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Duplication of the urethral meatus: case report of distal hypospadias and a glanular urethra
Journul of Plastic Surgery (1996), 49, 488-490 0 1996 The British Association of Plastic Surgeons
British
Duplication of the urethral meatus: case report of distal hypospadias and a glanular urethra V. C. Lees and B. C. Sommerlad St. Andrew’s Centre for Plastic Surgery, Billericay, Essex, UK SUMMAR Y. An unusual example of congenital fistula of the distal urethra presenting in a 3-year-old boy is described. The likelihood of this deformitv being a variant of hypospadias is explored and theories of the embryology of the anomaly are discussed. -
Case report A 3-year-old boy presented with the unusual anomaly of a primary fistula of the coronal urethra. The boy had previously had no urinary symptoms but came to medical attention when the parents noticed spraying of urine from an abnormal opening near the end of the penis. On examination, there was a fistulous opening of the urethra just proximal to the glans, in the presence of a normal glanular meatus and an otherwise normal foreskin (Fig. 1). The ventral wall of the urethra both distal and proximal to the fistula was of normal thickness. The fistula was closed electively. At operation, a probe admitted through the glanular meatus was found to lie in a blind-ending channel and this channel was demonstrated to have a proximal connection to the main urethral channel which lay on the ventral side (Figs 2A, B). The TurnerWarwick erection test revealed a mild degree of chordee. Cystoscopy showed no other abnormality of the lower urinary tract. The fistula was managed by division of the urethral bar separating the two channels and layered closure
of the fistula avoiding overlapping of the suture lines.’ The chordee was corrected during the mobilisation of layers for the fistula closure. The foreskin was retained at the parents’ request. Postoperatively, the patient made an uneventful recovery with no recurrence of the fistula. Discussion Congenital uncommon
fistulae of the distal urinary tract are and we are not aware of a previous
description of the specific abnormality described. Congenital urethral fistula occurs as a distinct entity in urethral duplication but is also seen in variants of the hypospadias anomaly where the fistula occurs in association with a blind-ending channel of the glanular urethra.2,3 Patterns of urethral duplication and congenital urethraljstula
Duplication of the urethra has been classified by Middleton and Melzer4 as follows: l
l
l
complete with the accessory urethra extending from any point proximal to the external sphincter and the fistulous opening anywhere other than the normal meatal position. incomplete with the accessory urethral meatus located anywhere other than the normal meatal opening and with the accessory urethra ending either blindly or communicating with the primary urethra at some point distal to the external sphincter. internal where the accessory urethra arises from and re-enters the urethra at another level (in this category there is no fistulous opening).4J
The majority of urethral duplications occur in the sagittal plane.3 Typically the accessory urethra runs dorsal to the main urethra with which it has no connection.6*7 Association of hypospadias with urethraljstula
Fig. 1 Figure l-Congenital fistula of the distal urethra of an otherwise normally developed foreskin.
Hypospadias is defined as a congenital anomaly in which the urethra ends on the ventral surface of the
in the presence
488
Duolication
of the
urethral
489
meatus
B Fig. 2 Figure 2-(A) The urethral sound urethra The black arrow points to sagittal section. Failure of complete ventral wall weakness, is believed to
lies in the blind-ending glanular urethra. The wire probe demonstrates the opening of the penile the bar of tissue separating the two channels. (B) Diagrammatic representation of the anomaly in canalisation of the glanular urethra. at the point indicated with a black arrow. coincident with a have led to ventral fistula formation.
