Diagnostic accuracy of abdominal ultrasound to detect pathology that needs surgical exploration in children with umbilical discharge.

BACKGROUND: Umbilical discharge is common in children and mostly attributed to infection or granuloma. However, an underlying congenital abnormality warranting surgery might also be present. Ultrasound is the imaging modality of choice to diagnose the presence of a congenital abnormality. The aim of this study is to investigate diagnostic accuracy of the ultrasound to detect pathology requiring surgical excision. METHODS: All patients ≤18 years with umbilical discharge from January 2008 to September 2019 were retrospectively included. Diagnostic accuracy, i.e., sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+) and negative likelihood ratio (LR-), were calculated. RESULTS: Eighty-one patients were included and 56 were operated. The ultrasound was false positive in 10 patients and false negative in 13 patients. The sensitivity of ultrasound was 71.1% (95% CI 55.7-83.6), specificity 72.2% (54.8-85.8), PPV 76.2% (64.7-84.8), NPV 66.7% (54.8-76.8), LR+ 2.6 (1.5-4.5) and LR- 0.40 (0.2-0.7). CONCLUSIONS: This study shows that the diagnostic accuracy of ultrasound for detecting underlying congenital abnormalities warranting surgery for umbilical discharge in the pediatric population is low, even with experienced pediatric radiologists. Therefore, the role of the ultrasound in the diagnostic workup and value in clinical decision making is limited. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: III.

Cowper's syringocele in the pediatric population: a retrospective study of 122 patients.

INTRODUCTION AND OBJECTIVE: Syringocele is a rare cystic dilatation of the duct of Cowper's gland, afflicting mostly the pediatric population. Syringoceles have a wide range of symptoms and may cause urethral obstruction. The authors analyzed to clarify the clinical manifestation, diagnostic approach, management, and incidence in the pediatric population. MATERIALS AND METHODS: All patients (122 cases) diagnosed with a syringocele at the department of Pediatric Urology in a tertiary referral university children's hospital between August 1991 and October 2016 were analyzed retrospectively by assessing medical charts. RESULTS: The clinical manifestation, diagnostic findings, and follow-up are summarized in the table. Half of the patients (50.0%) also had typical posterior urethral valves (PUVs) and/or a single valve in the 12 o'clock position (flap-valve). The symptoms of open and closed syringoceles showed no significant difference. Treatment consisted of incision of the syringocele with a diathermia hook. The incidence of urinary tract infection (UTI) before and after surgery in the group that had a syringocele only was significant different. The overall incidence of syringoceles seen at urethrocystoscopy in this series was 3.0%. DISCUSSION: This series suggests that the presenting age is strongly related to the consequences of syringoceles, as the youngest half of the patients had significantly more UTIs at presentation than older patients, who presented with significantly more obstructive voiding symptoms, postvoiding residuals, and incontinence. In addition, the younger group had a significantly higher incidence of vesicoureteral reflux and dilatation of the upper urinary tract. The found association between syringoceles and PUV may be due to overgrowth of epithelium, as possible origin in both anomalies. CONCLUSION: With an incidence of 3.0%, syringoceles, in this tertiary referral series, should be considered in the differential diagnosis of obstructive urethral lesions. The presentation ranges between signs of severe obstructions in the prenatal and postnatal period to mild urinary incontinence problems at later age. Urethrocystoscopy proved to be useful in confirming the diagnosis and allows for immediate transurethral incision.

Training potential in minimally invasive surgery in a tertiary care, paediatric urology centre.

