Systematic Review and Meta-analysis of Laparoscopic Versus Open Radical Nephrectomy for Paediatric Renal Tumors With Focus on Wilms' Tumor.

OBJECTIVE: To summarize and evaluate the outcomes of laparoscopic radical nephrectomy (LRN) and compare its safety and efficacy with open radical nephrectomy (ORN) in pediatric renal tumors (RT) and Wilms' tumors (WT). BACKGROUND: ORN is the gold standard treatment for pediatric RT, consisting predominantly of WT. LRN is gaining popularity but remains controversial in pediatric surgical oncology. METHODS: A systematic search was performed for all eligible studies on LRN and comparative studies between LRN and ORN in pediatric RT and WT. Meta-analysis, subgroup analysis, and sensitivity analysis were conducted. The main endpoints were cancer-related outcomes and surgical morbidity. Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines were followed. RESULTS: No levels I to II studies were identified. LRN was feasible in nearly 1 in 5 pediatric RT and WT after neoadjuvant chemotherapy, with pooled mid-term oncological outcomes (<7% local recurrence, >90% event-free survival) comparable with those of ORN. There was no strong evidence of an increased risk of intraoperative tumor spillage, but lymph node harvest was inadequate in LRN. Large tumors crossing the ipsilateral spinal border were associated with a trend for intraoperative complications and positive margins. Pooled complications rate and hospital stay duration were similar between LRN and ORN. Long-term (>3 years) outcomes are unknown. CONCLUSIONS: Available level III evidence indicates that LRN is a safe alternative to ORN for carefully selected cases, with similar spillage rates and mid-term oncological outcomes. However, there was no advantage in surgical morbidity and lymph node harvest was inadequate with LRN. Tumor-matched-group studies with long-term follow-up are required. LEVEL OF EVIDENCE: Level III.

Diagnostic accuracy of two-dimensional shear wave elastography in detecting hepatic fibrosis in children with autoimmune hepatitis, biliary atresia and other chronic liver diseases.

BACKGROUND: Although fibrosis is the main determinant of liver stiffness, other disease-related factors usually disregarded in studies on liver elastography, such as inflammation and cholestasis, may influence liver stiffness. OBJECTIVE: To evaluate the accuracy of two-dimensional (2-D) shear wave elastography in assessing liver fibrosis in children with chronic liver disease by controlling for the confounding role of several disease- and patient-related factors. MATERIALS AND METHODS: Three disease groups were studied: 1) various chronic liver diseases, 2) autoimmune hepatitis and 3) biliary atresia. The METAVIR (meta-analysis of histological data in viral hepatitis) score was used for fibrosis staging and grading of necroinflammatory activity. Multiple linear regression was used to evaluate the relationship between liver stiffness measurements and disease-related factors. The diagnostic accuracy of elastography for predicting fibrosis stages was assessed by calculating the area under the receiver operating characteristic curves. RESULTS: The various chronic liver diseases group (n=32; 7.1±4.9 [mean±standard deviation] years) showed liver stiffness of 8.9±5.0 (mean±standard deviation) kPa, the autoimmune hepatitis group (n=33; 8.1±4.4 years) of 7.1±2.7 kPa, and the biliary atresia group (n=19; 0.2±0.1 years) of 19.7±15.2 kPa. Liver stiffness measurements differed across METAVIR fibrosis categories in all disease groups. The highest values were found in biliary atresia, at fibrosis stages ≥F2 (F2: 12.4±1.6 kPa, F3: 17.8±2 kPa, F4: 41.5±12.4 kPa). Liver stiffness was strongly associated only with fibrosis (P<0.0001) in various chronic liver diseases, but with necroinflammatory activity (P<0.0001) and fibrosis (P=0.002) in autoimmune hepatitis, and with age (P<0.0001), fibrosis (P<0.0001) and cholestasis (P=0.009) in biliary atresia. Optimal cutoffs for detecting advanced fibrosis (≥F3) were 16 kPa (area under curve: 0.98; sensitivity: 87.5%; specificity: 96.7%) in biliary atresia and 8.7 kPa (area under curve: 0.98; sensitivity: 93.8%; specificity: 96.1%) in other chronic liver diseases. CONCLUSION: Two-dimensional shear wave elastography is reliable in assessing liver fibrosis in children with chronic liver diseases.

