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A phallia, or the congenital absence of the penis, is a rare abnormality. Neophallus creation presents one of the most challenging reconstructive cases. This technique relies on an abdominal skin flap in continuity with its adjacent blood supply.
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De Castro Technique Used to Create Neophallus - a case of aphalia - Willihnganz-Lawson.pdf
A phallia, or the congenital absence of the penis, is a rare abnormality. Neophallus creation presents one of the most challenging reconstructive cases. This technique relies on an abdominal skin flap in continuity with its adjacent blood supply.
A phallia, or the congenital absence of the penis, is a rare abnormality. Neophallus creation presents one of the most challenging reconstructive cases. This technique relies on an abdominal skin flap in continuity with its adjacent blood supply.
Create Neophallus: A Case of Aphallia Katie H. Willihnganz-Lawson, Bahaa S. Malaeb, and Aseem R. Shukla We report on a rare case of aphallia in an X, Y-born male treated at our institution. The child underwent phalloplasty at 5 years of age using the De Castro phalloplasty technique and an abdominal skin ap. Although moderate distal necrosis was seen, the overall immediate and 12-month postoperative results were highly encouraging for our patient. Thus, this technique should be seen as a temporizing reconstructive option for patients with aphallia. Long-term follow-up is necessary to determine whether the neophallic size augments with axial growth and the need for additional intervention to facilitate onset of sexual activity. UROLOGY 79: 11491151, 2012. Published by Elsevier Inc. A phallia, or the congenital absence of the penis, is a rare abnormality. It has been documented in the published data in 100 cases. 1 Aphallia is a sporadic anomaly of the genital tubercle, usually associated with a normal male karyotype and no abnormalities in gonadal development or sexual hormonal metabolism. 1,2 Because the penis plays an important role in urinary function, fertility, and psychosocial development, its ab- sence can be a devastating condition for the patient and his parents, both physically and psychologically. Neo- phallus creation presents one of the most challenging re- constructive cases for the pediatric urologist, typically re- quiring the use of a radial forearm or other types of musculocutaneous free ap transfers, with varying results. 3 De Castro et al 4 recently described a novel phallo- plasty that relies on an abdominal skin ap in continuity with its adjacent blood supply. This procedure is pre- sented as less complex than free aps and not requiring intensive multidisciplinary effort. We recently used this technique in the care of a boy with classic aphallia and present the rst experience with the abdominal ap tech- nique after De Castro et al, 4 as well as our intermediate- term results. CASE REPORT The boy was born prematurely at 36 weeks gestation by normal spontaneous vaginal delivery in February 2005. His prenatal course had been complicated by oligohy- dramnios, and on initial examination the infant was found to have aphallia with a normal scrotum, bilateral palpable testes and a very small perineal stula for a urethra. Additional imaging studies found a multicystic horseshoe kidney with abnormal architecture and 2 dis- torted collecting systemssuggesting a multicystic dys- plastic kidney. No ureters or bladder were identied. The infant was diagnosed with renal failure, and peritoneal dialysis was begun shortly after birth. At 22 months of age, the boy underwent a cadaveric renal transplant with cutaneous ureterostomy for diver- sion. After multiple episodes of stenosis of the uretero- cutaneous anastomosis, at 30 months old, he underwent takedown of the ureterostomy and creation of a catheter- izable stoma from terminal ileum and a modied Florida pouch using the right and transverse colon. 5 At about 5 years of age, the patient started to become aware of the anatomic differences between him and the other boys his age, and the situation of aphallia became distressing for his family. After reviewing the different treatment op- tions, the family agreed to proceed with construction of a neophallus using the De Castro abdominal ap tech- nique. 4 To begin the procedure, the child was placed in the supine position with the lower extremities abducted in the frog-legged position, and the abdominal wall skin was measured below a previous midline scar (Fig. 1). A quadrangular skin ap of 6 cm 7 cm based on the super- cial epigastric vasculature was marked. The marked skin lines were then taken down sharply through subcutane- ous fat to the aponeurosis of the external oblique. Next, taking care to avoid injury to the spermatic cord structures on either side of the skin ap, additional dis- section was completed. The edges of the skin incision were raised laterally and proximally to ensure closure of the abdominal wall incision, which was performed by everting mattress sutures in a Y-shaped plasty, using 4-0 polydiaxanone sutures at the points of tension. The skin ap was then rolled over a 14F nonlatex catheter and closed in 2 layers: 4-0 polyglactin suture for the inner layer and 5-0 polydiaxanone for the outer layer (Fig. 2). The phallic tip was tapered to create a conical From the Division of Pediatric Urology, Univeristy of Minnesota Amplatz Childrens Hospital, Department of Urology, University of Minnesota Medical Center, Minneap- olis, Minnesota Reprint requests: Aseem R. Shukla, M.D., Division of Pediatric Urology, Univeristy of Minnesota Amplatz Childrens Hospital, Department of Urology, University of Minnesota Medical Center, 420 Delaware Street Southeast, MMC 394, Minneapolis, MN 55455. E-mail: shukl011@umn.edu Submitted: September 13, 2011, received (with revisions): October 5, 2011 Published by Elsevier Inc. 0090-4295/12/$36.00 1149 doi:10.1016/j.urology.2011.10.004 glanular shape. In an attempt to reconstruct the coronal margin, a circumferential incision was taken down to the level of the dermis, but not beyond, to avoid injury to the subdermal plexus. Everting vertical mattress sutures were then used to close the circumferential incision. At the end of the procedure, the phallus measured approximately 8 cm (Fig. 3). The 14F tube was left in the phallus to act as a wound drain. At his 12-month follow-up visit, 3 cm of the distal neophallus had regressed and the conical, glanular shape was not fully preserved. Still, with an age-appropriate 5-cm phallus, the patient and parents were highly satis- ed and aware of the potential need for reconstruction in the future. COMMENT Aphallia is an extremely rare disorder, with an incidence of about 1 in 10-30 million births. 1 The typical pheno- type is associated with a normal-appearing scrotum, func- tioning testes, and the absence of a penile shaft. The anus is usually displaced anteriorly, with the urethral opening at the anal verge usually adjacent to a small skin tag. In some cases, the urethra might connect to the rectum. 1 Aphallia can also be associated with other genitourinary abnormalities, such as cryptorchidism, vesicoureteral re- ux, horseshoe kidney, renal agenesis, imperforate anus, and other abnormalities of musculoskeletal and cardio- pulmonary systems. 2 A review of 60 cases by Skoog and Belman 6 found that the more proximal the urethral me- atus, the greater the incidence of other anomalies and the greater the likelihood of neonatal death. Historically, gender reassignment was considered the most appropriate choice for children with aphallia. The need for a full evaluation by a team dedicated to disorders of sexual differentiation is clear, and a full psychosocial and endocrinologic evaluation is mandatory. Male as- signment might be prudent, because studies have shown that most of these patients have a male gender identity despite reconstruction as a femalepresumably inu- enced by prenatal and early postnatal androgen imprint- ing. 7 Reiner 8 found that genetic males with male-typical prenatal androgen effects had a high likelihood of recog- nizing a male sexual identity in the absence of a penis or in the presence of a vulva with disorders of sexual differ- entiation. Therefore, patients with aphallia should be raised as males despite limited experience with phallo- plasty. Phalloplasty for traumatic penile amputation has been reported in both adolescents and children using various techniques. 9 However, most involve a complicated pro- cedure requiring a skilled team of physicians, performed only at a few highly specialized centers. A case review by De Castro et al 4 described 4 postpubertal children with aphallia who underwent reconstruction using a quadran- gular abdominal skin ap for phalloplasty, followed by urethroplasty with either a bladder or buccal free graft. This technique is indicated only for patients without a midline scar from previous surgery. An otherwise reason- able alternative for patients with a midline scar is the Figure 1. Aphallia with normal scrotum. For neophallus creation, skin ap based on supercial epigastric vascula- ture marked below previous midline scar. Figure 2. Skin ap rolled over 14F nonlatex catheter and closed in 2 layers. Figure 3. Circumferential incision closed by everting verti- cal mattress sutures to simulate look of coronal margin. Phallus length was 8 cm. 1150 UROLOGY 79 (5), 2012 pedicle island groin ap, as described by Perovic. 3 An anterior-sagittal-transanorectal approach by De Castro et al 4 was used to separate the urethra from the rectum. Our patient had no functional bladder or urethra; thus, a buccal mucosa-based inner lining for the ap was not required. Although the surgical outcome we have described is not denitivea functional phallus will require placement of a penile prosthesis and perhaps additional augmentation the creation of a simple, cosmetically acceptable, vascular- ized neophallus can dissipate some of the profound psycho- social ramications of the devastating anomaly of aphallia. CONCLUSIONS To our knowledge, we present the rst case of neophallus reconstruction using the De Castro ap after the pilot description in 2007. A larger experience and longer fol- low-up is needed to evaluate the long-term potential of this technique, viability at onset of sexual activity and need for follow-up. 4 Our experience corroborates the initial experience, ease of the technique, immediate psy- chosexual benets, and extended viability of the neo- phallus. We continue to await longer term outcome re- sults to assess its versatility for additional reconstruction as the children grow. References 1. Evans JA, Erdile LB, Greenberg CR, et al. Agenesis of the penis: patterns of associated malformations. Am J Med Genet. 1999;84:47-55. 2. Palmer J. Abnormalities of the external genitalia in boysaphallia. In: Wein MD, ed. Campbell-Walsh Urology, vol. 4, 10th ed. Phila- delphia: Elsevier Saunders; 2011:3543-3544. 3. Perovic S. Phalloplasty in children and adolescents using the ex- tended pedicle island groin ap. J Urol. 1995;154:848-853. 4. De Castro R, Merlini E, Rigamonti W, et al. Phalloplasty and urethroplasty in children with penile agenesis: preliminary report. J Urol. 2007;177:1112-1116. 5. Lockhart JL, Powsang JM, Persky L, et al. A continent colonic urinary reservoir: the Florida pouch. J Urol. 1990;144:864-867. 6. Skoog SJ, Belman AB. Aphallia: its classication and management. J Urol. 1989;141:589-592. 7. Diamond M, Kaul K, Ignatoff J. Pediatric management of ambiguous and traumatized genitalia. J Urol. 1999;162:1021-1228. 8. Reiner WG. Gender identity and sex-of-rearing in children with disorders of sexual differentiation. J Pediatr Endocrinol Metab. 2005; 18:549-553. 9. Selvaggi G, Bander A. Penile reconstruction/formation. Curr Opin Urol. 2008;18:589-597. UROLOGY 79 (5), 2012 1151