Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report.

Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure's potential long-term risks, which will necessitate a limited but regular follow-up.

Pediatric primary spontaneous pneumothorax: a comparison of treatment at pediatric surgery vs. thoracic surgery departments.

Management of pediatric Primary Spontaneous Pneumothorax (PSP) is controversial and based on guidelines on adults. Therapeutic strategies include: observation, needle aspiration, chest drain, or surgery. We aimed to assess: i) differences in the management of PSP in pediatric vs. adult departments; ii) risk of recurrence associated to each therapeutic choice; iii) management of "large" pneumothorax (i.e. >3cm at the apex on chest X-Ray); iv) role of CT scan in addressing the treatment. We reviewed all PSP treated at Pediatric Surgery Unit (PSU) and Thoracic Surgery Unit for adults (TSU) in a 10-year period (2011 to 2020). We included a total of 42 PSP: 30/42 1st episodes and 12/42 recurrences. Among the 30/42 1st episodes, 15/30 were managed in the PSU and 15/30 in the TSU. Observation was significantly most common among PSU patients (9/15, 60%) vs. TSU cases (1/15, 6.7%; p=0.005]. Chest drain placement was reduced in PSU (3/15, 20%) vs. TSU (12/15, 80%; p=0.002). Observational was associated with a reduced risk of recurrence (0/10, 0%) compared to chest drain (7/15, 46.7%; p=0.01). Management of 20/42 "large" pneumothorax was: 4/20 (20%) observation, 10/20 (50%) chest drain, 2/20 (10%) needle aspiration, 4/20 (20%) surgery. Twentythree/ 29 PSP (79.3%) underwent CT-scan after the first episode. Bullae were detected in 17/23 patients and 5/17 (29.4%) had seven episodes of recurrence. PSP patients treated by PSU were more likely to receive clinical observation. Those managed by TSU were mostly treated by chest drain. Observation seems an effective choice for clinically stable PSP, with low risk of recurrence at a mid-term follow-up. CT-scan seems not to detect those patients at higher risk of recurrence.

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

STUDY OBJECTIVE: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making. DESIGN: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020). PARTICIPANTS: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions. RESULTS: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary. CONCLUSIONS: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

Surgical management of ovarian teratomas in childhood: a multicentric study on 110 cases and a literature review.

AIM OF THE STUDY: To compare a multicentric surgical experience on ovarian teratomas in childhood with the current management trends. DESIGN: A retrospective multicentric pediatric ovarian teratomas surgically treated between January 2000 and August 2020 at four Italian institutions. PubMed database was used to search for Reviews and Systematic Reviews published between January 2010 and August 2020: 15 manuscripts reported 3633 ovarian neoplasms in pediatric age, 1219 (33,5%) of which were ovarian teratomas. RESULTS: A hundred-ten patients with a mean age at diagnosis of 11.8 years were enrolled. Mature cystic teratomas accounted for the 90% of the masses. At surgery, 78 were oophorectomies and 32 were ovary sparing surgeries. Laparoscopy occurred in 16.3% of the surgeries.As regarding the current management trends, the mean age at diagnosis was 11.9 years and 80.5% of the cases were represented by mature teratomas. Of 430 procedures, 331 were oophorectomies while 99 were ovary sparing surgeries and laparoscopy was performed in 23.8% of cases. CONCLUSIONS: Ovary-sparing surgery with laparoscopic approach is increasingly offered as standard treatment for benign masses that fit the criteria for mature teratomas, in the attempt to achieve the best compromise between the preservation of fertility and the prevention of recurrences. Awareness should be raised among pediatric surgeons to reduce unnecessary radical surgery.

Minimally invasive vascular hitch to treat pediatric extrinsic ureteropelvic junction obstruction by crossing polar vessels: A systematic review and meta-analysis.