Ileocecal Valve Removal Reverses the Constipating Effects of Loperamide in Rats.

BACKGROUND: Previous evidence associating ileocecal valve removal (ICVR) with a reduced risk of fecal impaction of the ileocecum in cystic fibrosis indicated possible benefits from ileocecal valve loss in disorders with inhibited proximal colon transit caused by fecal dehydration and hypoperistalsis. We aimed to investigate the ability of ICVR in reversing fecal impaction in a loperamide-induced model of a similar pattern of inhibited proximal colon transit in rats. MATERIALS AND METHODS: Thirty pubertal Sprague-Dawley rats were rendered constipated with subcutaneous loperamide treatment (1 mg/kg/d) for 7 d. On day four, rats were allocated to groups: ICVR (n = 12), total colectomy (TC, n = 9), and sham operation (SO, n = 9). Fecal pellet number and consistency were assessed daily. On day seven, all rats were gavaged with barium. Two hours later, intestinal transit ratio (distance of barium head from the pylorus adjusted for small intestine length) and adjusted (for total intestine length) barium-to-anus distance were assessed. RESULTS: ICVR showed higher transit ratio and shorter barium-to-anus distance, that is, faster transit, than SO (P < 0.0001); differences between ICVR and TC were not significant (P > 0.06). Furthermore, ICVR and TC showed similar reduction in hard feces, compared with SO (P < 0.0001). TC showed higher diarrhea rate than ICVR (P < 0.0001). CONCLUSIONS: ICVR led to an effective, similar to TC, reversal of the constipating effects of loperamide and, unlike TC, was not associated with diarrhea. Our findings support the idea that ICVR might be beneficial in disorders with inhibited proximal colon transit resulting from fecal dehydration and hypoperistalsis, such as refractory cystic fibrosis-related intestinal obstruction. Potential clinical implications merit further study.

'Testis-epididymis dissociation' in cryptorchidism and hydrocele: the tip of the iceberg of a persistent genital mesentery.

PURPOSE: To investigate whether testis-epididymis dissociation encountered in boys with cryptorchidism/hydrocele is related with an abnormal persistence of the fetal mesentery of testis and associated ducts. METHODS: We examined the morphology of peritoneal folds of the testis, epididymis, and vas deferens in 25 boys operated for unilateral cryptorchidism [inguinal (n = 20), intrabdominal (n = 5)] and 20 boys operated for unilateral communicating hydrocele. Findings were compared with the normally persisting genital mesentery of rats (n = 30, both sides), a known animal model of the genital mesentery of human fetuses, as well as with the normal mature pattern of genital peritoneal folds in adult male cadavers (n = 12, both sides). Rats before testis descent [aged 18 days (n = 15)] served for comparison with boys with cryptorchidism, while rats after testis descent [aged 50 (n = 15)] known to retain patent processi vaginales for comparison with boys with hydrocele. RESULTS: A well-developed genital mesentery, identical to the fetal-type genital mesentery in the rat, was documented in cryptorchidism and hydrocele. The peritoneum enveloped the testis, epididymis, and vas deferens, and formed wide ligaments between testis-epididymis, epididymis-vas deferens, and vas-posterior wall; processus vaginalis was patent in all cases. The testis-epididymis ligament was related with testis-epididymis distancing, the so-called testis-epididymis dissociation. On the contrary, genital mesentery had involuted in the adult male cadavers, except for a small portion of testis-epididymis ligament corresponding to the so-called sinus epididymis. CONCLUSION: The testis-epididymis dissociation encountered in cryptorchidism/hydrocele is part of an anomalously persisting fetal-type genital mesentery.

