Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

VURD Syndrome in a Female.

VURD syndrome has been repeatedly described as unilateral reflux into a nonfunctioning renal moiety. This syndrome is considered a pop-off mechanism dissipating pressure in lower urinary tract obstruction: it may be found in association with other protective mechanisms occurring in utero, such as ascites and/or urinomas, and has been exclusively described in male patients. A premature female baby with signs and symptoms of outflow obstruction underwent diagnostic workup revealing congenital urethral hypoplasia with unilateral reflux into a dysplastic kidney. Obstetrical history was positive for early onset, serologically negative ascites without cardiomegaly, which required serial aspirations. Reconstructive surgery was carried out with good results: ascites and VURD syndrome were both deemed to be perinatal protective mechanism against excess pressure in the urinary tract. Although rare, lower urinary tract obstruction in the female can lead to the same protective mechanisms seen in male fetuses/newborns. VURD syndrome and ascites should be interpreted as such and require perinatal specialist counselling.

Isolated fetal ascites secondary to persistent urogenital sinus.

Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum.

Acute oligohydramnios: antenatal expression of VURD syndrome?

OBJECTIVE: Oligohydramnios (OA) is nowadays regarded as one of the best markers of renal function (RF) impairment in bladder outlet obstruction (BOO) detected in utero. As such, its onset is usually early and progressive because of decline in fetal urine production. A series of acute OA complicating pregnancies with BOO has never been reported. METHODS: Over a 7-year period, 5 fetuses with in utero suspicion of BOO exhibited an abrupt decrease of amniotic fluid after the 30th week of gestation. RESULTS: All fetuses were delivered by cesarean section: diagnosis was posterior urethral valves in 3 cases, urethral atresia in 1, and prune-belly syndrome in 1. Urologic work-up demonstrated a unilateral vesicoureteral reflux dysplasia (VURD syndrome) in all 5 fetuses. RF at 1 year was normal in 4 fetuses and impaired in 1. CONCLUSIONS: Besides obstetrical reasons, OA may also have acute onset occurring in the presence of anomalies of the urinary tract; although diagnosis is almost always BOO, functional and anatomical characteristics of the urinary tract are those of VURD syndrome with a non-functioning, refluxing renal unit. The associated acute OA/VURD syndrome may represent a milder expression of a pop-off mechanism advocated in this syndrome with a more favorable prognosis than progressive OA detected early in pregnancy.

Elevated serum alpha-fetoprotein in Wilms tumor may follow the same pattern of other fetal neoplasms after treatment: evidence from three cases.

Alpha-fetoprotein (AFP) is a common tumoral marker in pediatric neoplasms; increased serum levels are usually encountered in tumors arising from tissues producing AFP during fetal life. However, elevation of such protein is rarely found in patients with Wilms tumor (WT). Three patients with WT and elevated serum AFP were studied over the course of the disease. One patient had left WT with invasion of aorto-caval lymph nodes and lung metastases. The second patient was referred to our center for abdominal recurrence of bilateral WT whereas the third showed right WT with inferior vena cava thrombosis. AFP levels demonstrated a trend parallel to decrease/increase of tumor size, with further elevation in patients with metastases. Elevated AFP serum levels in patients with WT could be related to peculiar histological features and serial dosage might be a useful diagnostic and prognostic test.

Parents' informational needs at the birth of a baby with a surgically correctable anomaly.

Previous studies have assessed informational needs of parents of sick fetuses, neonates and children to identify favourable patterns of physician-parent interaction. The aim of this paper was to assess parents' informational needs before and after the operation when the one affected by a surgically correctable anomaly is a newborn. In the period ranging from 1997 to 2000 all couples with newborns undergoing major surgical procedures at birth, at the Newborn Surgery Unit of the Hospital Bambino Gesù, were surveyed by means of a questionnaire. Thirty couples form the object of the study. The two genders did not show significant difference in any of the considered items. All 30/30 mothers (M; 100%) and 29/30 fathers (F; 97%) had never heard about the anomaly before the diagnosis was established in their baby. All parents (100%) stated that it would be better if the surgeon informed them with written educational material. Principal informational needs before operation regard: the description of the anomaly (M = 10; F = 11) and the prognosis in terms of survival chances of the baby (M = 17; F = 15); after surgery the cause of the anomaly (M = 5; F = 3); the steps of the recovery process (M = 10; F = 12) as well as the quality of their baby's life (M = 9; F = 9). In the immediate perioperative period the surgeon should well address parents' informational needs, which may significantly differ from his communication plan.

Impalpable testis and laparoscopy: when the gonad is not visualized.

