Midshaft Hypospadias Research Articles

Identification of 46,XX/46,XY chimerism in an infant with ambiguous genitalia

A variety of molecular methods can aid in the elucidation of complex chromosomal aberrations. We report the case of a newborn infant with ambiguous genitalia born to a 32 year-old G2 P1. Physical exam at delivery showed a vigorous infant with a small phallus with mid-shaft hypospadias. A bifid scrotum was present, with a palpable gonad bilaterally. Abdominal ultrasound showed no female internal structures. Blood was obtained for karyotype and sex determination, and CAM was ruled out. Initial PCR was positive for sry sequences; amelogenin studies done on the same sample showed only X sequences. The preliminary karyotype showed only 46, XX cells. The amelogenin studies were repeated with a longer exposure, and this time in addition to the normal X sequences, very faint amplification of the Y sequences was visible. Final prophase karyotype showed the majority of cells to be 46, XX, however 4 of 22 cells examined showed a 46, XY pattern. Fluorescent in situ hybridization studies, using both whole chromosome paint for the Y chromosome, and a mixture of alpha satellite and classical satellite probes confirmed the presence of XY cells in the culture. The karyotype was 46.XX[18]/46, XY[4].ish (wcpY + ,DYZ1 + ,DYZ3 + ). The infant is doing well at 3 months and is being raised as a male. Good communications and coordination between the cytogenetic and molecular labs in this potentially confusing case allowed for a rapid diagnosis.

THE SPLIT PREPUCE IN SITU ONLAY HYPOSPADIAS REPAIR

Purpose We describe the surgical technique and report the results of the first 100 patients who underwent a modification of the onlay hypospadias repair, which we refer to as split prepuce in situ onlay repair. Materials and Methods We treated 100 boys with a mean age of 11 months at surgery who had coronal to mid shaft hypospadias with split prepuce in situ onlay hypospadias repair. The operative technique varies from that of the standard onlay procedure by preserving the whole blood supply of the half of the prepuce used for the island onlay flap, and using its abundant subcutaneous tissue to cover completely the suture lines used to create the neourethra. Results Only 5 complications required reoperation, including 1 hematoma evacuation and 4 urethrocutaneous fistulas. No patient had meatal stenosis, urethral stricture, meatal retraction or acquired urethral diverticulum necessitating reoperation. A good cosmetic result was obtained in all cases. Conclusions Split prepuce in situ onlay hypospadias repair is applicable in virtually all cases of coronal to mid shaft hypospadias. It optimizes the blood supply to the island flap and provides well vascularized coverage of the neourethra, resulting in a decreased complication rate.

One-stage hypospadias repair with stent or suprapubic diversion: Which is better?

Purpose: The authors report on 105 consecutive patients who underwent one-stage hypospadias repair based on use of suprapubic diversion or transurethral drainage with stenting. Methods: The surgical procedures included 52 metal-based flap urethroplasty (Mathieu) for coronal, subcoronal, and distal shaft hypospadias; 32 transverse island pedicle graft (Duckett) for mid and proximal shaft hypospadias; 21 transverse island pedicle (Duckett) plus rolied midline tube (Thierchs) for penoscrotal and scrotal hypospadias. To accomplish urinary drainage, suprapubic diversion (cystofix) was used in 28 of 52 Mathieu operations, in 17 of 32 Duckett operations, and in 11 of 21 transverse island pedicle graft plus rolled midline tube operations. In the rest of the cases, transurethral drainage with stenting was used. Results: All children had excellent cosmetic and functional outcomes. But the rates of complications such as fistula and meatal stenosis were significantly different between the groups in which suprapubic tube or urethral stent was used. In 56 of the 105 patients in whom suprapubic diversion was used, four (7.14%) had fistulas and three (5.35%) had meatal stenosis, in contrast to a fistula rate of 14.28% and meatal stenosis rate of 12.24% in patients that urethral stent is used for urinary drainage. Conclusion: The authors believe that the use of suprapubic diversion is advantageous for the outcome of one-stage hypospadias repair in relation to fistula occurrence and meatal stenosis.

The extended Mathieu procedure in coronal, distal penile, and midshaft hypospadias repair

The Mathieu procedure was adequately modified for glanular, coronal, and midshaft hypospadias variants with chordee and/or urethral hypoplasia in order to anatomically reconstruct the penis. A total of 129 patients aged 12 months to 22 years with coronal (35.7%), distal penile (51.9%), and midshaft (12.4%) hypospadias were operated upon. Chordee was found in 33.3% and distal urethral hypoplasia in 15.5% of the patients. The ventral flap was based anatomically on the external meatus, the glans was made pyramidal with an apical external meatus, the prepuce was resutured, and the neurethra was buried deeply within the glans. In patients with distal urethral hypoplasia the external meatus was incised proximally; in chordee the urethral plate was mobilized off the corpora cavernosa. Fistulas were uncommon (3.4%) in the primarily operated patients. The external meatus was always apical, and no stenosis was demonstrated at follow-up. The prepuce was reconstructed in 71.5% of patients. The extended Mathieu procedure appears recommendable for all coronal, distal penile, and midshaft variants regardless of associated anomalies of the penis. It also seems suitable for secondary repairs. The prepuce can be reconstructed in the majority of patients. The complication rate is significantly low compared to other methods, and the extended Mathieu operation is a good teaching tool with a high success rate and versatility.