Laparoscopic Repair of Duodenal Atresia: Systematic Review and Meta-Analysis.

PURPOSE: To investigate the outcome of laparoscopic repair of duodenal atresia (LRDA) in relation to different approaches with regard to suture material and anastomosis technique. To identify evidence for the safety and efficacy of LRDA compared with the conventional open repair. METHODS: Systematic search was performed for all studies on LRDA, excluding case reports, and all comparative studies between LRDA and open repair. Chi-square was used to assess associations between complications or conversions rates and different LRDA approaches (suture material, suturing technique). Meta-analysis was employed to compare LRDA and open repair. RESULTS: The complications and conversions rates of LRDA were not affected by the different suture materials (Silk, Vicryl, PDS; p = 0.51) or suturing technique (interrupted, continuous; p = 0.46). The meta-analysis did not show significant differences between LRDA and open repair in overall complications rate (p = 0.88), time to feeds (p = 0.12) and hospitalization time (p = 0.28), although it revealed longer operative time with LRDA (p < 0.0001). CONCLUSIONS: LRDA shows comparable safety and efficacy with the open repair, although it is associated with significantly longer operative time. There is no evidence that the type of the suture material or anastomotic technique affects the outcome of LRDA.

A diagnostic model for histologic damage in undescended testes based on testis rigidity measurement: an experimental study with a novel device.

BACKGROUND: We aimed to test whether testis rigidity (hardness) measured using a newly-designed device we previously introduced would offer more reliable assessment of histologic damage in undescended testes than conventional methods (consistency feel at palpation, volume measurement). MATERIALS AND METHODS: Forty-five 18-d-old Lewis rats underwent surgical inhibition of descent of left testes and were followed to 40 (n = 16), 63 (n = 14), or 90 days (n = 15). Another 45 18-d-old Lewis rats were sham operated (left side) and followed likewise (n = 14, n = 15, and n = 16). At the designated time points, testes were exposed bilaterally, rigidity was measured, and consistency at palpation was scored; testes were removed and subjected to length, width, weight measurements, volume calculation, and histomorphometry (mean Johnsen score [MJS], mean tubular diameter [MTD], and mean capsule width [MCW]). Testes of experimental group were compared with ipsilateral testes of sham-operated rats. RESULTS: At all time points, undescended testes had decreased rigidity, MJS, and MTD, increased MCW, decreased volume and weight; contralateral testes remained unaffected. Rigidity was associated only with MJS and MTD, and most strongly with MJS (multiple stepwise linear regression, F = 694.44, P < 0.0005). MJS could be precisely predicted from rigidity: MJS = 0.699 × testis rigidity (F = 1358.82, P < 0.0005). This model showed good fit between predicted and actual MJS values (R(2) = 0.94), low error, nonsignificant bias, sensitivity 75% and specificity 90%. Model validation showed low prediction error and nonsignificant bias, indicating generalizability. Testis volume and palpation proved imprecise MJS predictors. CONCLUSIONS: Testis rigidity is an effective predictor of histologic damage in rat undescended testes, with diagnostic value superior to testis palpation scoring and volume measurement.

Surgical disorders in pediatric and adolescent gynecology: Adnexal abnormalities.

Surgical abnormalities of the adnexa in children and adolescents include a variety of ovarian and paraovarian lesions ranging from benign functional cysts to malignant tumors, torsion of the ovary and/or the fallopian tube, and adnexal infectious lesions ranging from salpingitis to tubo-ovarian abscesses. Presentations vary from asymptomatic pelvic masses to acute abdomen, and some ovarian tumors might present with precocious puberty or virilization. Acute pain might be caused by hemorrhage or rupture of ovarian or paraovarian cysts, adnexal torsion or adnexal infection. Differential diagnosis of adnexal masses should include peri-appendiceal abscess in all age groups, and endometriomas and ectopic pregnancy in adolescents. This review provides guidance on the differentiation between adnexal abnormalities, based on important clues from clinical assessment and diagnostic workup, and ultimately on the decision making about the need for surgery, its level of urgency, and the type of surgery to clinicians of all specialties involved in the care of young females.