The diagnostic accuracy of laparoscopy for impalpable testis is well recognized. However, in some cases, laparoscopic findings may be misleading, and a viable gonad may be missed with significant medico-legal implications. From January 1993 to December 2000, 202 patients with 219 impalpable testes were evaluated. In 95 cases, the gonad was immediately visualized, and in 5, the presence of a testis was documented by inserting the scope into the processus vaginalis. In the 119 remaining cases, no gonad was seen while entering the abdomen with the laparoscope. All patients with documented vas and vessels exiting the inguinal ring were surgically explored. Ten testes were found, 8 ectopic, with significant changes in shape and position, and 2 were canalicular. In the absence of hormone stimulation, no testes were found while exploring patients with cord structures coursing a closed inguinal ring and with contralateral hypertrophy. In 1 patient with absent vas and vessels, the testis was found at the lower renal pole while removing a dysplastic kidney. Despite technical refinements and an increase in clinical practice, a small percentage of viable testes may be missed with laparoscopic findings consistent with absent/vanished inguinal testis. Therefore, inguinal exploration is mandatory in all these cases.

Bipedicled skin flaps for reconstruction of the abdominal wall in newborn omphalocele.

Despite technical refinements in surgery and advances in postoperative intensive care, abdominal wall closure in giant omphalocele remains a difficult endeavor. In this respect, bipedicled skin flaps obtained with longitudinal incisions along the margins of the rectus abdominis muscle may represent a good alternative solution to achieve a complete, tension-free midline closure. Incisional areas can subsequently be easily covered with split-thickness thigh grafts. Two neonatal cases were treated with this technique with good results. This has enabled avoidance of palliative coverage as well as complex musculo-cutaneous reconstructions at a later age.

Cardiopulmonary performances in young children and adolescents born with large abdominal wall defects.

BACKGROUND/PURPOSE: As long as the survival rate of patients with abdominal wall defects (AWD) increases, information about long-term follow-up is becoming necessary. Even though quality of life in these patients, in absence of associated anomalies, appears to be unaffected, respiratory impairment soon after birth has been documented; therefore, participation in sports rarely is addressed. METHODS: Eighteen patients, ranging in age from 7 to 18 years, operated on at birth for large abdominal wall defects (> 4 cm for gastroschisis; >6 cm for omphalocele) were asked to come for a stress test on a treadmill, with measurements of time of exercise (TE), maximal oxygen consumption (VO2 max) and continuous recording of vital parameters. Respiratory function also was assessed by Forced Vital Capacity (FVC). RESULTS: Ergometric data were compared with those of a normal pediatric population. All patients were able to perform the stress test with no cardiovascular abnormalities detected at rest or on exertion. Maximum heart rate was reached after a significantly shorter TE, and VO2 max was significantly reduced when comparing normal subjects with AWD subjects and AWD subjects in sports with those sedentary. FVC was only slightly reduced in AWD patients without reaching statistical significance. CONCLUSIONS: These findings indicate that patients operated on for AWD at birth exhibit a normal cardiorespiratory function; decreased TE and VO2 max are likely to reflect a lack of physical activity with a lower degree of fitness. Therefore, no limitations to motor performances should exist for these patients. Well-being may be greatly improved by regular exercise.

Fetal monolateral urinoma and neonatal renal function outcome in posterior urethral valves obstruction: the pop-off mechanism.

The "fetal urinoma" is a clinical and diagnostic entity due to urinary extravasation, early diagnosed in fetal and/or neonatal period. Both urinoma and urinary ascites, whose pathogenesis is not clear, are recognized associations of uterero-pelvic junction obstruction (UPJO) and neonatal posterior urethral valves (PUV) related with a protected fetal and neonatal renal function. Clinical and experimental studies have demonstrated that fetal urinary tract obstruction results in severe renal parenchymal injury. The so called "pop-off" valve mechanism has been advocated to justify the upper tract function preservation. Protective "pop-off" mechanisms, such as a unilateral reflux and dysplasia, urinary extravasation and congenital bladder diverticula are present in about 30% of patients with PUV. Their presence correlates with better overall long-term renal function. This mechanism has been justified as a sort of self derivation, to explain the renal function preservation in fetal and neonatal period. In the last two years we observed three cases of fetal monolateral urinoma, prenatally detected in fetuses with diagnosis of PUV. All three cases did well for that concerning renal function despite some current opinions suggesting the necessity of a bilateral urinary extravasation in order to preserve upper urinary tract function.

Exteriorization-aspiration minilaparotomy for treatment of neonatal ovarian cysts.

INTRODUCTION: Recent literature indicates that laparoscopic techniques are considered the most appropriate approach for management of neonatal ovarian cysts (NOC). However, laparoscopic equipment may be unavailable in most centers, has high costs, and demands a long learning curve, especially in newborns. METHODS: A minilaparotomy with exteriorization-aspiration ("catch and suck") approach was used in 14 female newborns with antenatally diagnosed ovarian cysts. Average operating time was 25 minutes (range 15-45 min.). Postoperative course was uneventful in all cases; no intra- or postoperative complications were recorded. Length of stay was 48 hours in all patients. CONCLUSIONS: Minilaparotomy with cyst aspiration and subsequent removal may prove a reliable and safe technique in the treatment of NOC: operating time and length of stay are not significantly longer than laparoscopic approach, making this procedure a valid alternative.