BACKGROUND: Minimally invasive surgery (MIS) is being utilized more frequently as a surgical technique in general surgery and in paediatric urology. It is associated with a steep learning curve. Currently, the centre does not offer a MIS training programme. It is hypothesized that the number of MIS procedures performed in the low-volume specialty of paediatric urology will offer insufficient training potential for surgeons. OBJECTIVE: To assess the MIS training potential of a highly specialized, tertiary care, paediatric urology training centre that has been accredited by the Joint Committee of Paediatric Urology (JCPU). STUDY DESIGN: The clinical activity of the department was retrospectively reviewed by extracting the annual number of admissions, outpatient consultations and operative procedures. The operations were divided into open procedures and MIS. Major ablative procedures (nephrectomy) and reconstructive procedures (pyeloplasty) were analysed with reference to the patients' ages. The centre policy is not to perform major MIS in children who are under 2 years old or who weigh less than 12 kg. RESULTS: Every year, this institution provides approximately 4300 out-patient consultations, 600 admissions, and 1300 procedures under general anaesthesia for children with urological problems. In 2012, 35 patients underwent major intricate MIS: 16 pyeloplasties, eight nephrectomies and 11 operations for incontinence (seven Burch, and four bladder neck procedures). In children ≥2 years of age, 16/21 of the pyeloplasties and 8/12 of the nephrectomies were performed laparoscopically. The remaining MIS procedures included 25 orchidopexies and one intravesical ureteral reimplantation. DISCUSSION: There is no consensus on how to assess laparoscopic training. It would be valuable to reach a consensus on a standardized laparoscopic training programme in paediatric urology. Often training potential is based on operation numbers only. In paediatric urology no minimum requirement has been specified. The number of procedures quoted for proficiency in MIS remains controversial. The MIS numbers for this centre correspond to, or exceed, numbers mentioned in other literature. To provide high-quality MIS training, exposure to laparoscopic procedures should be expanded. This may be achieved by centralizing patients into a common centre, collaborating with other specialities, modular training and training outside the operating theatre. CONCLUSION: Even in a high-volume, paediatric urology educational centre, the number of major MIS procedures performed remains relatively low, leading to limited training potential.

Therapeutic approach to Candida bezoar in children.

INTRODUCTION: Candida bezoar (CB) is a rare finding in neonates and infants with candiduria, presenting as necrotic debris with proliferating mycelia in the collecting system of the kidney. If initial antifungal medical treatment does not result in clearance of candiduria and disappearance of CB on ultrasound in dilated kidneys, invasive interventions like insertion of nephrostomy tubes (NT) or surgical interventions to drain the kidney are sometimes advocated(.). However, NT placement can be a technical challenge, especially in pre- and dysmature neonates, and NT displacement or obstruction by the CB can lead to suboptimal treatment. Identification of those children who will benefit from invasive renal drainage is important. OBJECTIVE: This study evaluates the management of patients with CB in three tertiary referral hospitals to determine criteria for intervention. MATERIALS AND METHODS: A retrospective multicenter chart analysis was conducted of children with candiduria and ultrasonographic demonstration of CB (diagnosed between March 1995 and August 2012). The indication for invasive renal drainage (if performed) and subsequent clinical outcome, serum creatinine levels and ultrasound findings were assessed. RESULTS: A total of 12 children were included, two of which were premature neonates. Eight children had congenital urogenital anomalies. One older child with acute myeloid leukemia had CB during chemotherapy and one ex-premature developed CB following cerebral candidiasis. All children received systemic antifungal medication; in seven children invasive treatment was added. Indications for invasive treatment were clinical deterioration, progressive renal dilation, pyonephrosis, rising creatinine levels and persistence of CB. Two underwent a Y-cutaneous ureterostomy and nephrostomy tubes were inserted in five children. Percutaneous renal drainage by nephrostomy led to complications in 3 of 6 procedures. In all patients, irrespective of therapeutic modality, follow-up ultrasound demonstrated no de novo changes. No additional parenchymal defects or deterioration of split renal function were seen on DMSA or MAG-3 scan. DISCUSSION: In the literature renal drainage is suggested in case of complete obstruction. However dilatation is a frequent finding in children as part of the congenital renal anomaly and does not necessarily mean that there is obstruction of the urinary tract. Even in children without candida infections the diagnosis of obstruction is not straightforward, while the results of a MAG 3 scan can be obscured by compromised kidney function, parenchymal bacterial infiltration and neonatal immaturity of the kidney. If candiduria and CB persist despite intensive medical treatment, intensive consultation is required before renal drainage, because NT insertion might be a surgical challenge. Complications such as displacement of the NT, urinoma development, or NT obstruction can occur and was seen in three of six procedures. Premature neonates seem to be more prone to complications due to their small anatomical proportions, requiring medical equipment with small diameters prone to displacement and obstruction. Some studies describe successful pharmaceutical management in the majority of patients with CB. Other studies describe unilateral surgical intervention in children with bilateral CB where unilateral drainage did not influence overall renal outcome. This is in line with our results. A limitation of the present study is its retrospective design. In this population, the motivation for invasive renal drainage or conservative management was not well documented in all cases, and was mainly based on clinical and diagnostic parameters like creatinine levels and radiographic findings. CONCLUSION: Renal drainage should be considered in selected cases after failure of systemic antifungal treatment. Inserting and maintaining a nephrostomy tube in young children is associated with a high rate of complications; conservative treatment is likely to be sufficient in the majority of patients with candiduria and CB.

