Scrotal flap phalloplasty as temporary neophallus in infants and children with penile agenesis: Multi-institutional experience and long-term follow-up.

INTRODUCTION: Aphallia is a rare congenital disorder pertaining to genotypic males. Early surgical creation of a neophallus is recommended to reinforce the child's male gender-identity, favoring proper psychosexual development. Modern microsurgical techniques used to create a neophallus in adults are not recommended in children due to the invasiveness and complexity of the procedures, along with high complication rates. Scrotal flap phalloplasty is a simple and reproducible technique to create a temporary neophallus in prepubertal boys with aphallia. OBJECTIVE: We present a multi-institutional experience, ten years after the initial description of the scrotal flap phalloplasty (SFP) technique, in which a flap from the well-developed scrotum is used to build a temporary neophallus, without obvious scars in patients with aphallia. STUDY DESIGN: The records of surgical neophalloplasty for aphallia patients from 4 centers between 2011 and 2021 were reviewed. All patients had at least one year follow-up to assess for short and long-term complications. Age at initial operation, associated anomalies, and other related surgical procedures were analyzed. RESULTS: The post-operative aesthetic result in all patients was satisfactory and has been maintained in the long-term follow-up, with all patients presenting a cylindrical structure resembling an uncircumcised penis, without evidence of significant contraction or loss of length. (Summary Figure) DISCUSSION: Non-microsurgical neophalloplasty techniques in patients with penile agenesis are temporary procedures that help to establish the body image and preserve the psychosexual development of the patient with aphallia. These techniques do not involve tissue transplant from a distant region, and are simpler to perform, with less scarring at the donor sites. Due to significant donor scars and considerable morbidity and complexity associated with the definitive phalloplasty techniques, we created a simple, reproducible and straightforward procedure to serve as a temporary neophallus in young boys with aphallia. As affected patients usually have a well-formed scrotum with normal and orthotopic testicles, it is the ideal donor site for a temporary neo-phallus in childhood. Furthermore, other donor sites are preserved for a definitive phalloplasty. There are limitations to this study, as quality of life could not be assessed and psychological or gender-identity investigations have not been carried out. None of these children have reached puberty, and hence decision and outcomes of definitive neophallus reconstruction has not been considered to date. CONCLUSION: Scrotal flap phalloplasty is a minimally invasive, simple and reproducible technique used to create a temporary neophallus in boys with aphallia, while waiting for definitive reconstructive surgery after puberty.

Sonographic Renal Parenchymal Measurements for the Evaluation and Management of Ureteropelvic Junction Obstruction in Children.

PURPOSE: To correlate sonographic renal parenchymal measurements among patients with ureteropelvic junction obstruction (UPJO) labeled society of fetal urology (SFU) hydronephrosis grades 1-4 and to examine whether sonographic renal parenchymal measurements could be used to differentiate conservative vs. surgical management. MATERIALS AND METHODS: Retrospective chart review and sonographic renal parenchymal measurements (renal length, medullary pyramid thickness, and renal parenchymal thickness) were performed in patients with SFU grades 1-4 hydronephrosis secondary to UPJO managed between 2009 and 2014. Exclusion criteria included other concomitant genitourinary pathology or incomplete follow-up. Anterior-posterior renal pelvic diameter (APRPD) and radionuclide renography were also evaluated when available. RESULTS: One hundred four patients with UPJO underwent 244 renal and bladder ultrasound (1,464 sonographic renal parenchymal measurements in 488 kidneys). Medullary pyramid thickness and renal parenchymal thickness progressively decreased from SFU grades 1-4 (p < 0.05). A similar trend was appreciated when comparing SFU grades 1 and 2 vs. 3 and 4, as well as SFU grades 3 vs. 4 (p < 0.05). SFU grade 3 and 4 patients who underwent pyeloplasty had longer renal length in comparison to those who were managed conservatively (p < 0.02). CONCLUSION: This is the first study that evaluates these objective, quantifiable sonographic renal parenchymal measurements in children with unilateral UPJO. These sonographic renal parenchymal measurements correlate closely with worsening of hydronephrosis graded by the SFU and APRPD classification systems. Prospective studies are needed to elucidate the role of sonographic renal parenchymal measurements in the management of children with UPJO.

Feminizing genital reconstruction in congenital adrenal hyperplasia.

The past several decades have seen multiple advances in the surgical reconstruction for girls born with Disorders of Sexual Differentiation. This surgery can be technically very demanding, and must be individualized for each patient, as the degree of virilization and level of confluence of the vagina and urogenital sinus will dictate the surgical approach.In this manuscript we present our approach and experience in the surgical options for girls born with Congenital Adrenal Hyperplasia, with special attention regarding clitoroplasty, urogenital mobilization, and vaginoplasty.

Corporeal grafting for severe hypospadias: a single institution experience with 3 techniques.

