligohydramnios.