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Re: Willihnganz-Lawson et al.: De Castro Technique Used to Create Neophallus: A Case of Aphallia (Urology 2012;79:1149-1151)
Letters to the Editor Re: Willihnganz-Lawson et al.: De Castro Technique Used to Create Neophallus: A Case of Aphallia (Urology 2012;79:1149-1151) TO THE EDITOR:
We read the article “De Castro technique used to create neophallus: a case of aphallia.”1 The occurrence of severe genitourinary abnormalities culminating in renal failure with postsphincteric perineal urethral orifice1 is, however, not in accordance with literature.2 It would be pertinent to know if the authors have an embryologic explanation for the same. In this letter, we differ with the results of De Castro phalloplasty1,3 and substantiate it by reporting our experience of managing a child with De Castro phalloplasty and its 2.5-year follow-up. A 3-month-old child with 46XY karyotype presented with aphallia and passage of urine from the anus since birth. This was accompanied with high-grade fever along with excessive crying and straining at micturition. Testes were bilaterally descended in a well-developed scrotum.
The renal function tests were normal. The urine culture sensitivity revealed heavy growth of Escherichia coli. The stenosed perineal urethral orifice opened in the anterior wall of the anorectum. Ultrasonography of the abdomen showed a small left kidney with moderate hydro-ureteronephrosis and an irregularly thickened urinary bladder. Renal scintigraphy revealed impaired cortical function, renal scars, and slow clearance on the left side with near-normal function on the right side. He was operated by anterior sagittal trans-scrotal approach in a lithotomy position. The urethral opening was separated from the anorectum and transposed at the penoscrotal junction as perineal urethrostomy (Fig. 1A). De Castro phalloplasty with 4 ⫻ 5-cm lower abdominal quadrangular flap3 was performed with suprapubic cystostomy (Fig. 1A). A postoperative cystogram showed a left grade V vesicoureteral reflux with patent perineal urethrostomy. He was discharged on antibiotic prophylaxis and anticholinergic with a straight phallic length of 4-5 cm. Two-months later, he presented with stenosis of the perineal urethrostomy and blackening of the distal 2 cm of neophallus. The perineal urethrostomy was revised. At the last follow-up, he was 2.5 years old, had urinary and
Figure 1. (A) Operative photograph showing lower abdominal quadrangular flap De Castro phalloplasty depicting the neophallus and perineal urethrostomy at the penoscrotal junction. (B) Residual neophallus at 2.5 years postoperatively with perineal urethrostomy, white arrow indicates the site of perineal urethrostomy at the penoscrotal junction. © 2012 Elsevier Inc. All Rights Reserved
0090-4295/12/$36.00 http://dx.doi.org/10.1016/j.urology.2012.06.056
1165
fecal continence, and was thriving well. The neophallus had regressed from straight phallic length of 5 cm to 2 cm (Figure 1B). The surgical scar at the base of the neophallus tends to hold it up as a pseudosuspensory ligament. The reports of gender dysphoria in genetic males reared as females, lead to a paradigm shift in the management of aphallia.4 Male gender assignment in aphallia includes a daunting task of phalloplasty. De Castro phalloplasty1,3 provides an easy and viable alternative of palliative phalloplasty in childhood until the patient can undergo the definitive repair at puberty.1,3 Although immediate results of this technique are gratifying,1,3 the long-term results in our patient revealed regression of the neophallus to nearly 50% of the original size. This probably could be because of the poor vascularity of the skin flap. For improvement of results, in retrospect, we suggest (1) preoperative mapping of origin and distribution of superficial inferior epigastric vessels,5 (2) to have a tension-free closure, the skin flaps should be marked by pinching the lower abdominal skin with knees and hip flexed, and (3) the skin flap for phalloplasty should be an isosceles trapezoid with the base being longer than the top. Archana Puri, M.B.B.S., M.S., M.Ch. Rahul Saxena, M.B.B.S., M.S. Department of Paediatric Surgery Lady Hardinge Medical College and Kalawati Saran Children’s Hospital New Delhi, India References 1. Willihnganz-Lawson KH, Malaeb BS, Shukla AR. De Castro technique used to create neophallus: a case of aphallia. Urology. 2012; 79:1149-1151. 2. Skoog SJ, Belman AB. Aphallia: its classification and management. J Urol. 1989;141:589-592. 3. 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; [Discussion:1117]. 4. Reiner WG. Gender identity and sex-of-rearing in children with disorders of sexual differentiation. J Pediatr Endocrinol Metab. 2005; 18:549-553. 5. Fukaya E, Kuwatsuru R, Iimura H, et al. Imaging of the superficial inferior epigastric vascular anatomy and preoperative planning for the SIEA flap using MDCTA. J Plast Reconstr Aesthet Surg. 2011;64:63-68.
