A 6-hour-old baby diagnosed with caudal duplication syndrome presenting with a double penis, two scrotum, two urethra, and two natal clefts
A mother delivered a male neonate 6 hours earlier. The baby weighed 2.6 kg. The mother had an uneventful prenatal course with no history of prenatal exposure. The baby was shifted to the neonatal intensive care unit. On examination, he had a double penis (diphalus), double scrotum, and dual urethral opening.
The baby was urinating normally from both the urethrae. The double scrotum contained one palpable testis in each. Examination also revealed two separated natal clefts. However, there was no anal orifice (imperforate anus).
The doctors made a working diagnosis of Caudal Duplication Syndrome (CDS) with anorectal malformation.
The Invertogram showed a high variety of anorectal malformation. Moreover, ultrasonogram revealed hydronephrosis of the left kidney. Echocardiography was normal and did not reveal any cardiac abnormalities.
With consent, the baby underwent a low descending colostomy that revealed a duplicated colon (4 stomas). Postoperatively, the baby was able to tolerate oral feed and to pass regularly from the colostomy (The two proximal ends).
Distal Loopgram showed duplicated colon down to the rectum. It did not reveal any fistulae with the urinary tracts.
Magnetic Resonance Imaging (MRI) showed:
- Left moderate hydronephrosis.
- Left congenital PUJ (pelvi-ureteric junction) stenosis.
- Duplicated urinary bladder.
- Tethered cord with no duplication of the vertebral column.
The doctors discharged the patient 3 days postoperatively for follow up and subsequent planning for a staged repair.
Maher A, Sabra TA, Ibrahim H, Mostafa M, Newborn with complete double penis and two separate scrotums: Int Journal of Surgery Case Reports; 2020; 77:490-497, ISSN 2210-2612, https://doi.org/10.1016/j.ijscr.2020.11.002.