Focus on Hypospadias

What is it

Hypospadias is a congenital abnormality of the penis due to an incomplete development of the male urethra. This involves the fact that the urinary meatus can be located in variable positions of the rod, from the glans to the perineum, as well as a ventral curvature of the rod, all the more serious the more proximal to the meatus. The curvature is due to the presence of fibrous tissue (cords) close to the ventral portion of the cavernous bodies distally to the urinary meatus. In less severe forms it is caused by insufficient development of the skin that ventrally covers the penis (skin cords). It is one of the most common congenital pathologies of the male genital apparatus in the child, which is not transmitted in a hereditary way but for which there may be a family predisposition. On average, for every child with hypospadias there is a 20% chance of having another family member with the same problem. The correction of hypospadias remains one of the most complex surgical specialties in the field of pediatric surgery and urology, but it can be corrected satisfactorily, without resorting to long hospitalizations, intervening in an adequate phase of the psychosexual development of the child that allows to minimize the psychological trauma and the relevant consequences on the future functional, aesthetic and relationship life that genital surgery entails.

Pediatric urological surgery: how and when to intervene on the congenital anomaly of the penis, one of the most common pathologies in children

How it looks

The urinary meatus can take on different morphologies and is usually restricted or point-like and the skin lining of the rod, in its lower side, consists of a particularly thin epithelium, tenaciously adhered to the underlying tissues. The median raphe of the penis, a direct continuation of the scrotal one, divides, distally to the meatus, to draw a triangular area whose vertex is represented by the urinary meatus itself and the base by the coronal sulcus. At the apexes of this triangular area the lateral margins of the dorsal preputial apron that is missing on the ventral side stop.

What it entails

It is a malformation that affects not only the aesthetic aspect but also the functional one. The abnormal position of the meatus makes erect urination impossible. The curvature of the rod causes painful erection and, in the adult, difficulty in penetration up to copulatory incapacity.

The different forms

Distal hypospadiorias

In most cases, "distal hypospadias" occur. In many children with distal hypospadias, the extent of the malformation and its importance in life are negligible from a urological point of view. They represent essentially aesthetic anomalies that do not prevent the future adult from living an absolutely normal life.In distal hypospadias the external urethral meatus is located near the coronal sulcus or at the glandular level. In these cases the penis is of normal size for gestational age or minimally reduced. The rod has a minimal or absent curvature and the penile urethra is of substantially normal morphology especially with regard to the spongy body. The same applies to the constituent elements of the auction (dartos, Buck's band).Many of the babies born with this problem have the classic malformation of the hypospadic foreskin which consists of a skin deficiency in the ventral portion of the shaft. The dorsal part is redundant and there are frequently two rounded skin markers historically called "eyes of Ombredanne". Sometimes, this cutaneous portion takes on a scrotal appearance and represents an imperfection such that it cannot be preserved during surgical correction. In the skin of the rod a clear transition zone is created towards the preputial cap described.Some children have glandular hypospadias with intact foreskin. Diagnosis is made in the later ages of life after dilation of the preputial ostium and resolution of preputial balan adhesions have occurred.

Proximal hypospadias

(25-30% of cases): medium-penile hypospadias, proximal and proximal peniles (scrotal or perineal). These forms of hypospadias take on a profoundly different meaning than the previous ones, therefore, both the diagnostic and therapeutic approach must also be completely different. The finding of these forms of hypospadias can represent, in fact, one of the manifestations of a polymalformative syndrome or an anomaly of sexual differentiation. Particular attention should be paid to looking for other elements that can provide a further suspicion of complex syndrome: the most important of these is mono testicular retention (mixed gonadal dysgenesis may be present) or bilateral (it is the typical mode of presentation of the newborn with adrenogenital syndrome). Infants with proximal hypospadias may have lower urinary tract abnormalities with persistence of Müllerian residues. It is advisable to perform a urinational cystography with urethrography to precisely define the proximal urethra and the possible presence of a vaginal sketch or a dilation of the prostate utricle (embryonic residue of the female sexual organs).

