**6. Congenital anomalies of the kidney and urinary tract**

#### **6.1. Pregnancy management**

should be clinically apparent and recognizable at birth in the delivery room [81]. If a prenatal diagnosis is not made, the diagnosis of bladder exstrophy should be clinically recognizable at delivery. A careful physical examination will differentiate bladder exstrophy from other congenital anomalies that involve abdominal wall defects, such as omphalocele, gastroschisis,

Following the prenatal diagnosis of bladder exstrophy, prenatal care includes the following

**1.** Education and counseling of the parents, touring the neonatal intensive care unit, meeting the pediatric urologic care team, and allowing the expectant parent(s) the opportunity to

**2.** Preparation for delivery. In many tertiary centers, one option for planning initial surgical management is an induced vaginal delivery that is scheduled in late gestation with coordination with an on-site pediatric urology service. This approach facilitates bladder closure

• Body stalk anomaly (also called limb-body wall complex) is a massively disfiguring and generally lethal malformation of the thorax and/or abdomen, often associated with limb defects. The intrathoracic and abdominal organs lie outside the abdominal cavity and are contained within a sac composed of amnioperitoneal membrane attached directly to the placenta. The umbilical cord may be totally absent or extremely shortened. Severe kyphoscoliosis is often present. Termination of pregnancy is usually offered since the abnormality is generally considered lethal [82]. However, repair has been performed in rare cases [82]. If the pregnancy is continued, vaginal delivery is recommended given the highly lethal nature of this disease, assuming there are no maternal contraindications to vaginal delivery. In this setting, the patient should be extensively counseled on the likelihood of neonatal demise, as well as the severe morbidity associated with a successful

• Cloacal exstrophy. The accuracy of sonographic diagnosis appears to be less than 25% due to the rarity of the disorder and the wide spectrum of anatomic variants, which depend upon the degree of cloacal septation completed. Fetal genetic studies during the initial evaluation can be useful, although cloacal exstrophy has not been reported to be associated with specific aneuploidies. The chromosomal findings, which will include gender, may influence the decision to terminate the pregnancy, perform a cesarean delivery for fetal indications, or initiate a series of corrective operations in the newborn period [41]. Although there are no studies of the optimum route of delivery for this rare disorder, cesarean delivery is generally reserved for standard obstetric indications. The umbilical cord should be clamped or ligated carefully to avoid injury to proximate structures. At delivery,

interact with other families with a child with bladder exstrophy.

The cesarean delivery should be reserved for obstetrical complications.

and cloacal exstrophy.

492 Congenital Anomalies - From the Embryo to the Neonate

within 72 hours of life.

repair [82, 83].

**5.4. Body stalk anomaly and cloacal exstrophy**

[16, 81]:

Congenital anomalies of the kidney and urinary tract (CAKUT) constitute approximately 20–30% of all anomalies identified in the prenatal period [84]. Defects can be bilateral or unilateral, and different defects often coexist in an individual child. In general, the optimal timing recommended for a screening antenatal ultrasound is between 16 and 20 weeks of gestation because of the following factors at this gestational age. Counseling of families with fetuses with CAKUT should be universally available. If the fetal prognosis is poor, as determined by severe bilateral disease, bilateral RA, oligohydramnios, or unfavorable amniotic fluid analysis, legal termination, if possible, can be offered. In all other cases, continued counseling throughout the pregnancy including discussion of postnatal management is required. In particular, discussion with parents regarding their wishes on the level of support given to offspring with severe oligohydramnios, who are at risk for lung hypoplasia that may be incompatible with life, is helpful in establishing guidelines for initial postnatal care [84].


Because amniotic fluid is predominantly composed of fetal urine, measurement of biochemical markers contained in amniotic fluid (fetal urine) can be used to assess fetal renal function [84].

• Indications for magnetic resonance imaging. MRI may be used to confirm or clarify suspected gastrointestinal abnormalities on ultrasound examination if this information is important for managing the pregnancy. Fetal bowel is well visualized by MRI and easily differentiated from adjacent liver, spleen, kidneys, bladder, and gallbladder. Meconium is also well visualized [87]. The normal esophagus, stomach, and duodenum should always

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• Assessment of additional anomalies and follow-up assessment. Many of these pregnancies are complicated by polyhydramnios. After diagnosis, the performance of periodic ultrasound examinations to look for any change in the appearance of the atresia or associated anomalies and to assess interval fetal growth and amniotic fluid volume is recommended. Nonstress tests or biophysical profiles are indicated in pregnancies in which the risk of antepartum fetal demise is increased, such as a fetal anomaly associated with growth re-

Atresia alone is not an indication for cesarean delivery in the absence of a standard obstetric indication. However, if the abdominal circumference is much larger than the head circumference, cesarean delivery should be considered due to the risk of fetal abdominal dystocia. Delivery should be planned at a center that has an appropriate level of neonatal support.

Vilnius University, Faculty of Medicine, Institute of Clinical medicine, Clinic of Obstetrics

[1] Copel J.Fetal Cardiac Abnormalities: Screening, Evaluation, and Pregnancy Management.

[2] Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A, et al. Diagnosis and treatment of fetal cardiac disease: A scientific statement from the

[3] Song MS, Hu A, Dyamenahalli U, Chitayat D, Winsor EJ, Ryan G, et al. Extracardiac lesions and chromosomal abnormalities associated with major fetal heart defects: Comparison

be filled with T2 hyperintense fluid (amniotic fluid).

Address all correspondence to: diana.ramasauskaite@mf.vu.lt

Aug 2017. Available rom: www.uptodate.com

American Heart Association. Circulation. 2014;**129**(21):2183

striction [86].

**Author details**

**References**

Diana Ramasauskaite

and Gynaecology, Vilnius, Lithuania

**7.2. Delivery management**

