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Congenital megalourethra with delayed obstructive symptoms
Journal of Pediatric Surgery Case Reports 47 (2019) 101232
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Congenital megalourethra with delayed obstructive symptoms a,∗
b
c
c
c
Sohum K. Patel , Ross Simon , Diana Cardona-Grau , Mark A. Rich , Hubert S. Swana a b c
T
University of Central Florida College of Medicine, 6850 Lake Nona Blvd, Orlando, FL 32827, United States University of South Florida Morsani College of Medicine, 12901 Bruce B Downs Blvd, Tampa, FL 33612, United States Orlando Health Arnold Palmer Hospital for Children, 92 W Miller St, Orlando, FL 32806, United States
ABSTRACT
Congenital megalourethra is a congenital non-obstructive dilation of the male urethra often associated with VACTERL anomaly malformations. We report a case of TE fistula associated congenital megalourethra with symptoms presenting approximately 2 months postpartum. At the time of referral, patient had distal penile flaccidity and poorly developed corpora. Imaging and cystoscopy showed development of hydronephrosis with significant sediment in the bladder. Attempts at retrograde catheterization failed, thus a Blocksum vesicostomy was required. This demonstrates the importance of serial physical examinations and ultrasound surveillance in patients with megalourethra.
1. Intro Congenital megalourethra is an uncommon dilation of the anterior urethra. We present a case of a 2-month-old boy with prenatally diagnosed megalourethra, initially managed expectantly, who developed complications due to progressive, incomplete bladder emptying. 2. Case report Urology consultation was obtained for a 2-month-old boy who had a history of a “urethral cyst” diagnosed prenatally. He was the product of a 30-week gestation, and at birth found to have esophageal atresia/TE fistula which was repaired shortly after birth. Prenatal ultrasound screening demonstrated a cystic dilation of the urethra (Fig. 1). Amniotic fluid levels were normal and no hydronephrosis was identified. Renal/bladder ultrasonography done shortly after birth as part of VACTERL work-up were unremarkable. Pt underwent repair of tracheoesophageal fistula and was urinating spontaneously. At 31 days of life, in anticipation of transfer to a lower acuity care nursery, urologic consultation was obtained. Physical exam at the time of referral showed descended testicles, a long penis with distal flaccidity and poorly developed distal corporal bodies. Repeat renal/bladder ultrasound revealed new mild hydronephrosis of the left kidney (Fig. 2) and significant debris in the bladder (Fig. 3). Patient became progressively unstable with frequent episodes of apnea. Broad-spectrum antibiotics were administered. Urine analysis was concerning for infection. Retrograde urethral catheterization at the bedside was not successful, however, cystoscopy revealed dilated distal urethra and poorly-formed corporal bodies distally (Fig. 4).
∗
A narrowing of the bulbar urethra was noted. A 0.025 glide wire was able to be passed into the bladder, but larger catheters could not be passed in a retrograde fashion. A Blocksum vesicostomy was created. The patient improved clinically, the hydronephrosis improved and debris cleared. 3. Discussion Congenital megalourethra, first described by Nesbitt, is characterized by dilatation of the anterior urethra [5,6]. Congenital megalourethra is classified into fusiform and scaphoid subtypes, based on urethrography. In the scaphoid subtype, defects in the corpus spongiosum lead to dilation of the ventral urethra, and in the fusiform subtype, defects in both corpus spongiosum and cavernosum lead to circumferential urethral dilatation. Often, patients' phenotypes are found to exist on a spectrum between these two subtypes [4]. Though the pathophysiology of this condition is not clearly defined, it is known that anomalies of the corpus cavernosa and spongiosa, tissue which normally supports the urethra, lead to urinary stasis and mechanical dilation [9]. Congenital megalourethra is a urinary tract malformation involving with an unclear embryologic origin. It is thought to result from aberrant mesenchymal development and recanalization of penile and glandular urethral tissue throughout embryogenesis [2,4]. Urethral tissue of 2 fetuses exhibiting megalourethra with definite urethral obstruction showed non-canalized epithelium in the glans obstructing the lumen of the patent urethra [9]. This suggests that megalourethra may result from a delay in recanalization as opposed to a failure of this process. If antenatal ultrasound screening detects congenital megalourethra
Corresponding author. E-mail address: [email protected] (S.K. Patel).
https://doi.org/10.1016/j.epsc.2019.101232 Received 17 May 2019; Accepted 26 May 2019 Available online 29 May 2019 2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101232
S.K. Patel, et al.