PRENATAL SONOGRAPHIC DIAGNOSIS OF MID SHAFT HYPOSPADIAS

We report the prenatal diagnosis of mid shaft hypospadias and describe the sonographic features of fetal hypospadias including an abnormal urethral canal, ventral curvature of the distal penis, extension of the penile glans beyond the prepuce, and fetal micturation in a plane perpendicular to the penile shaft. An accurate family history is an essential part of the evaluation of the milder degrees of fetal hypospadias.

Isograft Bladder Mucosal Transplantation for Hypospadias Repair in Identical Twins

Urethral reconstruction with a bladder mucosal free graft offers several advantages for patients with complex hypospadias. We report a case in which identical twins circumcised at birth presented with mid-shaft hypospadias and chordee. A bladder mucosa graft was harvested from 1 twin, divided and used to form 2 neourethras. The donor twin underwent hypospadias repair, followed immediately by repair in the second twin with the isograft neourethra. To our knowledge this is the first reported case of bladder mucosal transplantation for hypospadias repair.

Intracorporeal Pressure Monitoring During Artificial Penile Erection

In an effort to determine if artificial penile erection has any harmful effect in children intracorporeal pressures were measured in 12 boys (mean age 18 months) with mid shaft hypospadias. Using a solid state intracompartmental pressure monitoring system, intracorporeal pressures were obtained during full penile erection and after removal of the tourniquet at detumescence. The mean intracorporeal pressure obtained was 85.9 mm. Hg during erection and 21.6 mm. Hg at detumescence. The systemic arterial pressure obtained was 102 mm. Hg at full erection. The pressure studies would suggest that the intracorporeal pressures achieved during full artificial erection would not be harmful to the cavernosal tissue in the hypospadiae child.

Growth hormone deficiency, Wormian bones, dextrocardia, brachycamptodactyly, and other midline defects

We report on a 17-month-old boy with Wormian bones, short stature, growth hormone deficiency, developmental delay, brachycamptodactyly, dextrocardia, cryptorchidism, midshaft hypospadias, hypoplastic left kidney, and imperforate anus. This unique combination of abnormalities has not been reported previously.

Androgen Receptor Levels and 5α-Reductase Activities in Preputial Skin and Chordee Tissue of Boys with Isolated Hypospadias

The cause of hypospadias in the majority of patients is unknown. We examined the hypothesis that hypospadias might be explained by androgen receptor abnormalities in the atretic spongiosal tissue commonly known as chordee. We studied 10 patients with relatively severe hypospadias but with a predominantly male phenotype and no readily ascertained explanation for the defect, including no evidence of an abnormality in testosterone biosynthesis. Eight subjects had midshaft hypospadias and 2 had a penoscrotal meatus. All 10 patients had severe chordee. Serum concentrations of testosterone, and luteinizing and follicle-stimulating hormones were measured before human chorionic gonadotropin stimulation and a serum testosterone level was determined 24 hours after the last dose of a 5-day human chorionic gonadotropin stimulation (3,000 units per M.2 per day).Androgen receptor content and binding affinity were assayed in fibroblasts cultured from preputial skin and chordee tissue of patients and foreskin from normal male neonates. With the endogenous ligand dihydrotestosterone the mean number (maximum binding capacity) of androgen receptors was 1,013 fmol. per mg. deoxyribonucleic acid in preputial skin and 833 fmol. per mg. deoxyribonucleic acid in chordee tissue of patients, and 627 fmol. per mg. deoxyribonucleic acid in the foreskin of controls. With the nonmetabolizable, synthetic androgen methyltrienolone (R1881) the mean maximum binding capacity was 1,004, 722 and 758 fmol. per mg. deoxyribonucleic acid, respectively. Dihydrotestosterone receptor affinity (dissociation constant) was similar in preputial skin (0.20nM.) and chordee tissue (0.21nM.) from patients, and foreskin (0.29nM.) from controls. Androgen receptor binding affinity of R1881 also was similar (0.30, 0.24 and 0.21nM., respectively). Furthermore, the 5 α-reductase activity of preputial skin and chordee tissue of patients with hypospadias was similar to that of foreskin from normal neonates.In conclusion, isolated hypospadias in these subjects was not associated with androgen insensitivity of the spongiosal tissues on the basis of either decreased androgen receptor binding affinity, receptor number or conversion of testosterone to dihydrotestosterone.