PURPOSE: Correction of severe chordee by corporeal body grafting has been successfully performed using various grafts and biomaterials. We report a single institution comparison of our experience using small intestinal submucosa, tunica vaginalis and dermal grafts at stage 1 hypospadias repair. MATERIALS AND METHODS: A retrospective chart review was performed of the records of all patients who underwent staged hypospadias repair from 1985 to 2006 with corporeal body grafting at stage 1 with small intestinal submucosa, tunica vaginalis or dermal grafts. Age at grafting, time between stages, residual chordee at stage 2 repair and the need for additional plication or chordee correction at stage 2 were recorded. RESULTS: A total of 71 patients were identified with a median age of 10 months at stage 1 repair and a median of 7.6 months between stages 1 and 2 repair. Dermal grafts, tunica vaginalis and small intestinal submucosa grafts were used in 29, 21 and 20 patients, respectively. One patient received a combination of small intestinal submucosa and tunica vaginalis. None of the patients receiving tunica vaginalis graft required any further correction of chordee. One patient with a dermal graft and 1 receiving small intestinal submucosa required Nesbit plication at stage 2 repair for minor ventral chordee. One patients receiving small intestinal submucosa showed severe fibrosis at the graft site, requiring excision and repeat grafting with tunica vaginalis. This patient has been previously described. The 2 patients with small intestinal submucosa related complications had 4-ply grafts. We have seen no complications associated with 1-ply small intestinal submucosa. At limited followup we have not seen residual chordee after stage 2 repair. CONCLUSIONS: In a large group of children requiring corporeal grafting for severe chordee we observed successful chordee correction with 1-ply small intestinal submucosa, tunica vaginalis or dermal grafts.

Testosterone exacerbates obstructive renal injury by stimulating TNF-alpha production and increasing proapoptotic and profibrotic signaling.

Upper urinary tract obstruction is a common cause of renal dysfunction in children and adults. While there is clinical evidence of an increased male incidence and mortality rate with acute renal failure, the effect of gender and testosterone on obstructive renal injury has not previously been evaluated. We hypothesized that testosterone exacerbates proinflammatory TNF-alpha production and proapoptotic and profibrotic signaling during renal obstruction, resulting in increased apoptotic cell death and tubulointerstitial fibrosis. To study this, male, female, castrated male, and testosterone-treated oophorectomized female rats were subjected to sham operation or 3 days of unilateral ureteral obstruction (UUO). Renal cortical tissue was then analyzed for TNF-alpha production; proapoptotic caspase-8, -9, and -3 activity; apoptotic cell death; profibrotic transforming growth factor-beta1 production; and alpha-smooth muscle actin expression. In a separate arm, glomerular filtration rate (inulin clearance) was measured in rats pre- and post-UUO. Male and testosterone-treated oophorectomized female rats demonstrated a significant increase in TNF-alpha production, caspase activity, apoptotic cell death, tubulointerstitial fibrosis, and renal dysfunction during UUO compared with castrated males and normal female rats subjected to the same time course of obstruction. These results demonstrate that endogenous testosterone production in normal male rats and testosterone exogenously administered to oophorectomized females significantly increases TNF production and proapoptotic and profibrotic signaling during renal obstruction, resulting in increased apoptotic cell death, tubulointerstitial fibrosis, and renal dysfunction.

The role of interleukin-18 in renal injury.

Interleukin (IL)-18 is a relatively new pro-inflammatory cytokine, formerly known as interferon-gamma-inducing factor, which induces interferon-gamma production in T cells and natural killer cells. It is synthesized as a biologically inactive precursor, which requires cleavage into an active molecule by an intracellular cysteine protease similar to IL-1beta. This review examines the pro-inflammatory role of IL-18 in various types of renal injury (i.e., endotoxemia, cisplatin toxicity, allograft rejection, and ischemia-reperfusion injury) and explores the integral role of IL-12 in IL-18 function and activity.

A comparison of the Monti and Casale (spiral Monti) procedures.

PURPOSE: We present our long-term followup and comparison of outcomes between the Monti and Casale (spiral Monti) procedures in a large group of children and young adults. MATERIALS AND METHODS: A retrospective chart review was done, including all patients undergoing the Monti or Casale procedure at our institution with a minimum followup of 6 months. Age at surgery, the bowel segment used, stomal location, the number and type of revisions or endoscopic procedures required after channel creation, problems catheterizing and channel continence were documented, and a database was created. RESULTS: Of 188 patients identified with at least 6 months of followup 109 underwent a Monti procedure, while 79 underwent a Casale procedure. Patient age at surgery was 10 months to 31 years (mean 10.2 years). Mean followup was 43 months for the entire cohort, and 47.2 and 37.2 months for the Monti and Casale groups, respectively. A total of 43 open revisions were required in 36 patients (19.1%). Stomal revisions accounted for 18 procedures, while subfascial revisions accounted for 25 in 17 (9.0%) and 21 (11.2%) patients, respectively. A total of 21 endoscopic procedures requiring anesthesia were performed in 17 patients (9.0%). In the Monti group stomal revision was required in 11 patients (10.1%), while subfascial revisions were required in 9 (8.3%). In the Casale group stomal revision was required in 6 patients (7.6%), while subfascial revisions were required in 12 (15.2%). Of the channels 98% were completely continent at the stoma. CONCLUSIONS: In a large population of children and young adults we report durable and reliable long-term results with the Monti and Casale procedures, including continence at the stoma. The only significant difference noted between the 2 procedures was a higher incidence of subfascial revisions for umbilical stomas in each group. The need for subfascial revision is highest in spiral Monti channels placed in the umbilicus.

