No AccessJournal of UrologyPediatric Urology1 Jan 2005ACUTE RENAL FAILURE IN A MONOZYGOTIC TWIN PRESENTING WITH CLOACAL EXSTROPHY MICHAEL E. KARELLAS, JOHN M. GATTI, and JPATRICK MURPHY MICHAEL E. KARELLASMICHAEL E. KARELLAS More articles by this author , JOHN M. GATTIJOHN M. GATTI More articles by this author , and JPATRICK MURPHYJPATRICK MURPHY More articles by this author View All Author Informationhttps://doi.org/10.1097/01.ju.0000148422.02693.57AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "ACUTE RENAL FAILURE IN A MONOZYGOTIC TWIN PRESENTING WITH CLOACAL EXSTROPHY." The Journal of Urology, 173(1), pp. 230–231 References 1 : Embryology of the cloaca and embryogenesis of anorectal malformations. Birth Defects Orig Artic Ser1988; 24: 177. Google Scholar 2 : The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). Birth Defects Orig Artic Ser1978; 14: 253. Google Scholar 3 : Normal pulmonary function in a monoamniotic twin discordant for bilateral renal agenesis: report and review. Am J Med Genet1997; 73: 76. Google Scholar From the Children's Mercy Hospital and Clinics, Kansas City, Missouri© 2005 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 173Issue 1January 2005Page: 230-231 Advertisement Copyright & Permissions© 2005 by American Urological Association, Inc.Keywordsbladder exstrophytwins, monozygotickidney failurecloacaMetricsAuthor Information MICHAEL E. KARELLAS More articles by this author JOHN M. GATTI More articles by this author JPATRICK MURPHY More articles by this author Expand All Advertisement PDF downloadLoading ...

Objectives. To review our experience with hypospadias complications (seen after 10%-15% of repairs) and to identify factors influencing outcome.Methods. We reviewed the available medical records of 113 patients who underwent repeat operation for hypospadias complications. Of the 113 patients, 40% had undergone the initial repair at our institution (internal referral); 60% had undergone the initial repair elsewhere before referral (external referral). The variables potentially affecting outcome were reviewed, including the severity of the defect, concomitant disease, age at the initial operation and revisions, type of complications and treatment, and the number of revisions. Outcomes were compared on the basis of specialty and experience.Results. Isolated hypospadias was present in 81% and other genitourinary abnormalities in 10%. External referral patients were older at the first revision (7.3 versus 4.2 years, P = 0.027). Complications included fistula (73%), stricture (12%), breakdown of repair (10%), and diverticulum formation (11%). Successful revision was independent of the initial defect. The first, second, and third revision was successful in 77%, 64%, and 67% of patients, respectively. The cumulative success rate was 77%, 92%, and 97% after each respective repair attempt. The success of the repair was independent of the patient's age at the initial operation/revision and of the interval from the initial repair to reoperation. Internal referral and external referral patients had similar results. Specific experience with the repair of hypospadias complications correlated with a successful outcome (P < 0.001).Conclusions. Complications after hypospadias repairs are common, with fistula accounting for approximately 75%. The outcome in our series was independent of hypospadias severity, patient age at repair, number of revisions, stent use, and referral status. Repairs, performed by an experienced pediatric urologist were associated with improved outcomes (P < 0.001). (c) 2005 Elsevier Inc.