Surgical disorders in pediatric and adolescent gynecology: Vaginal and uterine anomalies.

Obstructive vaginal and uterine anomalies including imperforate hymen, transverse vaginal septum, and vaginal and/or cervical atresia or aplasia, might rarely present in infancy or childhood with hydrocolpos and/or hydrometra but they usually go unrecognized until presentation with amenorrhea and hematocolpos and/or hematometra in puberty. They should always be included in the differential diagnosis of a suprapubic and/or introital mass; in the latter case, vaginal vascular malformations and vaginal tumors should also be considered. Uterovaginal aplasia typically manifests with amenorrhea in puberty and needs to be differentiated from complete androgen insensitivity syndrome and gonadal dysgenesis of genetic males. Uterine fusion anomalies usually present with fertility and/or obstetrical complications in adulthood. However, a unicornuate uterus with a blind rudimentary contralateral horn containing functioning endometrium, and didelphys or septate uterus with a deviating obstructive septum might present in childhood or puberty with sequelae related with secretions or menstrual retention. This review provides a collective account of the most clinically important information about vaginal and uterine anomalies in childhood and adolescence for clinicians involved in the care of young females with the aim to provide guidance in appropriate evaluation and management.

Outcomes of Pediatric Endoscopic Pilonidal Sinus Treatment: A Systematic Review.

Treatment of pilonidal sinus disease with conventional excision techniques is associated with recurrence up to 20 to 30% (primary closure) or with prolonged healing that might last months (closure by secondary intention). Endoscopic pilonidal sinus treatment (EPSiT) is gaining increasing popularity. This systematic review aims to summarize and evaluate the reported outcomes of pediatric EPSiT (PEPSiT) to date. Systematic search was performed for all studies on PEPSiT in patients younger than 18 years, pertaining to demographics, technique, and outcomes. Fisher's test was used to assess the associations between success/recurrence rates and different approaches (fistuloscope vs. cystoscope, different wound care protocols). A total of 320 patients (9 studies, 2018-2022) with a weighted mean age of 15.7 years and follow-up duration of 13.5 months were included. PEPSiT was successful in 290 patients (90.9%) with weighted mean time to healing of 4.1 weeks. Recurrence was reported in 29 patients (9.1%) with weighted mean time to recurrence of 4.6 months. Outcomes were not significantly altered by the use of fistuloscope versus pediatric cystoscope (p = 1.0), or with perioperative laser epilation (p = 0.06), or postoperative regular shaving, depilatory creams, light pulse, or laser (p = 0.31). The weighted mean operative time was 38 minutes and hospital stay was 16 hours. Summary of available evidence confirms that PEPSiT is safe and effective. Added to its noninvasiveness, PEPSiT's pooled outcomes appear superior to those of numerous conventional techniques. Comparison with conventional techniques, particularly off-midline flap and semi-closure procedures, is, however, lacking. The superiority of PEPSiT needs to be confirmed in future comparative studies, including cost-benefit analysis.

Intrafamilial Transmission of Pneumococcal Acute Spontaneous Peritonitis.

Streptococcus pneumonia (S. pneumoniae, Pneumococcus) is a major cause of childhood morbidity and mortality worldwide. The most common presentations of invasive pneumococcal disease (IPD) in children include bacteremic pneumonia, meningitis, and septicemia. However, pneumococcal acute spontaneous peritonitis is a highly uncommon-and potentially life-threatening-presentation of invasive pneumococcal disease and should be considered in cases of abdominal sepsis. To our knowledge, we report the first case of intrafamilial transmission of pneumococcal peritonitis in two previously healthy children.

Laparoscopic versus open orchiopexy for palpable undescended testes: Systematic review and meta-analysis.