penile shaft or perineum. In association with this there is often chordee and in the majority of cases a dorsal hooded prepuce which is deficient ventrally. Our case is likely to have been a variant of the hypospadias anomaly distinguished from the classical abnormality by the presence of the distal urethral duplication and by the intact prepuce. The fact that our case had an intact prepuce does not preclude the diagnosis, as hypospadias has been reported in association with a normally developed foreskin, for example in a series of 9 cases by Hatch et al.8 Van der Meulen’ presented an analysis of that group of ‘hypospadiaslike’ anomalies which have been termed ‘cryptospadias’ and includes congenital urethral fistula, congenital short urethra and isolated chordee. The incidence of these conditions is probably greater than the literature would suggest. There appear to be two groups of distal congenital urethral fistulae.” The duplicated urethra may connect with the main urethral channel or be blind-ending in both cases. The groups are distinguished by the presence or absence of the ventral prepuce.‘.” As the ventral wall of the urethra appeared of normal quality proximal and distal to the ventral opening, we would discount the possibility of the case being an example of the ‘covered urethra’ variation of hypospadias (characterised by a hypoplastic ventral wall concealing a more proximal meatal opening). l1 It is probable that our case represents a form of cryptospadias and would be classified under the second of Van der Meulen’s categories and as an incomplete duplication on Middleton and Melzer’s scheme. Theories of urethrul development During normal development the urethral folds coalesce on the ventral aspect of the penile shaft forming the penile urethra. Simultaneously, a solid cord in the glans canalises from the external meatus to join the penile urethra, completing urethral formation.‘2-‘4 The ectopic ventral meatus of hypospadias
is thought to be caused by failure of fusion of the urethral folds. Ventral duplications originate from fusion errors of the genital folds and urethral plate.” An unusual opportunity to study a 9-week-old human embryo with hypospadias having a ventral meatus, a distal epithelial-lined pit and an accessory urethra presented to Rowsell and Morgan.” A urethral plate remnant on the ventral surface of the proximal glans supported Glenister’s theory that the proximal glanular urethra is formed by the fusion of urethral folds. The presence of epithelial lamella in the middle third of the glans suggested that the distal glanular urethra forms by the canalisation of epithelial cells which have grown in from the tip of the glans. These authors also suggested that the accessory urethra found in the distal shaft represented a primary abnormality of the urethral plate unrelated to any ingrowing cord of cells.” The pathogenesis of our case can only be surmised. It is possible that a partial failure of the ectodermal and endodermal urethral channels to coalesce left the blind-ending channel of the glanular urethra. Incomplete ventral closure of the endodermal urethral channel could then have led to the ectopic opening of the urethral channel at the level of the coronal sulcus.
References I. Retik AB, Keating M, Mandell J. Complications of hypospadias repair. Urol Clin North Am 1988; 15: 223-36. 2. Horton CE, Devine CJ, Adamson JE, Carraway JH. Hypospadias, epispadias and exstrophy of the bladder. In: Grabb WC, Smith JW, eds., Plastic surgery. 3rd ed. Boston: Little, Brown and Company, 1979; 856. 3. Woodhouse CRJ, Williams DI. Duplications of the lower urinary tract in children. Br J Urol 1979; 51: 481-7. 4. Middleton AW, Melzer RB. Duplicated urethra: an anomaly best repaired. Urol 1992; 39: 538-42. 5. Connolly AAP, Parker C, Briggs T, Miller R. Incomplete urethral duplication treated by endoscopic scissors. Br J Urol 1993; 48: 62334. 6. Townsend PLG. Accessory urethras in hypospadias. Br J Plast Surg 1978: 31: 309912.
British Journal of Plastic Surgery
490 7. Kvist E, Sjolin K-E, Stahl D. Accessory urethra in a male. Stand J Ural Nephrol 1993; 28: 211-12. 8. Hatch DA, Maizels M, Zaontz MR, Firlit CF. Hypospadias hidden by a complete prepuce. Surg Gynecol Obstet 1989; 169: 23334. 9. Van der Meulen JC. Hypospadias and cryptospadias. Br J Plast Surg 1971; 24: 101%3. 10. Gupta SC. An unusual type of hypospadias. Br J Plast Surg 1962; 15: 191-3. 11. Attalla MF. Subcoronal hypospadias with complete prepuce: a distinct entity and new procedure for repair. Br J Plast Surg 1991; 44: 12225. 12. Hart DB. On the role of the developing epidermis in forming sheaths and lumina to organs. J Anat 1908; 42: 50-6. 13. Jones FW. The development and malformations of the glans and prepuce. BMJ 1910; 1: 13778. 14. Glenister JW. The origin and fate of the urethral plate in man. J Anat 1954; 288: 413-25. 15. Stephens FD. Congenital intrinsic lesions of the posterior
urethra. In: Congenital malformations New York: Praeger, 1983: 95. 16. Rowsell AR, Morgan BDG. Hypospadias esis of the penile urethra. Br J Plast
of the urinary
tract.
and the embryogenSurg 1987; 40: 201-6.
The Authors V. C. Lees MD, FRCS(Plast), Fellow, Christine M. Kleinert Institute for Hand and Microsurgery, Louisville, USA B. C. Sommerlad FRCS, Consultant Plastic Surgeon, St. Andrew’s Centre for Plastic Surgery, Essex, UK. Correspondence to V. C. Lees MD, FRCS(Plast), Kleinert Institute for Hand and Microsurgery, Flexner Way, Louisville 40202, USA. Paper received 21 January 1994. Accepted 10 June 1996, after revision.