Fig. 1. Prenatal ultrasound with urethral cyst.
Fig. 4. Cystoscope in distal urethra demonstrating distal urethral dilation.
megalourethra should be performed including renal function testing and complete genitourinary tract imaging [1,7,11]. Treatment of patients depends on subtype, severity of disease, symptomatology and associated renal anomalies. Treatment for scaphoid subtype typically involves a longitudinal reduction urethroplasty as described by Nesbit. More recently, other techniques, such as urethral plication has been described [3,6]. Surgical repair of fusiform subtype is more complex, sometimes involving major phallic reconstruction, requiring long-term follow up [10,11]. Our patient is of interest. The child had normal levels of amniotic fluid during the pregnancy, no hydronephrosis and was urinating prior to repeat evaluation. We believe his infection was due to the sequalae of partial urinary obstruction, which led to urinary stasis and a symptomatic urinary tract infection. Earlier evaluation of this patient's lower urinary tract would have revealed the urethral narrowing.
Fig. 2. Mild hydronephrosis of left kidney.
4. Conclusion In patients with megalourethra, early and complete evaluation of the urinary tract is required. Due to the possibility of progressive partial urinary obstruction, continued urologic surveillance in patients with megalourethra is required [7,11]. Patient consent Consent for publication of the patient case report was obtained, including consent for photographic and radiographic images. This report does not contain any personal information that could lead to the identification of the patient.
Fig. 3. Bladder with urinary sediment.
based on sonographic penile dilatation, serial ultrasonography for AFI, bladder volume, and upper GU tract dilatation is indicated. Postnatal diagnosis is largely based on penile physical examination. Patients with megalourethra have been reported to have associated GU malformations including but not limited to renal dysplasia, VUR, posterior urethral valves, hypospadias, cryptorchidism, and also VACTERL system anomalies [8]. A complete workup of patients with congenital
Funding No funding or grant support. Authorship All authors attest that they meet the current IMCJE criteria for 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101232
S.K. Patel, et al.
Authorship.
[4] Jones EA, Freedman AL, Ehrlich RM. Megalourethra and urethral diverticula. Urol Clin 2002;29(2):341–8https://doi.org/10.1016/S0094-0143(02)00043-5. [5] Mohan CG, Prakash SV, Kumar VV, Babu RG. Isolated megalourethra: a rare case report and review of literature. J Indian Assoc Pediatr Surg 2014;19(3):178–80. https://doi.org/10.4103/0971-9261.136481. [6] Nesbitt TE. Congenital megalo-urethra. J Urol 1955;73(5):839–42https://doi.org/ 10.1016/S0022-5347(17)67484-6. [7] Nijagal A, Sydorak RM, Feldstein VA, Hirose S, Albanese CT. Spontaneous resolution of prenatal megalourethra. J Pediatr Surg 2004;39(9):1421–3. [8] Obara K, Yamazaki H, Yamana K, Kuroki H, Tomita Y. Congenital scaphoid megalourethra: a case report. Urol Case Rep 2017;14:3–4. https://doi.org/10.1016/j. eucr.2017.05.002. [9] Stephens D, Fortune DW. Pathogenesis of megalourethra. J Urol 1993;149(6):1512–6https://doi.org/10.1016/S0022-5347(17)36431-5. [10] Wakhlu AK, Wakhlu A, Tandon RK, Kureel SN. Congenital megalourethra. J Pediatr Surg 1996;31(3):441–3https://doi.org/10.1016/S0022-3468(96)90758-7. [11] Wax JR, Pinette MG, Landes A, Cartin A, Blackstone J. Prenatal sonographic diagnosis of congenital megalourethra with in utero spontaneous resolution. J Ultrasound Med 2009;28(10):1385–8.
Conflicts of interest The following authors have no financial disclosures: Sohum K. Patel, Dr. Ross Simon, Dr. Diana Cardona-Grau, Dr. Mark A. Rich, Dr. Hubert S. Swana. References [1] Appel RA, Kaplan GW, Brock WA, Streit D. Megalourethra. J Urol 1986;135(4):747–51https://doi.org/10.1016/S0022-5347(17)45839-3. [2] Hamzaoui M, Essid A, Laabidi B, Ben Attia M, Houissa T. [Neonatal congenital mega urethra]. Prog Urol 2002;12(4):680–3. [3] Heaton BW, Snow BW, Cartwright PC. Repair of urethral diverticulum by plication. Urology 1994;44(5):749–52.