BACKGROUND/PURPOSE: Laparoscopic orchiopexy (LO) was recently described as superior to open orchiopexy (OO) for palpable undescended testes (UDT). We aimed to investigate the outcomes of LO of palpable UDT in relation to high retroperitoneal dissection, Prentiss maneuver and intrascrotal testis fixation; also, to identify evidence for the safety, efficacy and cost of LO compared with OO in palpable UDT. METHODS: Systematic search was performed for all studies on LO for palpable UDT, and for all comparative studies between LO and OO in palpable UDT. Fisher's test was used to assess associations between success/complications rates and different LO approaches and meta-analysis to compare LO and OO. RESULTS: In LO, success rates were not affected by regular high dissection (p = 1.0), Prentiss maneuver (p = 1.0) or intrascrotal fixation (p = 1.0); in fact, higher complications rates were noticed with regular high dissection (p = 0.002) and Prentiss maneuver (p = 0.01). Meta-analysis showed no significant differences between LO and OO in success (p = 0.17) and complications (p = 0.14) rates, while LO cost was higher in all comparative studies. CONCLUSIONS: Evidence shows higher benefit-cost ratio for OO and, therefore, the latter should remain the procedure of choice. LO can be alternatively used, as it shows comparable safety/efficacy, but it should not include high dissection, Prentiss maneuver and testis fixation, when unnecessary. TYPE OF STUDY: Systematic review and meta-analysis LEVEL OF EVIDENCE: III.

Morphologic alterations of the genital mesentery implicated in testis non-descent in rats prenatally exposed to flutamide.

BACKGROUND: There is an endless debate on whether androgens mediate testis descent through developmental changes in the gubernacular or the cranial suspensory ligament. OBJECTIVE: To investigate the relation of any possible morphologic changes in the genital mesentery, that is, the system of genital peritoneal folds including the gubernacular and cranial suspensory ligaments, with the event of testis non-descent in rats prenatally exposed to the antiandrogen flutamide. MATERIALS AND METHODS: Time-pregnant Sprague Dawley rats received flutamide (100 mg/kg/d) or vehicle subcutaneously on gestational days 16-17. Flutamide-treated male offspring (n = 67), and vehicle-treated male (n = 34) and female (n = 28) offspring were surgically explored under microscope on postnatal day 50. Testicular position was examined bilaterally. Dimensions of genital mesentery parts were also assessed bilaterally. Association of flutamide-induced morphologic changes with descended (n = 61) and undescended (n = 50; 33 cryptorchid and 17 ectopic) testes was investigated with logistic regression analysis. RESULTS: The male genital mesentery comprised a cranial and a caudal fold converging on the vas deferens. Flutamide resulted in enlarged cranial and reduced caudal folds. Of all flutamide-induced alterations, the increased length of the posterior fixation of the cranial fold and the decreased length of the gubernacular ligament of the caudal fold were found to independently increase the odds of testis non-descent. Testicular ectopy, unlike cryptorchidism, was associated with a short gubernacular ligament only. The female genital mesentery consisted of a cranial fold only. CONCLUSION: Our findings showed a combined contribution of both cranial and caudal folds of the genital mesentery to testis non-descent, through an abnormally long mesentery root and an abnormally short gubernacular ligament, respectively. Inhibition of male-specific development of the genital mesentery with flutamide did not result in a feminized architecture.

Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises.