Christine M. 225 Abraham
British
Duplication of the urethral meatus: case report of distal hypospadias and a glanular urethra V. C. Lees and B. C. Sommerlad St. Andrew’s Centre for Plastic Surgery, Billericay, Essex, UK SUMMAR Y. An unusual example of congenital fistula of the distal urethra presenting in a 3-year-old boy is described. The likelihood of this deformitv being a variant of hypospadias is explored and theories of the embryology of the anomaly are discussed. -
Case report A 3-year-old boy presented with the unusual anomaly of a primary fistula of the coronal urethra. The boy had previously had no urinary symptoms but came to medical attention when the parents noticed spraying of urine from an abnormal opening near the end of the penis. On examination, there was a fistulous opening of the urethra just proximal to the glans, in the presence of a normal glanular meatus and an otherwise normal foreskin (Fig. 1). The ventral wall of the urethra both distal and proximal to the fistula was of normal thickness. The fistula was closed electively. At operation, a probe admitted through the glanular meatus was found to lie in a blind-ending channel and this channel was demonstrated to have a proximal connection to the main urethral channel which lay on the ventral side (Figs 2A, B). The TurnerWarwick erection test revealed a mild degree of chordee. Cystoscopy showed no other abnormality of the lower urinary tract. The fistula was managed by division of the urethral bar separating the two channels and layered closure
of the fistula avoiding overlapping of the suture lines.’ The chordee was corrected during the mobilisation of layers for the fistula closure. The foreskin was retained at the parents’ request. Postoperatively, the patient made an uneventful recovery with no recurrence of the fistula. Discussion Congenital uncommon
fistulae of the distal urinary tract are and we are not aware of a previous
description of the specific abnormality described. Congenital urethral fistula occurs as a distinct entity in urethral duplication but is also seen in variants of the hypospadias anomaly where the fistula occurs in association with a blind-ending channel of the glanular urethra.2,3 Patterns of urethral duplication and congenital urethraljstula
Duplication of the urethra has been classified by Middleton and Melzer4 as follows: l
l
l
complete with the accessory urethra extending from any point proximal to the external sphincter and the fistulous opening anywhere other than the normal meatal position. incomplete with the accessory urethral meatus located anywhere other than the normal meatal opening and with the accessory urethra ending either blindly or communicating with the primary urethra at some point distal to the external sphincter. internal where the accessory urethra arises from and re-enters the urethra at another level (in this category there is no fistulous opening).4J
The majority of urethral duplications occur in the sagittal plane.3 Typically the accessory urethra runs dorsal to the main urethra with which it has no connection.6*7 Association of hypospadias with urethraljstula
Fig. 1 Figure l-Congenital fistula of the distal urethra of an otherwise normally developed foreskin.
Hypospadias is defined as a congenital anomaly in which the urethra ends on the ventral surface of the
in the presence
488
Duolication
of the
urethral
489
meatus
B Fig. 2 Figure 2-(A) The urethral sound urethra The black arrow points to sagittal section. Failure of complete ventral wall weakness, is believed to
lies in the blind-ending glanular urethra. The wire probe demonstrates the opening of the penile the bar of tissue separating the two channels. (B) Diagrammatic representation of the anomaly in canalisation of the glanular urethra. at the point indicated with a black arrow. coincident with a have led to ventral fistula formation.