3
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Congenital megalourethra with delayed obstructive symptoms a,∗
b
c
c
c
Sohum K. Patel , Ross Simon , Diana Cardona-Grau , Mark A. Rich , Hubert S. Swana a b c
T
University of Central Florida College of Medicine, 6850 Lake Nona Blvd, Orlando, FL 32827, United States University of South Florida Morsani College of Medicine, 12901 Bruce B Downs Blvd, Tampa, FL 33612, United States Orlando Health Arnold Palmer Hospital for Children, 92 W Miller St, Orlando, FL 32806, United States
ABSTRACT
Congenital megalourethra is a congenital non-obstructive dilation of the male urethra often associated with VACTERL anomaly malformations. We report a case of TE fistula associated congenital megalourethra with symptoms presenting approximately 2 months postpartum. At the time of referral, patient had distal penile flaccidity and poorly developed corpora. Imaging and cystoscopy showed development of hydronephrosis with significant sediment in the bladder. Attempts at retrograde catheterization failed, thus a Blocksum vesicostomy was required. This demonstrates the importance of serial physical examinations and ultrasound surveillance in patients with megalourethra.
1. Intro Congenital megalourethra is an uncommon dilation of the anterior urethra. We present a case of a 2-month-old boy with prenatally diagnosed megalourethra, initially managed expectantly, who developed complications due to progressive, incomplete bladder emptying. 2. Case report Urology consultation was obtained for a 2-month-old boy who had a history of a “urethral cyst” diagnosed prenatally. He was the product of a 30-week gestation, and at birth found to have esophageal atresia/TE fistula which was repaired shortly after birth. Prenatal ultrasound screening demonstrated a cystic dilation of the urethra (Fig. 1). Amniotic fluid levels were normal and no hydronephrosis was identified. Renal/bladder ultrasonography done shortly after birth as part of VACTERL work-up were unremarkable. Pt underwent repair of tracheoesophageal fistula and was urinating spontaneously. At 31 days of life, in anticipation of transfer to a lower acuity care nursery, urologic consultation was obtained. Physical exam at the time of referral showed descended testicles, a long penis with distal flaccidity and poorly developed distal corporal bodies. Repeat renal/bladder ultrasound revealed new mild hydronephrosis of the left kidney (Fig. 2) and significant debris in the bladder (Fig. 3). Patient became progressively unstable with frequent episodes of apnea. Broad-spectrum antibiotics were administered. Urine analysis was concerning for infection. Retrograde urethral catheterization at the bedside was not successful, however, cystoscopy revealed dilated distal urethra and poorly-formed corporal bodies distally (Fig. 4).
∗
A narrowing of the bulbar urethra was noted. A 0.025 glide wire was able to be passed into the bladder, but larger catheters could not be passed in a retrograde fashion. A Blocksum vesicostomy was created. The patient improved clinically, the hydronephrosis improved and debris cleared. 3. Discussion Congenital megalourethra, first described by Nesbitt, is characterized by dilatation of the anterior urethra [5,6]. Congenital megalourethra is classified into fusiform and scaphoid subtypes, based on urethrography. In the scaphoid subtype, defects in the corpus spongiosum lead to dilation of the ventral urethra, and in the fusiform subtype, defects in both corpus spongiosum and cavernosum lead to circumferential urethral dilatation. Often, patients' phenotypes are found to exist on a spectrum between these two subtypes [4]. Though the pathophysiology of this condition is not clearly defined, it is known that anomalies of the corpus cavernosa and spongiosa, tissue which normally supports the urethra, lead to urinary stasis and mechanical dilation [9]. Congenital megalourethra is a urinary tract malformation involving with an unclear embryologic origin. It is thought to result from aberrant mesenchymal development and recanalization of penile and glandular urethral tissue throughout embryogenesis [2,4]. Urethral tissue of 2 fetuses exhibiting megalourethra with definite urethral obstruction showed non-canalized epithelium in the glans obstructing the lumen of the patent urethra [9]. This suggests that megalourethra may result from a delay in recanalization as opposed to a failure of this process. If antenatal ultrasound screening detects congenital megalourethra
Corresponding author. E-mail address: [email protected] (S.K. Patel).
https://doi.org/10.1016/j.epsc.2019.101232 Received 17 May 2019; Accepted 26 May 2019 Available online 29 May 2019 2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101232
S.K. Patel, et al.