Epispadias in boys with an intact prepuce.

OBJECTIVE: To present an overview of the clinical presentation and pathological anatomy, and the results of surgical correction of 7 cases of epispadias with intact prepuce; a rare condition that has only occasionally been reported in literature. PATIENTS AND METHODS: A retrospective search was performed in the surgical and diagnoses database between 1991 and 2011. Seven cases of epispadias with intact prepuce were identified. Five presented as a webbed and buried penis, 1 as phimosis and 1 with suspicion for congenital anomaly of the genitalia. RESULTS: In 3 of 7 cases, epispadias was suspected or diagnosed at first presentation and could be surgically corrected in the first intervention. In the other 4 cases, epispadias was discovered during surgery, requiring an additional intervention to perform epispadias repair in 3 cases. One boy was diagnosed with glandular, 3 with coronal, 1 with shaft and 2 with penopubic epispadias. Epispadias repair was successful with regard to cosmesis and erectile function. Five patients developed normal continence after surgery, 1 after intensive urotherapy. An under average penile length was the main reported problem during follow-up. CONCLUSION: In the diagnostic process for a concealed penis, the possibility of epispadias should be considered. If epispadias is suspected or confirmed, epispadias repair can occur in the first intervention, reducing the number of additional interventions. Epispadias with intact prepuce appears to have a better prognosis concerning urinary continence compared to classical epispadias.

Wrap plication of megaureter around normal-sized ureter for complete duplex system reimplantations.

PURPOSE: A duplex collecting system is a common congenital renal tract abnormality associated with different clinical problems. We describe our experience with ureteral reimplantations of a complete duplex collecting system where 1 megaureter needing recalibration and 1 normal-sized ureter coexisted. Recalibration of the megaureter was done by wrap plication around the normal-sized ureter. MATERIALS AND METHODS: Operative logs and case notes were reviewed of consecutive children with a complete duplex collecting system treated with wrap plication of the megaureter around the normal-sized ureter and reimplantation between 1997 and 2010. Reoperation, vesicoureteral reflux and obstruction rates were assessed. RESULTS: A total of 25 children underwent wrap plication and ureteral reimplantation. Of the cases 19 (76%) were completely successful and 6 (24%) needed reoperation. Three children (12%) had persistent high grade vesicoureteral reflux, 2 (8%) underwent endoscopic correction and 1 (4%) underwent repeat reimplantation of the duplex system. Three children (12%) had postoperative obstruction and 2 (8%) underwent endoscopic incision of the ureteral orifice. In 1 child (4%) a nonfunctioning lower moiety of the kidney developed, which was managed by heminephrectomy. CONCLUSIONS: Wrap plication of a megaureter around the normal-sized ureter before reimplantation seems to be a relatively safe method in the surgical management of children with a complete duplex collecting system of the kidney. Sufficient spatulation of the lower pole ureter seems to be crucial.

Characteristics and outcome and the omphalocele circumference/abdominal circumference ratio in prenatally diagnosed fetal omphalocele.

OBJECTIVE: To evaluate the outcome of fetuses with prenatally diagnosed omphalocele and to investigate the predictive value of the omphalocele circumference/abdominal circumference (OC/AC) ratio - a measure for the relative size of the omphalocele. MATERIALS AND METHODS: This study includes all fetuses prenatally diagnosed with omphalocele at our centre between 1995 and 2007. Medical records and footage of ultrasound examinations were reviewed. Omphalocele was classified in four groups: isolated, chromosomal, syndromic, and multiple anomalies. RESULTS: Eighty-eight cases were identified: 21 (24%) were isolated and 67 had additional structural anomalies. Of the 44 fetuses (50%) with chromosomal anomalies, 2 had omphalocele as a solitary finding. Fifty-three pregnancies (60%) were terminated because of the size of the lesion or associated structural or chromosomal anomalies. Twenty-one cases resulted in a live birth, of which 17 were vaginal deliveries (81%, all uncomplicated) including 3 cases of giant omphalocele (≥5 cm). The OC/AC ratio was found predictive for herniation of the liver, respiratory insufficiency and type of surgical reconstruction. Currently, 12/88 fetuses (14%) are alive and well, including 2 infants with multiple anomalies. CONCLUSION: Identification of omphalocele should arouse suspicion of genetic abnormalities, even in cases that appear isolated. The OC/AC ratio may influence counselling regarding the postnatal course.