Reply by the Authors TO THE EDITOR:
We welcome this opportunity to continue dialogue on the increasing experience with penile reconstruction using the anterior abdominal wall flap. The patient discussed in our report presented with aphallia associated with a wide array of genitourinary malformations— en1166
tirely consistent with the well-described proportionality of urinary malformations with proximal meatal location (reference 2 in letter). Although our article did not declare sphincteric association, the perineal fistula in our case opened just distal to the anal sphincteric complex. We concur with the correspondents that early distal necrosis and moderate contraction of the initially lengthy neophallus is a valid perioperative concern, but we posit that the rescission in length—if destined to manifest— occurs rapidly, followed by flap stability and longer-term viability. Beyond that, the salutary cosmetic outcomes and familial satisfaction are encouraging enough to appropriate a significant role for this procedure in the armamentarium for treating aphallia. Aseem R. Shukla, M.D. Division of Pediatric Urology Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Katie Willihnganz-Lawson, M.D. University of Minnesota Amplatz Children’s Hospital Veterans Affairs Medical Center Urology and Pediatric Urology Minneapolis, Minnesota
Re: Penbegül et al.: Safety and Efficacy of Ultrasound-Guided Percutaneous Nephrolithotomy for Treatment of Urinary Stone Disease in Children (Urology 2012; 79:1015-1019) TO THE EDITOR:
We read with interest the report on the safety and efficacy of ultrasound-guided percutaneous nephrolithotomy (PCNL) for the treatment of urinary stone disease in children. The authors conclude that PCNL can be safely performed with ultrasound guidance, providing the advantages of less radiation exposure, less adjacent organ injury, and similar success and complication rates compared with fluoroscopic guidance. We believe that color Doppler could have been added to ultrasonography to create the access during puncture to distinguish the important renal vascular branches and thus avoid vascular injury and decrease the incidence of hemorrhage.1 Vascular injury of renal parenchyma can be minimized when puncture is through the avascular Brödel line/plane, which is difficult to distinguish using B-mode ultrasonography. However, this plane can be adequately displayed using Doppler ultrasonography.2 Most recent ultrasound machines have color Doppler built in to facilitate its use, when needed. UROLOGY 80 (5), 2012
We read the article “De Castro technique used to create neophallus: a case of aphallia.”1 The occurrence of severe genitourinary abnormalities culminating in renal failure with postsphincteric perineal urethral orifice1 is, however, not in accordance with literature.2 It would be pertinent to know if the authors have an embryologic explanation for the same. In this letter, we differ with the results of De Castro phalloplasty1,3 and substantiate it by reporting our experience of managing a child with De Castro phalloplasty and its 2.5-year follow-up. A 3-month-old child with 46XY karyotype presented with aphallia and passage of urine from the anus since birth. This was accompanied with high-grade fever along with excessive crying and straining at micturition. Testes were bilaterally descended in a well-developed scrotum.
The renal function tests were normal. The urine culture sensitivity revealed heavy growth of Escherichia coli. The stenosed perineal urethral orifice opened in the anterior wall of the anorectum. Ultrasonography of the abdomen showed a small left kidney with moderate hydro-ureteronephrosis and an irregularly thickened urinary bladder. Renal scintigraphy revealed impaired cortical function, renal scars, and slow clearance on the left side with near-normal function on the right side. He was operated by anterior sagittal trans-scrotal approach in a lithotomy position. The urethral opening was separated from the anorectum and transposed at the penoscrotal junction as perineal urethrostomy (Fig. 1A). De Castro phalloplasty with 4 ⫻ 5-cm lower abdominal quadrangular flap3 was performed with suprapubic cystostomy (Fig. 1A). A postoperative cystogram showed a left grade V vesicoureteral reflux with patent perineal urethrostomy. He was discharged on antibiotic prophylaxis and anticholinergic with a straight phallic length of 4-5 cm. Two-months later, he presented with stenosis of the perineal urethrostomy and blackening of the distal 2 cm of neophallus. The perineal urethrostomy was revised. At the last follow-up, he was 2.5 years old, had urinary and
Figure 1. (A) Operative photograph showing lower abdominal quadrangular flap De Castro phalloplasty depicting the neophallus and perineal urethrostomy at the penoscrotal junction. (B) Residual neophallus at 2.5 years postoperatively with perineal urethrostomy, white arrow indicates the site of perineal urethrostomy at the penoscrotal junction. © 2012 Elsevier Inc. All Rights Reserved
