Scrotal phalloplasty: A novel surgical technique for aphallia during infancy and childhood by pre-anal anterior coronal approach.

All the currently known reconstructions for aphallia are carried out around puberty. We describe a novel technique as a temporizing procedure which would see the patient through childhood. This would not cause any hindrance while undertaking any of the other established procedures later on after puberty.

INTRODUCTION

Aphallia is absence of penis in a genotypic male. It is due to failure of development of genital tubercle during embryonic life. The newborn with aphallia presents as a devastating social emergency to parents and family. These are genetic males with normal testes and scrotum. There is absence of penis and urethral opening is located in the anterior rectal wall in the majority of cases. Phallic reconstruction using the free radial forearm flap (RFF) or the pedicled anterolateral thigh flap (ALTF)[1] has been routinely used in penile inadequacy or in female-to-male transsexuals. However, these reconstructions are carried out around puberty. Even if any reconstruction in infancy is carried out successfully, it is likely to cause encumberance during childhood and a further source of social embarrassment. We describe a novel technique as a temporizing procedure which would see the patient through childhood.

CASE REPORT

We describe 2 patients who presented at the age of 9 months and 1 year with penile agenesis. Both were passing urine and stools per rectum. The physical examination revealed complete absence of a phallus, a well-developed scrotum and normal sized testes. The urethral opening was located on the anterior rectal wall, about 2 cm. from the anal verge. Parents of both the children wanted them to be raised as males. The karyotype confirmed 46XY. The hormonal profile was within normal limits. The spinal radiographs were normal. An ultrasonography revealed normal kidneys and no other associated anomalies. The voiding cystourethrography revealed a short urethra with urethro-rectal communication a short distance beyond the bladder neck. While MRI confirmed the above findings, it also revealed a normal looking urinary bladder and absence of corpora cavernosa and the corpus spongiosum. Urinary bladder was seen to open lower down on the anterior rectal wall through the short posterior urethra ending approximately 2.0 cm from the anal verge. T2 weighted images revealed the prostate gland (typical hyperintensity). There was no additional, detectable structural anomaly.

TECHNIQUE

Urethral re-location

Bowel preparation is done by peglec solution (polyethylene glycol with electrolytes) administered orally for 48 hours before surgery. Under general anesthesia, the child is placed in lithotomy position. Urethral lumen is catheterized easily by inserting a #8F feeding tube through the anal verge. We use a pre-anal, anterior coronal approach, to detach and mobilize the urethral opening from the anterior rectal wall [Figure 1a]. A semilunar incision is given on the perineum, anterior to the anal verge, from 3 O’clock to 9 O’clock. The urethra-easily identified over the feeding tube-is mobilized and detached from the anterior surface of rectum. The rectal end of the opening is closed by a single layer of seromuscular, running, PDS suture.

Figure 1

(a) Pre-anal anterior coronal approach: Semilunar incision on the perineium, anterior to the anal verge, from 3 O’clock to 9 O’clock. Urethra easily identified with prior insertion of feeding tube, mobilized from anterior surface of rectum and the rectal end of opening closed in two layers (b) Quadrangular incision on the anterior surface of scrotum for phalloplasty. Note the caudal projection for configuring the prepuce

Scrotal flap-phallic reconstruction

A quadrangular flap is taken from the anterior scrotal skin and raised with its dartos layer. The two edges of the flaps are folded together and the edges sutured together to create a neophallus [Figure 1b]. The previously mobilized urethral opening is re-located in the midline, between the two scrotal halves and fixed to the root of the neophallus [Figures 2a and b]. The scrotal defect is closed in the midline configuring to a median raphe. There is no tissue loss and the adjoining tissues on the abdominal wall remain undisturbed for any possible future use. Six months later, a buccal mucosal graft can be laid on the undersurface of the neophallus [Figure 3a] and later tubularized.

Figure 2

(a) Infolding and suturing together of scrotal folds to form a ‘neo-phallus’. Please note the urethral opening in scrotal location (b) Post-operative appearance after scrotal phalloplasty

Figure 3

(a) Appearance later with buccal mucosal graft being laid on the undersurface of neo-phallus (b) One year later

DISCUSSION

Aphallia is a rare birth defect seen in 1 in 30 million births. In the past, gender re-assignment was the preferred approach.[2] However, pre- and postnatal effects of androgens on brain, cause a male-typical shift in terms of psychosocial and psychosexual development, resulting in gender dysphoria later in life. As a result, the current trend is to rear these children as males.[34]

Urethral opening is typically present lower down in the rectum in the post-sphincteric location (87%) and less commonly in the presphincteric or prostatourethral location. Both our cases had the former, typical presentation. Cystography and MRI are useful investigations to look for any associated anomaly as well as defining the anatomy. Cystography, through suprapubic route, is often difficult to perform in un-cooperative children. MRI should be the preferred modality of management as has been first demonstrated by us.[5]

Phallic reconstruction to treat aphallia is a challenging venture. Available procedures are recommended for older children[6] or after puberty. The development of microsurgical free-flap techniques made the first microsurgical phalloplasty possible using a free radial forearm flap.[178] However, there are several problems with early phalloplasty, such as, lack of further growth and the large neo-phallus being an encumberance to the young child. The microvascular techniques are more demanding at a young age and any tissue utilized earlier would hinder, any later age appropriate surgery. Also, none of these procedures provide erectile tissue. They are, anatomical phallic configurations in which, at best, penile implants could be inserted.

At the same time, postponing surgery till post-pubertal period adds to parental anxiety, affects psychosocial development of the child who continues to pass urine per rectum. Therefore, a temporizing procedure is necessary which may see the child through childhood [Figure 3b]. Scrotal phalloplasty, using scrotal skin to reconstruct a temporary phallus, can be performed easily. Urethro-rectal disconnection is easily performed by our pre-anal anterior coronal approach. It provides an age-appropriate phallic appearance and does not disturb the tissues which may be required for post-pubertal phalloplasty.

Therefore, scrotal phalloplasty serves as a temporizing procedure which may see the child through early school going years. Adding buccal mucosal graft allows urethral reconstruction and thus enables the child to micturate while standing. As he approaches teenage the other already available procedures[178] could be introduced with ease, as, scrotal phalloplasty does not encroach on tissues used for any future surgeries.