Vaginal reconstruction in female cloacal exstrophy patients.

OBJECTIVE: To present our experience of vaginal reconstruction in female cloacal exstrophy patients. PATIENTS AND METHODS: The records of 7 postpubertal female cloacal exstrophy patients (median age, 17 years; range, 11-26 years) were retrospectively reviewed. Complete duplication of the uterus and vagina was noted in all patients. All of them underwent various types of vaginoplasty at the time of urinary tract reconstruction. When a pull-through vaginoplasty was difficult, the native vagina was anastomosed to the neovagina using the large or small bowel. Alternatively, the native bladder remaining connected to the vagina was used as a neovagina with simultaneous construction of the gastroileal composite reservoir. RESULTS: The median age at vaginoplasty was 6 years (range, 5-8 years). The techniques were pull-through vaginoplasty in 1 patient, intestinal vaginoplasty in 2 patients, and vaginoplasty using the native bladder in 4 patients. Five patients required revision surgery for hematometrocolpos after the onset of puberty (median age, 14 years; range, 11-16 years). Three patients underwent an end-to-side reanastomosis of the uterus to the neovagina using the bowel or the native bladder, whereas 2 patients received a side-to-side anastomosis of the uterus to the neovagina using the native bladder. No patient developed recurrence of hematometrocolpos after the revision surgery, with a mean follow-up of 71 months. CONCLUSION: Our recent approach to vaginoplasty can be an alternative for female cloacal exstrophy patients. A side-to-side anastomosis of the uterus to the neovagina is a technically easy procedure in surgical management of hematometrocolpos.

VURD Syndrome: Report of Three Cases.

Hoover and Duckett identified the relationship between valves, reflux, and dysplasia, commonly known as VURD (Posterior urethral valve, Unilateral vesicoureteral reflux, Renal dysplasia) syndrome. They noted preserved contralateral renal function in patients with unilateral reflux into a non-refluxing kidney. The proposed mechanism of this protection is that the refluxing collecting system acts as a pressure pop-off. Here we report three cases of VURD syndrome.

Male infant with a single-system ectopic ureter that required the removal of the ureteral stump after nephrectomy.

The necessity of removing ureteral stumps after upper tract surgery for ectopic ureters has been debated, because the procedure can be difficult to carry out and might cause injury to bladder continence mechanisms. Fortunately, ureteral stumps rarely cause problems. We herein report the case of a single-system ectopic ureter that required the removal of the ureteral stump after nephrectomy. In our case, reflux into the ectopic ureter adversely affected the dilation of the ureteral stump. Although urine might flow through an ectopic opening during the voiding phase as a result of high pressure, residual urine might be unable to flow during the storage phase because of low pressure in the ureteral stump. The removal of ureteral stumps might be required in patients with reflux into ectopic ureters. When surgical intervention is necessary for patients with megaureters, cystourethroscopy should be carried out to more accurately clarify the anatomy of the lower urinary tract.

Two cases of late-diagnosed ovotesticular disorder of sex development.

Ovotesticular disorder of sex development (ovotesticular DSD) is defined as the presence of testicular and ovarian tissue in the same individual. Both external and internal genitalia of patients with ovotesticular DSD display a spectrum of phenotypes. Most children present with ambiguous genitalia in combination with unilateral or bilateral undescended gonads. We experienced two late-diagnosed children who presented with proximal hypospadias and bilateral scrotal gonads. One should consider the possibility of ovotesticular DSD when managing patients with proximal hypospadias even if both gonads are palpable in the scrotum.

Nephrectomy and upper pole heminephrectomy for poorly functioning kidney: Is total ureterectomy necessary?

OBJECTIVE: We analyzed our experience with nephrectomy and upper pole heminephrectomy combined with subtotal ureterectomy to determine the incidence of the problems associated with the distal ureteral stump. MATERIALS AND METHODS: The records of 48 children who underwent nephrectomy or upper pole heminephrectomy with subtotal ureterectomy for a poorly functioning kidney were reviewed. RESULTS: No patient who underwent nephrectomy for a poorly functioning single-system kidney required secondary surgery. No secondary surgery was required in any patient who underwent upper pole heminephrectomy for ectopic ureter, while secondary surgery was required in five of 17 patients (29%) who underwent upper pole heminephrectomy for ectopic ureterocele. All five patients presented with recurrent febrile urinary tract infection after upper pole heminephrectomy. They also had reflux into the ipsilateral lower moiety, contralateral kidney, or distal ureteral stump. Our results and data from other reports suggest that recurrent urinary tract infections after upper pole heminephrectomy were treatment outcomes based on the initial surgical approach to ectopic ureterocele rather than isolated problems associated with the distal ureteral stump. CONCLUSION: We consider that total ureterectomy is unnecessary at the time of nephrectomy or heminephrectomy for a poorly functioning kidney because the distal ureteral stump rarely causes a problem.