Diagnostics

The first evaluation in the hypospadic baby is carried out at the hospital where the baby is born. The neonatologist will assess the severity of the penile abnormality, the presence of the testicles and their normal descent into the scrotum; diagnostic screening investigations will also be performed to assess the presence of coexisting abnormalities of the urinary tract or other body districts. If the child has no descent of the testicles, forms of severe hypospadias or associated abnormalities affecting other organs, genetic counseling will be appropriate.

The recommended age for surgery

Between 6 and 12 months of the baby's life.

Treatment

Hypospadias necessarily requires surgical treatment that will be adapted to each individual patient, after careful pre-operative evaluation and the administration of anesthesia (general or epidural). In patients suffering from distal hypospadias with a good urethral shower, we usually intervene for the construction of the neourethra with the tubulization of the incised urethral plate (T.I.U.P.), an intervention that allows to use the embryological tissue intended to create the urethra and return to the penis an anatomy as close as possible to normal, obtaining excellent results over time. This is among other things the intervention by election also for the well-known American surgeon, specialist in pediatric urology, Warren Snodgrass.

For the correction of proximal hypospadias, on the other hand, multiple surgical approaches can be used. A return to the two surgical times has returned to the present, following the results presented by the English surgeon Aivar Bracka. In this case and in the correction of hypospadias complicated by previous surgical interventions, the use of the buccal mucosa as a replacement material is providing encouraging results: the removal of the mucous membrane from the cheek and / or from the inner surface of the lip is easy and does not involve either functional or aesthetic consequences and post-operative discomfort is limited.

Possible complications

In the phase of healing and consolidation of the reconstructed anatomical structures, which lasts a few months, a series of complex biological mechanisms occur, namely tissue healing. These natural events can cause negative effects on the reconstructed genital organ, compromising its normal urinary and sexual functions. It is therefore advisable to wait before assessing the success of the intervention. Among the most frequent complications, the appearance of: fistulas along the course of the reconstructed urethral canal and urethral strictures (narrowing of the lumen of the new urethra) may occur.

The post-operative

The use of a temporary urinary derivation (catheter) is essential to ensure the healing and consolidation of the walls of the neo-urethra. Depending on the type of surgery, the surgeon decides whether to use it or not. For distal forms and in young children, who have not yet achieved bladder emptying control, the catheter may not be applied. The older child, who has achieved continence, usually requires a urinary derivation in the first days after surgery. In cases where it is required, preference is for a silicone bladder transurethral catheter, fixed externally to the glans from a suture point and left to drip into the diaper: it allows a faster discharge, not conditioning the baby to the bed. The catheter is extremely well tolerated by both the child and parents and can easily be removed on average after 7-10 days.

In the post-operative period, moreover, after the removal of the dressing, the penis may still be swollen and ecchymotic for a few days, of unpleasant appearance.

For a successful intervention

The achievement of good results in treatment depends on a set of relevant aspects. First of all, from the technical ability and experience of the surgeon and the choice of the type of surgical technique most suitable for the individual case, in addition to the choice of the most appropriate time to intervene surgically on the child in relation to the evolutionary phase and the pre-operative preparation. The equipment available to the surgeon is also important. In the field of reconstructive surgery of hypospadias, significant technical advances have been achieved that have contributed to the improvement of the functional and aesthetic results of the pathology. Among the technological innovations useful for this purpose, the modern means of optical magnification should be noted, in addition to new suture materials and postoperative management methods (from catheters to materials used for example for bandaging). Equally decisive for the success of the intervention is in fact the right attention to the post-operative course and compliance with the necessary and regular checks.

Correct information, therefore, together with a proactive collaboration between parents and surgeon contribute significantly to the success of the intervention, reducing the risks of complications and the discomfort of the moment for the child.

In our Pediatric Surgery and Urology Center, the child, with his family, finds the place of reception and complete care of hypospadias: the center where he can face the entire path, from the diagnosis of hypospadias, to treatment up to post-surgery.