URETHRAL ATRESIA

MURCS ASSOCIATION

MURCS association is a rare, lethal constellation of unusual non-random findings that include (1-2):

1. Mullerian duct aplasia.
2. Renal aplasia.
3. Cervicothoracic somite dysplasia.

PATHOGENESIS

Link to Pathogenesis

ULTRASOUND (1-4)

• Female fetus.
• Massive cystic umbilical cord (which may involve the entire cord) due to a patent urachus.
• Enlarged bladder (which does not appear to change during the scan suggesting urethral obstruction).
• Bladder opened through the anterior abdominal wall via a patent urachus.
• Single kidney or severely dysplastic kidney (2).
• Urethral obstruction / atresia (2).
• Oligohydramnios.
• Uterus and fallopian tubes may be normal, hypoplastic or atretic.
• Vertebral anomalies e.g. hemivertebrae.
• Occipital encephalocele.

Severe urethral stenosis Female fetus. Megacystis. Megaureter. Renal obstruction. Oligohydramnios. “Prune-belly” secondary to urinary obstruction.

DIFFERENTIAL DIAGNOSIS

• VACTERL association.
• Klippel-Feil phenotype (vertebral segmentation or fusion defects, cardiac defects, deafness, cleft palate, renal dysplasia). Mullerian female reproductive tract anomalies are an important feature of MURCS and are not seen in the other two conditions.
• Goldenhar syndrome.

VACTERL versus MURCS association
	VACTERL	MURCS
Mullerian duct failure	-	+
Vertebral defects	+	+
Limb abnormalities	+	+
Anorectal anomalies	+	-
Renal defects	+	+
Cardiovascular anomalies	+	-
Tracheo-esophageal anomalies	+	-

REFERENCES

1. Duncan PA, Shapiro LR, Stangell J et.al. The MURCS association: Mullerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr 1979;95:399.
2. Fernandez CO, McFarland RD, Timmons C et.al. MURCS association: Ultrasonographic findings and pathologic correlation. J Ultrasound Med 1996;15:867-870.
3. Colavita N, Orazi C, Logroscino C et.al. Does MURCS association represent an actual non-random complex of malformations? Diagn Imaging Clin Med 1986;55:172.
4. Greene RA, Bloch MJ, Huff DS et.al. MURCS association with additional anomalies. Hum Pathol 1986;17:88.