Familial transverse testicular ectopia unrelated to persistent Müllerian duct syndrome.

Transverse testicular ectopia (TTE) is rare. Although several familial cases of TTE have been reported, most of them were associated with persistent Mullerian duct syndrome (PMDS), which may be inherited as an autosomal-recessive or X-linked recessive trait. We experienced two brothers with TTE unrelated to PMDS. A genetic etiology may be involved even in TTE unrelated to PMDS.

Prepubertal testicular tumors: a 20-year experience with 40 cases.

Testicular tumors are rare in children. In addition, prepubertal testicular tumors (PTT) are fundamentally different from their postpubertal counterparts.We reviewed our 20-year experience with 40 cases to suggest an algorithm for the appropriate surgical approach for patients with PTT. All tumors ultrasonographically suspected of being benign were confirmed to be benign postoperatively. There was a significant difference between median preoperative a-fetoprotein (AFP) levels of infants with yolk sac tumor and teratoma. The majority of our cases were benign, with the most common histopathological subtype being teratoma, as recently reported. Testis-sparing surgery was carried out in five patients with teratoma and three patients with epidermoid cyst. Other than one patient with teratoma, none of the patients developed recurrence or testicular atrophy after testis-sparing surgery. When testis-sparing surgery is considered, our algorithm based on ultrasonographic findings and preoperative AFP levels might be helpful.

Does overgrowth of costal cartilage cause pectus excavatum? A study on the lengths of ribs and costal cartilages in asymmetric patients.

PURPOSE: The cause of pectus excavatum has been hypothesized to be overgrowth of the costal cartilage. According to this theory, the length of costal cartilages must be longer in the side of deep depression in asymmetric patients. To challenge this hypothesis, we measured the lengths of ribs and costal cartilages and investigated lateral differences. SUBJECTS AND METHODS: Twenty-four adolescent and adult patients with asymmetric pectus excavatum (14-30 years of age) with no history of surgery were investigated in this study. The fifth and sixth ribs and costal cartilages were individually traced to measure their full lengths on 3-dimensional computed tomographic (CT) images. As an index of asymmetry, sternal rotation angle was measured in the chest CT images. Patients with a 21 degrees or greater angle of sternal twist were designated as an asymmetric group and those with an angle of smaller than 20 degrees as a symmetric group. Lateral differences in the fifth and sixth costal and costal cartilage lengths were compared between the groups. RESULTS: On comparison of the costal and costal cartilage lengths in the asymmetric group, the right fifth ribs and costal cartilages were significantly shorter than the left (P = .02 and .03, respectively), and right sixth ribs were also significantly shorter than the left (P = .004), but right sixth costal cartilages were not (P = .31). In the symmetric group, the lengths of the left and right fifth ribs and costal cartilages were showing no significant difference (P = .20 and P = .80, respectively), and those of the sixth ribs and costal cartilage were also showing no significant difference (P = .97 and P = .64, respectively). DISCUSSION: The ribs and costal cartilages on the right side with severer depression were significantly shorter or not different than those on the contralateral side. Based on these findings, the theory of costal cartilage overgrowth is contradictory. The etiology of asymmetric chest deformity should be reevaluated.

Evaluation of the Nuss procedure using pre- and postoperative computed tomographic index.