Skin graft for 2-stage treatment of severe hypospadias: back to the future?

PURPOSE: Despite the introduction of more refined surgical techniques, the optimal treatment of the most severe forms of hypospadias remains to be determined. Single stage procedures, whether with the use of flaps or grafts, have long been regarded as the best approach, although the complication rate is nonnegligible with all procedures. MATERIALS AND METHODS: We report the use of a 2-stage repair with preputial graft interposition and subsequent tubularization of the urethral plate applied it to all severe cases of hypospadias with significant chordee or small glans. RESULTS: Both stages of the procedure were completed in 34 patients. Complications in 8 cases (23.5%) included 4 glans disruption in 4, coronal grove fistula in 2, urethral diverticulum in 1 and urethral stenosis due to balanitis xerotica obliterans in 1. Two pinhole fistulas also occurred which closed spontaneously. No complete disruptions or postoperative hematomas/bleeding was noted. Cosmetic and functional outcome after a minimum followup of 1 month was optimal in all cases with a normally located "slit" meatus and straight penile shaft. CONCLUSIONS: Although the controversy between use of grafts and flaps will probably continue forever, we believe that our 2-stage approach should be considered as a valid alternative for the most severe forms of hypospadias. Long-term results appear to outnumber the necessity of a learning curve for appropriate graft manipulation.

Prevalence of hypercontractility in male and female infants with vesico-ureteral reflux.

PURPOSE: To establish whether infants with vesico-ureteral reflux (VUR) have bladder dysfunction, with difference in gender, age at presentation and severity. PATIENTS AND METHODS: 37 infants (24 male and 13 female) aged 2 to 24 months with II degrees to V degrees degree VUR underwent cystometry. Of those, 10 underwent natural filling cystometry. We considered: instability and maximal voiding detrusor pressure (VDP) to be "high" when it exceeded 90 cm H2O. We defined hypercontractility as high VDP and/or instability. RESULTS: The prevalence of hypercontractility was 75% (18/24) in male and 46% (6/13) in female infants (p<0.004). High VDP was found in 50% (12/24) of male and 7% (1/13) of female patients (p < 0.001); no significant difference was found between male (25%) and female ones (38%) with instability alone. The mean VDP was significantly higher in male than in female infants (p < 0.001), in patients < 1 year of age than in older ones (p<0.001) and in severe than in moderate reflux (p<0.006). The mean voiding detrusor pressure of male infants was higher in severe (108+/-46cm H2O) and bilateral (101.3+/-44cm H2O) than in moderate (76+/-24 cm H2O) and unilateral (73.7+/-24 cm H2O) and in infants < 1 year of age (101.7+/-42 cm H2O) than in older ones (70.2+/-21 cm H2O). Natural filling cystometry confirmed the results of standard urodynamic studies. CONCLUSIONS: Bladder dysfunction is confirmed also in infants with reflux, particularly in male younger patients, and it differs in gender. The pathogenesis of congenital reflux is not always a feature of malformation of the vesico-ureteral junction; therefore, patients with bladder dysfunction must be identified early.

Are preoperative parameters of unilateral pyelo-ureteric junction obstruction in children predictive of postoperative function improvement?

OBJECTIVES: Surgery on the pyelo-ureteric junction obstruction (PUJO) has long been thought to affect postoperative renal function. However, preoperative assessment of which kidneys will benefit from such surgery remains unreliable. MATERIAL AND METHODS: Pre- and postoperative data relating to renal function were obtained by renal scan for 69 patients who were operated upon for PUJO. These patients were divided into two groups: group A (improved differential renal function) and group B (unimproved or decreased differential renal function). The two groups were then compared with regard to age at operation and presence or absence of clinical symptoms. Ultrasound (anteroposterior diameter of the pelvis, parenchymal thickness) and renal scan (glomerular filtration rate, differential renal function of the affected kidney, obstructive pattern) parameters were also taken into consideration. Some variables were also made dichotomous (pelvic diameter < or > 15 mm, parenchymal thickness < or > 5 mm, differential renal function < or > 40%). Statistical correlation was sought with parametric and non-parametric tests. RESULTS: No correlation whatsoever was found between the two groups for any of the parameters under consideration, so that any attempt at logistical regression analysis failed. CONCLUSIONS: None of the currently adopted diagnostic tests can be used to indicate which renal units will benefit from surgery through an improved renal function. The presence or absence of clinical symptoms does not appear to affect renal function either. There is evidence that parents should be provided with such information when giving their informed consent to pyeloplasty.