PURPOSE:Children with a wet, draining, or infected umbilicus are often referred to pediatric surgeons. Unfortunately, uniform guidelines regarding diagnostic imaging are lacking. Historically, the persistence of the urachus was attributed to intrauterine distal urinary obstruction. Today, many surgeons continue to advocate preoperative voiding cystourethrogram (VCUG). METHODS:Records of children with urachal abnormalities over the past 10 years were reviewed. Demographics, presentation, imaging, genitourinary anomalies, operations, length of stay, and complications were recorded. Statistical evaluation was by descriptive analysis. RESULTS:Fifty-six children were diagnosed with urachal anomalies. Age at operation was 2.5 years (1 day-13 years). Fifty percent of patients were less than 1 year. Ultrasound was used in 88% of cases. Voiding cystourethrogram (34%) and computed tomography (14%) were also used. Average hospitalization was 1.9 (0-13) days. Thirty-two percent underwent operations as outpatients. Seven percent developed wound infections. Eight children (14%) had genitourinary anomalies. However, no VCUG examination (n = 19) documented an obstructive process. CONCLUSIONS:The current study represents the largest reported series of symptomatic urachal anomalies in children. Disorders of the urachus are variable in presentation with the diagnosis reliably made by history and ultrasound alone. Further testing, including VCUG, is not warranted, adding additional cost, an invasive procedure, and inconvenience to the child.

PURPOSETraditional laparoscopic procedures use expensive cannulas to facilitate the insertion and removal of laparoscopic instruments. We report our experience with limited access stab incisions for the insertion of instruments into the peritoneal cavity during laparoscopic urological procedures to minimize the use of disposable cannulas.MATERIALS AND METHODSAll patients undergoing laparoscopic urologic procedures using stab incisions, as performed by us, from November 1999 through March 2003 were included. Procedures included nephrectomy, partial nephrectomy, varicocelectomy, nephroureterectomy, orchiopexy and adrenal procedures. A single cannula was used for telescope access. In select cases additional cannulas were used for unique instruments or specimen manipulation/extraction. Abdominal wall stab incisions were used for the remaining instruments. Stab incisions were closed with a Steri-Strip (3M Healthcare, St. Paul, Minnesota) at the skin level only.RESULTSA total of 53 procedures were performed during the study period. Pneumoperitoneum was maintained in all cases. There were no complications associated with the use of stab incisions. A total of 105 cannulas were saved using our technique. At a cost to the patient of dollars 140 per cannula the overall cost saving was dollars 14,700 with an average saving of dollars 277 per case.CONCLUSIONSLaparoscopic urological procedures can be performed effectively and safely with stab incisions for instrument access. There are significant cost savings related to the elimination of cannulas. We believe that our technique of stab incisions for instrument access is equivalent to the traditional cannula approach and should be used when possible.

No AccessJournal of UrologyPediatric Urology1 Sep 2004PRENATAL DIAGNOSIS OF BLADDER EXSTROPHY BY 3-DIMENSIONAL ULTRASOUND APOSTOLOS EVANGELIDIS, J. PATRICK MURPHY, and JOHN M. GATTI APOSTOLOS EVANGELIDISAPOSTOLOS EVANGELIDIS More articles by this author , J. PATRICK MURPHYJ. PATRICK MURPHY More articles by this author , and JOHN M. GATTIJOHN M. GATTI More articles by this author View All Author Informationhttps://doi.org/10.1097/01.ju.0000135595.83972.6aAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "PRENATAL DIAGNOSIS OF BLADDER EXSTROPHY BY 3-DIMENSIONAL ULTRASOUND." The Journal of Urology, 172(3), p. 1111 References 1 : Exstrophy, epispadias, and other bladder anomalies. In: . Philadelphia: W. B. Saunders Co.2002: 2138. sect. IX, chapt. 61. Google Scholar 2 : Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol1995; 85: 961. Google Scholar 3 : Implications of prenatal ultrasound screening in the incidence of major genitourinary malformations. J Urol2001; 165: 1677. Link, Google Scholar 4 SonoPortal. Available at: http://www.sonoportal.net/ Google Scholar From the Department of Surgery, Children’s Mercy Hospital, Kansas City, Missouri© 2004 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 172Issue 3September 2004Page: 1111 Advertisement Copyright & Permissions© 2004 by American Urological Association, Inc.Keywordsbladder exstrophyultrasonography, prenatalprenatal diagnosisMetricsAuthor Information APOSTOLOS EVANGELIDIS More articles by this author J. PATRICK MURPHY More articles by this author JOHN M. GATTI More articles by this author Expand All Advertisement PDF downloadLoading ...