The repair of penile hypospadias by the van der Meulen technique

During the years 1980–1985, 80 children with penile hypospadias underwent repair using a slight modification of the van der Meulen technique. All the children had mid or distal shaft hypospadias excluding those with glandular or penoglandular position. All patients except 10 had chordee which necessitated a release at a previous operation. Two children had previously unsuccessful repairs by the Denis Browne technique. The overall hospitalization was at the mean of 4.6 days per child. In 10 children (12.5%) some complications occurred, of which 3 (3.75%) had fistula and in six (7.5%) meatal or neo-urethral strictures developed. In a small number of patients there was some degree of torsion, mostly temporary, and in no case did it cause any functional disturbance.

Modification of the “Mustarde´-Mathieu“ and “Horton-Devine“ urethroplasty in the management of hypospadias

This paper reports on the method of repairing distal or mid-shaft hypospadias in 42 patients using a modification of the Mustarde´-Mathieu and Horton-Devine techniques. A terminal meatus was achieved in all cases; there were two fistulae and one meatal stenosis.

Onlay Island Flap in the Repair of Mid and Distal Penile Hypospadias Without Chordee

The onlay island flap is a variation of the transverse preputial island flap for hypospadias repair. It is useful in patients without fibrous chordee whose meatus is mid penile or subcoronal. This technique was used in 50 patients and the results were compared to those of 34 patients undergoing the Mathieu meatal-based flap (flip-flap) during the same period. The cosmetic results with the onlay island flap were quite satisfactory and the complication rate was 6 per cent, which was identical to that observed with the Mathieu repair. The onlay island flap is applicable particularly in patients with mid shaft hypospadias without chordee, if the meatus is too proximal for a Mathieu repair and in patients with distal penile hypospadias with deficient ventral skin.

Comparative Study of Hodgson I and King’s Methods of 1-stage Hypospadias Repair

We compared the surgical results of the Hodgson I and King’s methods of repair with and without urinary diversion in 33 patients with juxtaglandular to mid shaft hypospadias and mild to moderate chordee. Over-all, fistulas developed along the suture line in 4 patients, of whom only one underwent urinary diversion. Of 21 cases in which King’s method of repair was done only 1 fistula developed. Of the 12 cases repaired by the Hodgson I method fistulas developed in 3 and 1 had a stricture at the site of the new urethro-urethral anastomosis. The best surgical results were achieved in those patients who had undergone King’s method of repair with urinary diversion.

“Burried strip” versus “tube” in hypospadias repair: Analysis of 82 consecutive cases

The goal of this study was to analyze, discuss, and compare risks and results obtained with the “buried strip” and two “tube” techniques for hypospadias repair in a personal series. From 1976 through 1982, 82 patients underwent complete two-stage repair of hypospadias using either the Denis Browne procedure (23 patients) or the Belt-Fuqua technique (41) and Byars procedure (18), respectively. Only those patients with originally distal and mid shaft hypospadias plus chordee were included in this series. The mean age at the time of repair was 4.9 years, with a range of two to sixteen years. The overall complication rate was 13.4 per cent (11 of 82 The tube procedures alone had a complication rate of 6.8 per cent (4 of 59) compared with 30 per cent (7 of 23) with the Denis Browne technique. Complications noted were: fistulas 6 (Denis Browne 5, Belt-Fuqua 1); meatal stenosis 4 (Denis Browne 2, Byars 2); urethral stricture 1 (Belt-Fuqua Our results confirm the general belief that tube techniques have a higher reliability than does the Denis Browne procedure. Each procedure improves with the experience of the surgeon, although the tube technique appears to cause less trouble in our hands. The running subcutaneous and intracutaneous sutures give a far smoother healing. We believe this study supports the virtues of two-stage tube repairs.

Experience with the Belt-fuqua Hypospadias Repair

From 1977 through 1979, 30 patients underwent complete repair of hypospadias using a modification of the Belt-Fuqua technique. Only those patients with distal and mid shaft hypospadias and chordee were included in this series. Three patients had undergone previous attempts at repair by other methods. The mean age at the time of repair was 6.4 years, with a range of 1 to 18 years. The over-all complication rate was 10 per cent (3 of 30), with 2 fistulas (6.6 per cent) and 1 distal stricture (3 per cent). All patients had an excellent functional result. The absence of midline scars and the glandular meatus resulted in a superior cosmetic appearance. The reliability of this technique, as well as the functional and cosmetic results, warrant consideration in the selection of a procedure for hypospadias repair.