0090-4295/12/$36.00 http://dx.doi.org/10.1016/j.urology.2012.06.056
1165
fecal continence, and was thriving well. The neophallus had regressed from straight phallic length of 5 cm to 2 cm (Figure 1B). The surgical scar at the base of the neophallus tends to hold it up as a pseudosuspensory ligament. The reports of gender dysphoria in genetic males reared as females, lead to a paradigm shift in the management of aphallia.4 Male gender assignment in aphallia includes a daunting task of phalloplasty. De Castro phalloplasty1,3 provides an easy and viable alternative of palliative phalloplasty in childhood until the patient can undergo the definitive repair at puberty.1,3 Although immediate results of this technique are gratifying,1,3 the long-term results in our patient revealed regression of the neophallus to nearly 50% of the original size. This probably could be because of the poor vascularity of the skin flap. For improvement of results, in retrospect, we suggest (1) preoperative mapping of origin and distribution of superficial inferior epigastric vessels,5 (2) to have a tension-free closure, the skin flaps should be marked by pinching the lower abdominal skin with knees and hip flexed, and (3) the skin flap for phalloplasty should be an isosceles trapezoid with the base being longer than the top. Archana Puri, M.B.B.S., M.S., M.Ch. Rahul Saxena, M.B.B.S., M.S. Department of Paediatric Surgery Lady Hardinge Medical College and Kalawati Saran Children’s Hospital New Delhi, India References 1. Willihnganz-Lawson KH, Malaeb BS, Shukla AR. De Castro technique used to create neophallus: a case of aphallia. Urology. 2012; 79:1149-1151. 2. Skoog SJ, Belman AB. Aphallia: its classification and management. J Urol. 1989;141:589-592. 3. 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; [Discussion:1117]. 4. Reiner WG. Gender identity and sex-of-rearing in children with disorders of sexual differentiation. J Pediatr Endocrinol Metab. 2005; 18:549-553. 5. Fukaya E, Kuwatsuru R, Iimura H, et al. Imaging of the superficial inferior epigastric vascular anatomy and preoperative planning for the SIEA flap using MDCTA. J Plast Reconstr Aesthet Surg. 2011;64:63-68.
Reply by the Authors TO THE EDITOR:
We welcome this opportunity to continue dialogue on the increasing experience with penile reconstruction using the anterior abdominal wall flap. The patient discussed in our report presented with aphallia associated with a wide array of genitourinary malformations— en1166
tirely consistent with the well-described proportionality of urinary malformations with proximal meatal location (reference 2 in letter). Although our article did not declare sphincteric association, the perineal fistula in our case opened just distal to the anal sphincteric complex. We concur with the correspondents that early distal necrosis and moderate contraction of the initially lengthy neophallus is a valid perioperative concern, but we posit that the rescission in length—if destined to manifest— occurs rapidly, followed by flap stability and longer-term viability. Beyond that, the salutary cosmetic outcomes and familial satisfaction are encouraging enough to appropriate a significant role for this procedure in the armamentarium for treating aphallia. Aseem R. Shukla, M.D. Division of Pediatric Urology Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Katie Willihnganz-Lawson, M.D. University of Minnesota Amplatz Children’s Hospital Veterans Affairs Medical Center Urology and Pediatric Urology Minneapolis, Minnesota
Re: Penbegül et al.: Safety and Efficacy of Ultrasound-Guided Percutaneous Nephrolithotomy for Treatment of Urinary Stone Disease in Children (Urology 2012; 79:1015-1019) TO THE EDITOR:
We read with interest the report on the safety and efficacy of ultrasound-guided percutaneous nephrolithotomy (PCNL) for the treatment of urinary stone disease in children. The authors conclude that PCNL can be safely performed with ultrasound guidance, providing the advantages of less radiation exposure, less adjacent organ injury, and similar success and complication rates compared with fluoroscopic guidance. We believe that color Doppler could have been added to ultrasonography to create the access during puncture to distinguish the important renal vascular branches and thus avoid vascular injury and decrease the incidence of hemorrhage.1 Vascular injury of renal parenchyma can be minimized when puncture is through the avascular Brödel line/plane, which is difficult to distinguish using B-mode ultrasonography. However, this plane can be adequately displayed using Doppler ultrasonography.2 Most recent ultrasound machines have color Doppler built in to facilitate its use, when needed. UROLOGY 80 (5), 2012