Pre-operative diagnostic biopsy and surgery in paediatric liver tumours--the Amsterdam experience.

AIM: To report 24 years of pre-treatment biopsy and surgical experience in primary liver tumours in children. METHODS: Between 1979 and 2003, 53 children presented with a primary liver tumour of whom 48 who underwent surgical resection were evaluated (two died, two were unresectable, and one was transplanted). Biopsy data, per- and post-operative complications, mortality, and survival were retrospectively reviewed. RESULTS: Benign tumours were diagnosed in eight patients. Surgical resection for a malignant tumour was performed in 40 patients (26 hepatoblastomas (HB), eight hepatocellular carcinomas (HCC) (four had fibrolamellar HCC), three rhabdomyosarcomas (RMS), one neuroblastoma, one non-hodgkin lymphoma (NHL), and one teratoma). Primary resection was performed in one HB, and four HCCs. The cumulative survival without evidence of disease was 73% for HB (median 7 years) and 88% for HCC (median 3.5 years). CONCLUSION: The treatment results are comparable with those of larger international series except for HCC. The existing diagnostic pitfalls in differentiating between the various liver malignancies justify the use of a diagnostic biopsy.

Use of passive function testing in venous insufficiency: a prospective study.

In a prospective study, the results of venous function tests in 19 lower extremities with deep venous insufficiency and in seven control extremities were compared. First, the routine method of asking the patients to exercise their calves was used. Second, cuffs around the calves were rapidly inflated, producing standardized external compression to the calf muscle pump. Our aim was to decrease the influence of artifacts from involuntary muscle efforts and especially to render reproducible results of pressure reduction after passively pumping. The passive function test proved to be the only technique with which a decrease in pressure measured with strain gauges while the patient was in the standing position could significantly be discriminated between diseased and control extremities. Furthermore, the correlation of pressure decrease invasively versus measured noninvasively improved to 37% and thus became significant. Simulating calf venous pump function with the aid of inflatable cuffs is easily applicable in the vascular laboratory. It enables the vascular surgeon to reliably quantify venous recovery time and pressure reduction.

Construction of peritoneal venous valves: an experimental study in rats and piglets.

In the search for new techniques to improve venous valve insufficiency, a peritoneal patch with a mesothelial layer on either side was employed in the venous circulation to construct a venous valve de novo. This material was used because of the resemblance in fibrinolytic activity of mesothelial cells to endothelium. The behavior of double-sided flaps of peritoneum was first studied on only one side in the caval vein wall of 10 rats. All veins remained open, and smooth incorporation of the patch into the vein wall was observed. It was therefore decided to use the same material for construction of a cusp blade in the venous circulation. This venous reconstruction was microsurgically performed in 21 rats and 4 piglets. In our hands, intramuscular anesthesia proved superior to intraperitoneal analgesia in rats. For the piglets mechanical ventilation and general anesthesia were used. In 9 of 21 rats a fair or good result was observed, although it did not seem possible to create a fully competent valve with only one cusp blade in the 1.5-mm-diam caval veins. The same fact evolved from the study using four 7-mm-diam piglet veins. All peritoneal flaps in the venous circulation demonstrated rebuilding of their structure, perhaps induced by the relatively exaggerated central venous flow. To our knowledge, this technique has never been used before. It seems to offer many advantages in the venous circulation, where hardly any thrombogenic surface is tolerated in contrast to the arterial circulation. Application in humans is yet premature, but future research may resolve several problems that occurred in this study.

Failure of quantitative venous photoplethysmography to assess venous insufficiency: a prospective clinical study.

The increase of deep venous reconstructive surgery necessitates reliable tests for evaluation of the results, which requires quantization of the expelled volume. This could only be done with invasive measurements, but now it seems possible to calibrate photoelectric cells, and quantize the results. Nineteen limbs of patients with deep venous disease and seven limbs of healthy volunteers were evaluated, with methods known from the literature. In addition to measurements in standing position, these methods were used in the more patient-friendly sitting position. Invasively and noninvasively measured recovery times correlated well in the standing (R = 0.83) and in the sitting position (R = 0.86). However, the quantization of expelled volume did not significantly correlate. Also, the differences between expelled volume of controls and patients were only significant with invasive measurements in the standing position (P = 0.006) as well as in the sitting position (P = 0.004). The differences were not significant with the noninvasive method. In our opinion, photoelectric cells still do not offer an alternative for invasive measurements in the evaluation of results after venous reconstructive surgery.