Urachal remnant presenting as a giant, cystic umbilical cord

Excerpt

A male infant was born by planned cesarean section at 36 6/7 weeks for intrauterine cysts diagnosed at 21 weeks gestation that appeared to be enlarging. Prenatal amniocentesis with chromosomal analysis, FISH, and SNPa array results were normal with 46 XY karyotype. Prenatal history and labs were otherwise unremarkable except for maternal iron deficiency anemia. On delivery, the patient was crying vigorously and had Apgars of 9 and 9. A giant umbilical cord with multiple cysts was noted (Fig. 1a). No other gross abnormalities were found on exam and he was transferred to the NICU. On exam, the patient had no appreciable fascial defect at the umbilicus and no external drainage or sinus coming from either the epithelialized portion of the umbilicus or Wharton’s Jelly. The cord clamp was placed beyond the most distal cyst. Three cord vessels were visualized with cord transection. An ultrasound was notable for cystic lesions of the umbilical cord with no obvious connection to the bowel or bladder and with no associated vascular anomalies. This was felt to be consistent with a pseudocyst. Abdominal X-ray showed no intra-abdominal pathology. The patient was observed for 24 h and remained stable on room air. Operative exploration performed on day of life two revealed an epithelial lined structure within Wharton’s Jelly that was separate from the three cord vessels (Fig. 1b). An 8 French red rubber catheter was used to interrogate the sinus tract. The catheter met with resistance just above the level of the fascia. We could not express urine from the sinus, but the tract below the catheter was clearly in continuity with the bladder (Fig. 1c).

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