penile shaft or perineum. In association with this there is often chordee and in the majority of cases a dorsal hooded prepuce which is deficient ventrally. Our case is likely to have been a variant of the hypospadias anomaly distinguished from the classical abnormality by the presence of the distal urethral duplication and by the intact prepuce. The fact that our case had an intact prepuce does not preclude the diagnosis, as hypospadias has been reported in association with a normally developed foreskin, for example in a series of 9 cases by Hatch et al.8 Van der Meulen’ presented an analysis of that group of ‘hypospadiaslike’ anomalies which have been termed ‘cryptospadias’ and includes congenital urethral fistula, congenital short urethra and isolated chordee. The incidence of these conditions is probably greater than the literature would suggest. There appear to be two groups of distal congenital urethral fistulae.” The duplicated urethra may connect with the main urethral channel or be blind-ending in both cases. The groups are distinguished by the presence or absence of the ventral prepuce.‘.” As the ventral wall of the urethra appeared of normal quality proximal and distal to the ventral opening, we would discount the possibility of the case being an example of the ‘covered urethra’ variation of hypospadias (characterised by a hypoplastic ventral wall concealing a more proximal meatal opening). l1 It is probable that our case represents a form of cryptospadias and would be classified under the second of Van der Meulen’s categories and as an incomplete duplication on Middleton and Melzer’s scheme. Theories of urethrul development During normal development the urethral folds coalesce on the ventral aspect of the penile shaft forming the penile urethra. Simultaneously, a solid cord in the glans canalises from the external meatus to join the penile urethra, completing urethral formation.‘2-‘4 The ectopic ventral meatus of hypospadias
is thought to be caused by failure of fusion of the urethral folds. Ventral duplications originate from fusion errors of the genital folds and urethral plate.” An unusual opportunity to study a 9-week-old human embryo with hypospadias having a ventral meatus, a distal epithelial-lined pit and an accessory urethra presented to Rowsell and Morgan.” A urethral plate remnant on the ventral surface of the proximal glans supported Glenister’s theory that the proximal glanular urethra is formed by the fusion of urethral folds. The presence of epithelial lamella in the middle third of the glans suggested that the distal glanular urethra forms by the canalisation of epithelial cells which have grown in from the tip of the glans. These authors also suggested that the accessory urethra found in the distal shaft represented a primary abnormality of the urethral plate unrelated to any ingrowing cord of cells.” The pathogenesis of our case can only be surmised. It is possible that a partial failure of the ectodermal and endodermal urethral channels to coalesce left the blind-ending channel of the glanular urethra. Incomplete ventral closure of the endodermal urethral channel could then have led to the ectopic opening of the urethral channel at the level of the coronal sulcus.
References I. Retik AB, Keating M, Mandell J. Complications of hypospadias repair. Urol Clin North Am 1988; 15: 223-36. 2. Horton CE, Devine CJ, Adamson JE, Carraway JH. Hypospadias, epispadias and exstrophy of the bladder. In: Grabb WC, Smith JW, eds., Plastic surgery. 3rd ed. Boston: Little, Brown and Company, 1979; 856. 3. Woodhouse CRJ, Williams DI. Duplications of the lower urinary tract in children. Br J Urol 1979; 51: 481-7. 4. Middleton AW, Melzer RB. Duplicated urethra: an anomaly best repaired. Urol 1992; 39: 538-42. 5. Connolly AAP, Parker C, Briggs T, Miller R. Incomplete urethral duplication treated by endoscopic scissors. Br J Urol 1993; 48: 62334. 6. Townsend PLG. Accessory urethras in hypospadias. Br J Plast Surg 1978: 31: 309912.
British Journal of Plastic Surgery
490 7. Kvist E, Sjolin K-E, Stahl D. Accessory urethra in a male. Stand J Ural Nephrol 1993; 28: 211-12. 8. Hatch DA, Maizels M, Zaontz MR, Firlit CF. Hypospadias hidden by a complete prepuce. Surg Gynecol Obstet 1989; 169: 23334. 9. Van der Meulen JC. Hypospadias and cryptospadias. Br J Plast Surg 1971; 24: 101%3. 10. Gupta SC. An unusual type of hypospadias. Br J Plast Surg 1962; 15: 191-3. 11. Attalla MF. Subcoronal hypospadias with complete prepuce: a distinct entity and new procedure for repair. Br J Plast Surg 1991; 44: 12225. 12. Hart DB. On the role of the developing epidermis in forming sheaths and lumina to organs. J Anat 1908; 42: 50-6. 13. Jones FW. The development and malformations of the glans and prepuce. BMJ 1910; 1: 13778. 14. Glenister JW. The origin and fate of the urethral plate in man. J Anat 1954; 288: 413-25. 15. Stephens FD. Congenital intrinsic lesions of the posterior
urethra. In: Congenital malformations New York: Praeger, 1983: 95. 16. Rowsell AR, Morgan BDG. Hypospadias esis of the penile urethra. Br J Plast
of the urinary
tract.
and the embryogenSurg 1987; 40: 201-6.
The Authors V. C. Lees MD, FRCS(Plast), Fellow, Christine M. Kleinert Institute for Hand and Microsurgery, Louisville, USA B. C. Sommerlad FRCS, Consultant Plastic Surgeon, St. Andrew’s Centre for Plastic Surgery, Essex, UK. Correspondence to V. C. Lees MD, FRCS(Plast), Kleinert Institute for Hand and Microsurgery, Flexner Way, Louisville 40202, USA. Paper received 21 January 1994. Accepted 10 June 1996, after revision.
Christine M. 225 Abraham