Creation of continence mechanisms (Mitrofanoff) without appendix: the Monti and spiral Monti procedures.

This is a review of the technique to create a continent catheterizable stoma using the Yang-Monti principle of a transversely tubularized segment of bowel. Pediatric urologists have widely used this technique for years, especially when the appendix is either not available or suitable for use. It provides the surgeon with a reliable, predictable, and durable method for creating a continent catheterizable channel. The indications for its use, principles of construction, and outcomes in a large population of children are described.

Pediatric urologic emergencies and urgencies.

The more common urologic problems seen in infancy and childhood that require urgent or emergent pediatric urologic referral are discussed, including a brief description of the usual presenting signs or symptoms, differential diagnoses, proper diagnostic work-up recommended before or at the time of referral, and the usual therapeutic course of management after evaluation by the urologist. These conditions include the acutely swollen scrotum, scrotal masses, penile swelling and erythema, exstrophy, hematuria, urinary retention, abdominal masses, and various genital abnormalities including interlabial masses, hypospadias with any degree of cryptorchidism, and ambiguous genitalia. This article is designed to provide the primary care practitioner with a focused review and a useful resource for managing children who have genitourinary abnormalities in the hospital or clinic setting.

TNF-alpha neutralization decreases nuclear factor-kappaB activation and apoptosis during renal obstruction.

BACKGROUND: Obstruction of the upper urinary tract is an important cause of progressive renal injury in children. While tumor necrosis factor-alpha (TNF-alpha) and nuclear factor kappaB (NFkappaB) have independently been implicated in the pathophysiology of this process, TNF-alpha's role in obstruction-induced NFkappaB activation has not previously been investigated. MATERIALS AND METHODS: To study this, male Sprague Dawley rats were subjected to 3 days of unilateral ureteral obstruction (UUO) versus sham operation. Twenty-four hours prior to surgery and 2 days after, rats received either a vehicle or a pegylated form of soluble TNF receptor type 1 (PEG-sTNFR1). The kidneys were harvested 3 days postoperatively, and tissue samples were analyzed for TNF-alpha expression (ELISA), NFkappaB activation (EMSA, immunohistochemistry), IkappaB degradation (Western blot), angiotensinogen expression (Western blot), and apoptosis (TUNEL). RESULTS: Renal cortical TNF-alpha levels, NFkappaB activation, IkappaB degradation, angiotensinogen expression, and apoptotic cell death were significantly increased in response to obstruction. In contrast, TNF-alpha neutralization significantly reduced obstruction-induced TNF-alpha production, NFkappaB activation, IkappaB degradation, angiotensinogen expression, and renal tubular cell apoptosis. CONCLUSION: TNF-alpha's potent pro-inflammatory and cytotoxic effect during renal obstruction is directed through NFkappaB activation via increased IkappaB-alpha phosphorylation. As the role of TNF-alpha and NFkappaB in renal obstruction are further defined, the development of therapeutic strategies that limit or prevent obstruction-induced renal injury may be realized.

Metastatic seminoma presenting with pulmonary embolus, inferior vena caval thrombosis, and gastrointestinal bleeding.

Testicular cancer metastasis to the retroperitoneum can be associated with a variety of complications due to obstruction or invasion of adjacent structures. In this case report, we present an unusual patient with invasion of the duodenum from germ cell cancer and discuss the management of this challenging scenario.

Serendipitous renal cell carcinoma in the post-CT era: continued evidence in improved outcomes.

PURPOSE: To compare patient and tumor characteristics, including survival data, between serendipitous and non-serendipitously discovered renal cell carcinoma (RCCA) in an era of more frequent use of CT scanning and ultrasonography. MATERIALS AND METHODS: The Tumor Registry of the Audie L. Murphy VA Hospital in San Antonio, TX, was reviewed for new diagnoses or initial treatment of RCCA from January 1985 through December 1999. Records were evaluated as to whether the initial diagnosis of RCCA was made serendipitously. Prognostic and epidemiological variables, were collected and disease-specific and overall survival data were computed. RESULTS: Of 257 patients with RCCA, 93 (36.2%) presented with serendipitously discovered tumors and 100 presented with metastases at diagnosis. Mean tumor size was smaller in the serendipitous group, compared both pathologically (6.74 cm vs. 4.49 cm, P < 0.0001) and by radiographic measurement (8.04 cm vs. 4.87 cm, P < 0.0001). Sixty-six (71%) of 93 serendipitously discovered tumors were Stage I at diagnosis, vs. only 30 (18.4%) of 163 non-serendipitous tumors (P < 0.0001). When non-serendipitous tumors with metastatic disease at presentation were excluded, the percentage of patients with Stage I disease was lower than for serendipitous tumors (46.8% vs. 71%, P = 0.004). Pathologically confirmed tumor stage was more favorable for serendipitously discovered tumors: 40 of 77 (60%) non-serendipitous tumors were