We report a case of an incomplete bladder duplication diagnosed by prenatal ultrasonography at 35 weeks of gestation. The patient was followed until bladder neck obstruction and high-grade vesicoureteral reflux of a solitary kidney prompted definitive repair at 6 months of age. Bladder duplication is a rare anomaly. To our knowledge this is the first documented case of an incomplete bladder duplication discovered antenatally.

BACKGROUND/PURPOSE:Intestinal atresia occurs in approximately 10% to 20% of children with gastroschisis and may be missed at the initial closure if a thick peel obscures the bowel. Some investigators have identified intestinal atresia as a significant contributor to morbidity and mortality. The authors reviewed their experience with gastroschisis and intestinal atresia in an attempt to answer the following questions. What is the incidence of this association? How often is the intestinal atresia unrecognized as a result of the peel? What is the optimal management for infants with atresia and gastroschisis, and does the atresia affect morbidity or mortality?METHODS:The hospital charts and medical records of all patients with gastroschisis treated at our institution from 1969 to present were reviewed thoroughly. Parameters analyzed included gestational age (GA), birth weight (BW), antenatal diagnosis, mode of delivery, type of closure, era of repair, presence of other major anomalies, and development of necrotizing enterocolitis. Morbidity and mortality rates were examined. Characteristics of patients with and without atresia were compared. Chi-squared was used for crosstabular analysis. Sample parameters were compared with Student's t test. P values of less than.05 were considered significant.RESULTS:A total of 199 babies had gastroschisis and 25 (12.6%) had intestinal atresia. Intestinal atresia was initially unrecognized in 3 patients. Most patients (80%) underwent primary closure of the abdominal wall. Initial stoma formation and delayed anastomosis was performed in 12 (48%) patients, none of whom required prosthetic material for abdominal wall closure. Initial stomas were avoided in 5 patients who required SILASTIC (Dow Corning, Midland, MI) silos. Skin closure alone was used in 2 babies. The level of the atresia was most commonly jejunoileal (20 of 25, 80%). Mean hospital stay was increased in babies with intestinal atresia, 36.2 versus 63.1 days (P <.001).CONCLUSIONS:Although patients with intestinal atresia did have feeding delays, an increased incidence of adhesive intestinal obstruction, and prolonged hospitalization, neither chi(2) nor logistic regression analysis showed any correlation with mortality. Intestinal repair at the first operation is sometimes possible and depends on the severity of the peel. Delayed repair of the atresia after a period of bowel decompression and parenteral nutrition is preferred, but in certain situations (colonic atresia, necrotic intestine, complicated atresia) may not be possible. The combination of stomas and prosthetic material can be avoided in almost all patients. A management algorithm for patients with atresia and gastroschisis is discussed.

Objectives. To assess the reliability, cosmesis, and complication rate of the glans approximation procedure (GAP). Methods. We reviewed 37 consecutive GAP urethroplasties performed at the Children’s Mercy Hospital in Kansas City, Missouri over a 5-year period, performed by three different pediatric surgeons. All patients selected had a large, deep ventral groove, typically with a wide open urethral meatus. Results. The mean age was 18 months, with a mean follow-up of 28 months. Of the 37, there was one urethrocutaneous fistula that has since been easily repaired with good results. The parents have been very pleased with the results in all cases, with a straight and strong urinary stream. There was one episode of transient erythema, possibly representing an infection, which resolved after 3 days of oral antibiotics. Conclusions. The GAP is technically easy, reliable, and offers a relatively low complication rate for the repair of a very select group of patients with hypospadias with a deep ventral glanular groove and wide-mouthed meatus.

Horseshoe kidney is a common anomaly with frequent associated anomalies. The confluence of the lower poles of the kidneys lies anterior to the inferior vena cava. The authors were unable to find any reports in the world's literature of a vena caval obstruction caused by the presence of a horseshoe kidney. The authors report such a case presenting as bilateral lower leg swelling.

