Posterior Urethral Valves Leading to Massive Hydronephrosis in A Four-Day Old Baby

Clinical Images | DOI: https://doi.org/10.31579/2768-2757/103

Posterior Urethral Valves Leading to Massive Hydronephrosis in A Four-Day Old Baby

  • Sourya Acharya 1
  • Samarth Shukla 2
  • Raju Shinde 3
  • Tapesh Dutt Nagaria 4*

1 PGY2 Resident, Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences University (Deemed University), Wardha, Maharashtra, India.

2 Professor, Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences University (Deemed University), Wardha, Maharashtra, India.

3 Professor, Department of Pathology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences University (Deemed University), Wardha, Maharashtra, India.

4 Professor, Department of Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences University (Deemed University), Wardha, Maharashtra, India.

*Corresponding Author: Tapesh Dutt Nagaria, PGY2 Resident, Department of General Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences University (Deemed University), Wardha, Maharashtra, India.

Citation: Acharya S., Shukla S., Shinde R., Tapesh D Nagaria., (2024), Posterior Urethral Valves Leading to Massive Hydronephrosis in A Four-Day Old Babys, Journal of Clinical Surgery and Research, 5(1); DOI:10.31579/2768-2757/103

Copyright: © 2024, Tapesh Dutt Nagaria. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: 03 November 2023 | Accepted: 08 December 2023 | Published: 15 January 2024

Keywords: congenital disorder; diagnosis; cystourethrogram

Abstract

Posterior urethral valves (P.U.V.s), also referred to as congenital obstructing posterior urethral membranes (C.O.P.U.M.) is a congenital malformation affecting males where the membranous folds of the urogenital membrane obstruct the membranous and prostatic urethra. It is a congenital disorder only seen in males, with an incidence of 1 per 4000 to 1 per 25000 live births in some areas. Type 1 P.U.V. results from the abnormal insertion and absorption of the distal Wolffian duct during the development of the bladder. Type 3 PUV is observed as a membrane in the posterior urethra, originating from the incomplete canalisation between the anterior and the posterior urethra. Type 2 P.U.V. is now considered a normal anatomical variant. Regardless of the type, P.U.V.s present with urinary tract obstruction, bladder distension, abdominal distension, difficulty voiding, poor stream of urine, recurrent Urinary Tract Infection (U.T.I.), diurnal enuresis and a failure to thrive. They are diagnosed Antenatally by Ultrasonography showing distended or thick-walled bladder, bilateral hydroureters, and bilateral hydronephrosis. However, the gold standard for the diagnosis is a Micturating  CystoUrethrogram (MCU), also known as a Voiding Cystourethrogram (V.C.U.) shows dilation/elongation of the posterior urethra during voiding along with signs of vesicoureteral reflux if present. This patient was antenatally diagnosed with posterior urethral valves at seven months of fetal age and came to us for further management. Antenatal diagnosis of Posterior Urethral Valves on Ultrasonogram involves visualisation of 1. Keyhole sign- due to the distension of the urethral proximal to the valves and a distended bladder, 2. marked distention or hypertrophy of the bladder, 3. Hydronephrosis and hydroureter.  An Ultrasonogram of the abdomen and pelvis done postnatally revealed bilateral gross hydroureteronephrosis, which was missed on antenatal scans. The baby was born to a G2P1L1 mother and weighed 3.04kg at birth. The baby cried immediately after birth and maintained oxygen saturation at 100% on room air. The patient was managed with Cystoscopy with Posterior Urethral Valve fulguration with circumcision.

Images

Figure 1: a Micturating Urethrogram showing bilateral grossly dilated ureter and renal pelvis along with posterior urethral valve as a narrowing in the posterior urethra.

Figure 2: A Micturating Urethrogram - Lateral view - showing bilateral grossly dilated ureter and renal pelvis along with posterior urethral valve as a narrowing in the posterior urethra.

                                                                          Figure 3: A Micturating Urethrogram - showing filled-up urinary bladder.

Figure 4: A Micturating Urethrogram - Lateral view - showing bilateral grossly dilated ureter and renal pelvis along with posterior urethral valve as a narrowing in the posterior urethra.

Final Diagnosis

Bilateral Grade V Hydronephrosis with Posterior Urethral Valves

Differential Diagnosis-

Urethral Stricture

References

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