BACKGROUND: Objective assessment of the chest in patients with pectus excavatum after the Nuss procedure has not been published. This study evaluated the results of the Nuss procedure using computed tomographic (CT) index (CTi). METHODS: We have performed the Nuss procedure in 382 patients since 1998, and 150 patients who underwent bar removal were included in this study. Computed tomographic scans were obtained before the Nuss procedure and after bar removal, and then preoperative CTi (pre-CTi) and postoperative CTi (post-CTi) were calculated. Computed tomographic scans of 62 age-matched patients without chest deformity were collected as controls. Patients were divided at 10 years of age into the younger and older groups, and groups with mild and severe deformity were defined using a pre-CTi value of 5 as border. These CT indices were compared and statistically analyzed. RESULTS: Mean pre-CTi in all cases was 5.97 +/- 3.31 and improved to 3.08 +/- 0.64. Postoperative CTi was not significantly different from that of the control (2.47 +/- 0.32, P = .17). In the group with mild depression, pre-CTi was 4.15 +/- 0.62, and post-CTi was 2.88 +/- 0.50. Preoperative CTi in the group with severe deformity (7.44 +/- 3.82) improved to 3.25 +/- 0.69. Postoperative CTi values between the severe and mild groups were not significantly different (P = .75). Computed tomographic index of the young group improved from 6.20 +/- 3.58 to 2.93 +/- 0.49 and in older group from 5.50 +/- 2.64 to 3.40 +/- 0.79. These 2 post-CTi values were not significantly different (P = .73). CONCLUSION: Postoperative CT scan could provide objective evaluation of sternal elevation. Mean CTi after the Nuss procedure was statically equivalent to that of the control cohort. Good sternal elevation can be achieved with the Nuss procedure regardless of the severity of chest depression or age.

Retroperitoneal ganglioneuroblastoma resected 8 years after mass screening: a case report.

An 8-year-old girl presented with abdominal tumor that was discovered incidentally. At surgery, the tumor originated from the retroperitoneal sympathetic trunk; and the histologic diagnosis was ganglioneuroblastoma, nodular (GNBn), unfavorable histology on Shimada's classification, International Neuroblastoma Staging System (INSS) stage 1. This patient was found positive for neuroblastoma (NB) by mass screening at 6 months old. There was no tumor detected, and tumor markers decreased to normal range by 18 months of age. We examined her previous computed tomographic films retrospectively and noticed a mass in the same region indicating that the tumor had been there for 8 years without treatment. This is the first report of infantile mass screening-positive NB appearing after long-term follow-up with unfavorable histology. And the follow-up interval was the longest ever reported. This case is suggestive of considering the natural history and treatment strategies for infantile NBs, and the relationship between infantile NB and ganglioneuroblastoma, nodular. It is important to follow mass screening-positive cases of NB over the long term if wait-and-see policy is adopted.

Experience in 100 cases with the Nuss procedure using a technique for stabilization of the pectus bar.

The Nuss procedure is a new, minimally invasive technique for the repair of pectus excavatum. We describe our experience with this operation, in a relatively large series in a single institution, and introduce our technical modification for stabilizing the pectus bar. In 107 patients (75 male and 32 female) with pectus excavatum who underwent the Nuss procedure, age at operation ranged from 3 to 23 years old (mean: 7.5+/-4.1) and Haller's CT index ranged from 33 to 2.6 (mean: 6.1+/-3.5). To stabilize the pectus bars, we tied the rib and both edges of the bars with surgical steel wire, 0.8 mm in diameter, using a Duchenne needle to guide the wire behind the rib. Average operating time with this technique (n=100) was 48+/-20 min. The stability of pectus bars after the operation was assessed by lateral chest X-ray films. The position of the center of the pectus bars, facing the sternum at right angles, was classified as excellent. A minimal bar displacement of less than 45 degrees was classified as incomplete. The position of the bars that had rotated 90 degrees was classified as poor. The post-operative course was uneventful in all cases except for three patients who showed wound infection, hemothorax or pneumothorax, independently. Two patients required wire removal due to skin irritation as a late complication. Five patients underwent re-operations due to bar displacement. Among them, only two cases were included in the group of wire fixation. Only five teen-aged patients required a lateral stabilizing bar. Lateral chest X-ray films of 100 patients showed that the position of the pectus bar was excellent in 86 cases, incomplete in 12 cases and poor in two cases. In 70 patients aged less than 10 years, the position in 91% was excellent and in 9% was incomplete. In 30 patients aged 10 years and older, 14 required two pectus bars and the results were excellent in all cases. The other 16 patients with a single bar showed excellent results in eight (50%) cases, incomplete in six (37.5%) and poor in two (12.5%). The wire-fixation technique was safe, effective and time efficient to perform in young cases. Teen-aged patients, however, were at high risk of bar displacement. Therefore, the use of two bars and lateral stabilizing bars are recommended for these high-risk patients.