BACKGROUND/PURPOSE: In the few studies on intestinal complications and growth of cystic fibrosis (CF) patients with a history of meconium ileus (MI), operated MI has not been investigated separately. We aimed to investigate the incidence of long-term intestinal obstruction sequelae [constipation, distal intestinal obstruction syndrome (DIOS)] and growth in CF patients operated for MI. METHODS: Retrospective study (1989-2016) including operative diagnoses and procedures, constipation and DIOS events, yearly Body Mass Index (BMI) measurements. Outcomes were examined in subgroups operated for MI only and for MI with atresia and/or volvulus. RESULTS: Of 49 patients followed-up for 15 (mean) years, 5 (10.2%) developed constipation and 14 (28.6%) DIOS. BMI was within normal percentiles in 53 patients over a 10-year follow-up. MI only and MI with atresia and/or volvulus did not differ in constipation and/or DIOS incidence (11/34 vs. 7/15, p=0.39) or in BMI (p=0.47). Cases with ileocecal valve resection (ICV-R) showed lower constipation and/or DIOS incidence than those without ICV-R (0/6 vs. 11/28, p=0.02) and no different BMI (p>0.05). CONCLUSIONS: CF patients operated for MI were in long-term risk for constipation/DIOS; their growth was normal. Interestingly, underlying atresia/volvulus neither increased constipation/DIOS risk nor affected growth. Strikingly, ICV-R showed no constipation/DIOS risk and no impact on growth. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: III.

Right or left thoracotomy for esophageal atresia and right aortic arch? Systematic review and surgicoanatomic justification.

INTRODUCTION: The optimal thoracotomy approach for the management of esophageal atresia and tracheoesophageal fistula (EA/TEF) with a right aortic arch (RAA) remains controversial. METHODS: Systematic review of complications and death rates between right- and left-sided repairs, including all studies on EA/TEF and RAA, apart from studies focusing on long-gap EA and thoracoscopic repairs. Review of right- and left-sided surgical anatomy in relation to reported complications. RESULTS: Although no significant differences were elicited between right- and left-sided repairs in complications (9/29 vs. 1/6, p = 0.64) and death rates (2/29 vs. 0/6, p = 0.57), unique anatomic complications - such as injury to the RAA covering the esophagus and intractable bleeding - associated with mortality were revealed in the right thoracotomy group. Left-sided repairs following failed repair through the right showed higher complications rate (3/3) than straightforward right- (9/29) or left-sided repairs (1/6) (p = 0.024). Right thoracotomies converted to left thoracotomies led to staged repairs more frequently (4/9) than straightforward right (5/38) or left thoracotomies (0/6) (p = 0.03). CONCLUSIONS: There is not enough evidence to support that right thoracotomy, characterized by unique surgicoanatomic difficulties, is equivalent to left thoracotomy for EA/TEF with RAA. Both approaches might be required, and, therefore, surgeons should be familiarized with surgical anatomy of mediastinum approached from right and left. Systematic review, Level of Evidence III.

Enterobius Vermicularis-Associated Pelvic Inflammatory Disease in a Child.

BACKGROUND: Enterobius vermicularis is the most highly prevalent intestinal worm in childhood and is generally considered of low pathogenicity. Little is known about the inflammatory complications of the female genital tract induced by E. vermicularis in childhood. CASE: A case of E. vermicularis-associated pelvic inflammatory disease with right salpingitis mimicking acute abdomen due to appendicitis in an 11-year-old girl is presented. CONCLUSION: E. vermicularis-related pelvic inflammatory disease should be included in the differential diagnosis of abdominal pain in children. Increased awareness is necessary to avoid an unnecessary surgery and to choose the correct antibiotic treatment.

Appendiceal phlegmon mimicking intussusception on ultrasound: correspondence between sonographic and operative findings.

Little is known about the possibility that ruptured appendicitis may produce a false sonographic appearance of intussusception. We present here a case of a periappendiceal phlegmon mimicking ileocolic intussusception on ultrasound in a 3.5-year-old girl and provide a surgico-anatomic explanation on the basis of the intraoperative findings for the false sonographic image. CT imaging was used to make the diagnosis. Intraoperatively, it was revealed that the cecum and sigmoid, which were adherent to each other with pseudomembranes, formed an intestinal mass around the appendix. Accordingly, the appendicolith at the center of the phlegmon was responsible for the central echogenicity, and the surrounding cecum and sigmoid for the external hypoechoic and hyperechoic rings of the target-sign appearing mass on the preoperative ultrasound. Such an understanding of the etiology of the false sonographic image may help to increase awareness and avoid misdiagnosis.