Necrotizing enterocolitis (NEC) primarily affects premature newborns. Regional and national decreases in the mean birthweight and gestational age of neonatal intensive care unit (NICU) admissions prompted a review of NEC in VLBW (very low birth weight, defined as <1,000 g) infants in our institution over a 25-year period. There were 266 patients treated for NEC during the study interval. We compared 71 VLBW with 195 non-VLBW infants and found that VLBW infants were: fed later (6.4 days v 4.1 days, P=.009), developed NEC later (20.8 days v 13.1 days, P=.002), had significantly lower 1- and 5-minute Apgar scores, were more likely to require surgery (51% v 34%, P=.016), more often had panintestinal (defined as >75% of intestinal length) involvement (10% v 4%, P=.043), and had poorer survival (56% v 72%, P=.013). Overall survival after NEC has improved over the study interval, both in our series and in other reports. However, the increasing number of VLBW infants who have NEC represent a subgroup who appear to be generally more ill, develop NEC later, require surgery with greater frequency than their non-VLBW counterparts, and are less likely to survive.

Children with neurological impairment (NI) frequently require feeding gastrostomy, and this often aggravates or produces gastroesophageal reflux (GER). From 1976 to 1994, 141 children with severe NI underwent Thal fundoplication and gastrostomy (GT). GER was evident in 80%; in the rest, fundoplication was an adjunct to GT. Ph results were positive in 38 cases, and 57 children had reflux according to the barium studies. There were no major intraoperative complications. Disruption of the repair and/or recurrent GER was noted in 14 cases (10%); 8 were redone as Thals, and 6 were converted to Nissen procedures. Pyloroplasty was done later in 9 children (6%). Bowel obstruction was seen in 4 patients (3%). Clinical follow-up (mean, 54 months) showed improvement in 96%; only 5 of the 141 (3.2%) have residual symptoms. Of the patients with an intact Thal, 67% could burp or vomit. The ability to vomit may protect the Thal fundoplication and avoid disruption of the repair.

Using a posterior repair and rectal suspension procedure for those patients who need surgical treatment of rectal prolapse, we have treated 46 patients over a period of 17 years at Children's Mercy Hospital in Kansas City, MO. One patient with caudal dysgenesis died of multiple congenital anomalies following two unsuccessful attempts at posterior repair and suspension. Four patients developed a recurrence afterwards, which was found to be due to sigmoid intussusception and, presumably, had played a major part in their original prolapse. Two of these required resection, one from the transanal approach and one from the transabdominal approach. One resolved spontaneously and another is as yet unresolved. Three patients had minor mucosal prolapse that was transient and two patients had extrusion of silk sutures but continued to have a very satisfactory result. Overall, 42 patients had satisfactory resolution of their rectal prolapse. Three of the four patients who had unsatisfactory results had associated anomalies that contributed to their poor outcome.

There are conflicting views on the pathogenesis of the intestinal malfunction seen in infants with gastroschisis. It has been variously ascribed to abnormalities of ganglion cells and smooth muscle elements, intestinal ischemia, and the "peel" which invests the serosa of the intestine. Review of the clinical and experimental literature showed only limited information on the histology of the eviscerated human intestine. In order to add to this data base, and to further investigate the pathogenesis of the intestinal malfunction from a histologic standpoint, we reviewed surgical and autopsy material from our experience with 105 neonates with gastroschisis. Ten specimens were satisfactory for evaluation from a standpoint of tissue integrity. The specific mural components of mucosa, submucosa, muscularis, and ganglion cells were examined and found to be either normal, or to show nonspecific abnormalities that varied from case to case, and were related mostly to intestinal infarction due to compromise of the gut at the site of the gastroschisis defect. In six patients, this progressed to atresia formation. The most consistent abnormalities were found in the serosal layer with its peel. Using special stains, the peel was found to be composed largely of fibrin and collagen. Based on this study, we feel that edema and ischemic changes, though often present, are much less prominent than the peel, as the leading histologic abnormality of the intestine of gastroschisis. Squamous epithelial cells were seen in the peel in four cases, suggesting that the peel had been "appliqued" onto the serosa of the herniated fetal gut.