Is preoperative laparoscopy useful for impalpable testis?

PURPOSE: Laparoscopic assessment of pelvic anatomy has gained wide popularity over the years. Today surgical treatment of impalpable testis is nearly always preceded by diagnostic laparoscopy. The actual role of such a procedure remains undefined. We performed a prospective randomized clinical trial in patients with impalpable testis to evaluate the clinical usefulness of laparoscopy before surgical exploration. MATERIALS AND METHODS: We studied pediatric, age matched patients with impalpable testis who were randomized to group 1-30 who underwent open surgery only and group 2-31 who underwent laparoscopy and open surgery. Anatomical findings, operative procedures, operative time and cost, number of recurrences and testicular volume at followup were then compared in the 2 groups. RESULTS: There were no statistically significant differences in the 2 groups for any of the considered parameters except operative cost and time, which were significantly higher in the laparoscopy group. CONCLUSIONS: Preoperative laparoscopy does not provide any significant advantage over open surgery for treating impalpable testis.

Persistent cloaca: are we ready for a correct prenatal diagnosis?

Cloacal malformations are rare and can present in variable aspects. The importance of ultrasound in detecting these anomalies is well known. Sonographic features vary in accordance with the type of malformation and the gestational age. A positive diagnosis is not possible because of the lack of specific ultrasound findings, which can show similar aspects to other abnormalities. We present 3 cases of prenatal diagnosis of this malformation, emphasizing that in the presence of a plurilobed cystic pelvic fetal mass with associated malformations, such as cardiac, renal, and vertebral anomalies, a persistent cloaca can reasonably be suspected.

Gastroesophageal reflux in neurologically impaired children: partial or total fundoplication?

BACKGROUND: It is difficult to give guidelines when approaching gastroesophageal disease in neurologically impaired children. Indication for surgery has been increasing over recent years, but there is no consensus on the surgical technique of choice. Nothing has been written specifically comparing the results of different procedures in these patients, so far. STUDY DESIGN: We retrospectively compare the short- and long-term results of two different types of fundoplication in a series of children operated on for documented gastroesophageal reflux disease at our institution. RESULTS: One group (group A) of 27 patients, operated on between 1977 and 1993, underwent Nissen fundoplication, the other (group B), formed of 20 patients all of whom were operated on between 1993 and 1995, underwent Thai fundoplication. We compared the results in terms of positive outcome (recovery) and negative outcome (minor and major complication), computing the relative odds of group A versus group B in terms of risk of complication, and we compared the mean operative time and the length of hospital stay by means of a student's t-test analysis. CONCLUSIONS: Our results show that there is no statistical difference between the two procedures in terms of relative risk of complication and success rate. The duration of surgery and hospital stay were significantly shorter in group B. The Thal procedure can, therefore, be proposed as first choice in the management of these patients.

Stenting for caustic strictures: esophageal replacement replaced.

METHODS: From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesù Children's Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption. RESULTS: No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03). CONCLUSION: Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

Ureteral occlusion prosthesis.

Although temporary or definitive complete ureteral occlusion is rarely needed, there is a considerable number of reports introducing different devices to achieve this goal, most of which can be inserted with minimally invasive procedures. Easy placement is considered of paramount importance, as the candidates are very often in bad general condition as a result of previous surgery, radiotherapy, or other palliative treatments for cancer. A device that can be inserted and removed percutaneously is presented herein. It can be employed in cases of ureteral fistulas resulting from radiotherapy and ureterosigmoidostomy with good results. The technique is simple and not time consuming.

Maximal oxygen consumption and stress performance in children operated on for congenital diaphragmatic hernia.

The long-term follow-up of patients operated on for congenital diaphragmatic hernia (CDH) at birth has been extensively evaluated, both clinically and with respect to respiratory function. However, little is known about the sports practice and stress performance of these subjects. Fifteen of 107 patients operated on for CDH underwent exercise stress testing with a stepwise increase in workload. A questionnaire was provided, which requested information on sports practice and lifestyle. Maximal oxygen consumption [Vo2 max] was measured along with dynamic lung volumes. Clinical examination included a whole-body assessment (height, weight, skinfolds) and vital parameters (heart rate and blood pressure). Fifteen healthy children who practiced regular physical activity (2 to 4 hours/week) served as controls. All the CDH patients experienced a good lifestyle, but only 8 of them were participating in sports. Exercise duration and Vo2 max were significantly lower for the CDH patients, and were lowest for the sedentary patients. Therefore, the reduced Vo2 max of these otherwise healthy children most likely represents a lower degree of physical fitness rather than decreased respiratory function. Fitness is an expression of well-being; thus, there is evidence that these patients could safely participate in competitive motor activities.