Fig. 1. Prenatal ultrasound with urethral cyst.
Fig. 4. Cystoscope in distal urethra demonstrating distal urethral dilation.
megalourethra should be performed including renal function testing and complete genitourinary tract imaging [1,7,11]. Treatment of patients depends on subtype, severity of disease, symptomatology and associated renal anomalies. Treatment for scaphoid subtype typically involves a longitudinal reduction urethroplasty as described by Nesbit. More recently, other techniques, such as urethral plication has been described [3,6]. Surgical repair of fusiform subtype is more complex, sometimes involving major phallic reconstruction, requiring long-term follow up [10,11]. Our patient is of interest. The child had normal levels of amniotic fluid during the pregnancy, no hydronephrosis and was urinating prior to repeat evaluation. We believe his infection was due to the sequalae of partial urinary obstruction, which led to urinary stasis and a symptomatic urinary tract infection. Earlier evaluation of this patient's lower urinary tract would have revealed the urethral narrowing.
Fig. 2. Mild hydronephrosis of left kidney.
4. Conclusion In patients with megalourethra, early and complete evaluation of the urinary tract is required. Due to the possibility of progressive partial urinary obstruction, continued urologic surveillance in patients with megalourethra is required [7,11]. Patient consent Consent for publication of the patient case report was obtained, including consent for photographic and radiographic images. This report does not contain any personal information that could lead to the identification of the patient.
Fig. 3. Bladder with urinary sediment.
based on sonographic penile dilatation, serial ultrasonography for AFI, bladder volume, and upper GU tract dilatation is indicated. Postnatal diagnosis is largely based on penile physical examination. Patients with megalourethra have been reported to have associated GU malformations including but not limited to renal dysplasia, VUR, posterior urethral valves, hypospadias, cryptorchidism, and also VACTERL system anomalies [8]. A complete workup of patients with congenital
Funding No funding or grant support. Authorship All authors attest that they meet the current IMCJE criteria for 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101232
S.K. Patel, et al.
Authorship.
[4] Jones EA, Freedman AL, Ehrlich RM. Megalourethra and urethral diverticula. Urol Clin 2002;29(2):341–8https://doi.org/10.1016/S0094-0143(02)00043-5. [5] Mohan CG, Prakash SV, Kumar VV, Babu RG. Isolated megalourethra: a rare case report and review of literature. J Indian Assoc Pediatr Surg 2014;19(3):178–80. https://doi.org/10.4103/0971-9261.136481. [6] Nesbitt TE. Congenital megalo-urethra. J Urol 1955;73(5):839–42https://doi.org/ 10.1016/S0022-5347(17)67484-6. [7] Nijagal A, Sydorak RM, Feldstein VA, Hirose S, Albanese CT. Spontaneous resolution of prenatal megalourethra. J Pediatr Surg 2004;39(9):1421–3. [8] Obara K, Yamazaki H, Yamana K, Kuroki H, Tomita Y. Congenital scaphoid megalourethra: a case report. Urol Case Rep 2017;14:3–4. https://doi.org/10.1016/j. eucr.2017.05.002. [9] Stephens D, Fortune DW. Pathogenesis of megalourethra. J Urol 1993;149(6):1512–6https://doi.org/10.1016/S0022-5347(17)36431-5. [10] Wakhlu AK, Wakhlu A, Tandon RK, Kureel SN. Congenital megalourethra. J Pediatr Surg 1996;31(3):441–3https://doi.org/10.1016/S0022-3468(96)90758-7. [11] Wax JR, Pinette MG, Landes A, Cartin A, Blackstone J. Prenatal sonographic diagnosis of congenital megalourethra with in utero spontaneous resolution. J Ultrasound Med 2009;28(10):1385–8.
Conflicts of interest The following authors have no financial disclosures: Sohum K. Patel, Dr. Ross Simon, Dr. Diana Cardona-Grau, Dr. Mark A. Rich, Dr. Hubert S. Swana. References [1] Appel RA, Kaplan GW, Brock WA, Streit D. Megalourethra. J Urol 1986;135(4):747–51https://doi.org/10.1016/S0022-5347(17)45839-3. [2] Hamzaoui M, Essid A, Laabidi B, Ben Attia M, Houissa T. [Neonatal congenital mega urethra]. Prog Urol 2002;12(4):680–3. [3] Heaton BW, Snow BW, Cartwright PC. Repair of urethral diverticulum by plication. Urology 1994;44(5):749–52.
3