INTRODUCTION: Vascular hitch (VH) gained an increasing success in treating ureteropelvic junction obstruction (UPJO) by crossing vessels (CV) in pediatrics. AIMS OF THE STUDY: We aimed: (i) to compare laparoscopic VH versus laparoscopic dismembered pyeloplasty (DP) to treat UPJO by CV; (ii) to review possible amelioration given by a robot-assisted procedure. METHODS: Using defined search strategy, three investigators identified all studies on laparoscopic VH. Those studies comparing VH versus DP or versus robot-assisted VH were included in the meta-analysis. The meta-analysis was conducted using RevMan 5.3. Data are mean ± SD. RESULTS: Systematic review - Of 2783 titles/abstracts screened, 43 full-text articles were analyzed. Twelve studies on laparoscopic VH (298 pts) reported 98.3% success rate, with 1.3% intra-operative complications. Meta-Analysis - Five studies compared laparoscopic VH versus laparoscopic DP (277 pts). Operative time was reduced in VH (102.5 ± 47.5min) compared to DP (165.7 ± 53.7min; p < 0.00001). Complications were similar (VH 4/119 pts, 3.4 ± 1.2% versus DP 15/158 pts, 9.5 ± 6.8%; p = ns). Hospital stay was shortened in VH (1.1 ± 0.9dd) versus DP (3.3 ± 3.2dd; p < 0.0001; Summary Figure). The success rate was comparable (VH 115/118 pts, 97.5 ± 1.6% versus DP 157/158 pts, 99.4 ± 0.5%; p = ns). Two prospective studies compared robot-assisted VH to laparoscopic VH (53 pts). No differences were found among complications (robot-assisted VH 0/13 pts, 0% versus laparoscopic VH 1/40 pts, 2.5%; p = ns) and success rate (robot-assisted VH 13/13 pts, 100% versus laparoscopic VH 39/40 pts, 97.5%; p = ns). DISCUSSION: Several studies have been reported long-term results of laparoscopic VH in children. However, few papers demonstrated its superiority over laparoscopic DP to treat extrinsic UPJO. In the present study, we found similar incidence of complications and success rates when comparing VH versus DP. Nonetheless, the operative time and the length of hospital stay were significantly reduced in VH compared to DP. An increasing number of surgeons performed robotic-assisted VH, reporting promising outcomes. However, only a couple of studies compared robot-assisted VH to laparoscopic VH, with a similar incidence of complications and success rate in both procedures. The main limitations of the study were related to the slight number of papers included and to their quality, since all of them were retrospective studies or prospectively followed-up cohort of patients. CONCLUSIONS: Laparoscopic VH seems to be a safe and reliable procedure to treat UPJO by CV. The procedure appeared quicker than laparoscopic DP, with shortened hospital stay. Further studies are needed to corroborate these results and to establish amelioration given by a robot-assisted procedure.

Secondary hydrosalpinx in adolescents: a challenging decision-making process for surgical choice and future fertility preservation.

Hydrosalpinx in pre-pubertal children and non-sexually active adolescents is a rare finding with several etiology and negative impact on future female fertility. The therapeutic approach in these specific populations is debated and it must consider its etiology and adult guidelines focused on fertility issues, which suggest laparoscopic salpingectomy. We described two adolescent cases (15 years asymptomatic and 13 years with abdominal pain) presenting a monolateral hydrosalpinx secondary to surgery for Hirschsprung's disease and complicated appendicitis, respectively. Both patients underwent to uncomplicated robotic-assisted salpingectomy, with uneventful follow-up and preserved ovarian function. Robotic-assisted salpingectomy for hydrosalpinx secondary to previous surgical conditions is a safe and careful approach for adolescents in order to preserve ovarian vascularization, function and future fertility.

Spontaneous regression of cystic dysplasia of the rete testis in an 18-month-old boy: the key role of ultrasonography.

Cystic dysplasia of the rete testis (CDT) is a rare cause of scrotal swelling in children. It is a congenital disorder and it can be associated with other genitourinary abnormalities. At present, there is no clear consensus on treatment. Surgical approach has traditionally been the treatment of choice, while, more recently, conservative approach has been applied, justified by the benign nature of the lesion and after few cases of spontaneous regression have been documented. Ultrasonography, supported by negative tumor markers, plays a key role in the diagnostic work up and during observational follow-up. We report a further case of spontaneous regression of suspected CDT in an 18-month-old boy, who has been followed with clinic and ultrasonographic checks.

Esophageal Atresia and Associated Duodenal Atresia: A Cohort Study and Review of the Literature.

INTRODUCTION: Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. MATERIALS AND METHODS: We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. RESULTS: Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. CONCLUSION: The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

A Rare Case of Giant Mesenteric Lipoblastoma in a 6-Year-Old Child and Review of the Literature.

Giant mesenteric lipoblastoma is a rare benign tumor arising from the adipocytes. It can mimic malignant tumors, and its diagnosis is difficult before surgery. Imaging studies could lead the diagnosis but not confirm it. Those tumors arising in the abdomen are usually larger and can cause symptoms of compression. Surgical excision is the treatment of choice, and a long-term follow-up is necessary to detect local recurrences. Only a few cases of lipoblastomas arising from the mesentery are reported in literature. We present a case of a rare giant lipoblastoma arising from the mesentery of a 6-year-old girl, with a history of postprandial abdominal pain.

Successful lung response after surgical repair in an infant with right-sided congenital diaphragmatic hernia.

Congenital diaphragmatic hernia is a rare condition associated with pulmonary complications as the abdominal viscera herniated into the chest may affect lungs development. We present the case of a male newborn baby with a prenatal diagnosis of a posterolateral defect (Bochdalek hernia) involving the right side. The infant underwent surgical repair at 3 days of life, and the post-surgery chest X-ray did not reveal morpho-structural alterations of the lungs and diaphragmatic profile. Our clinical case shows that patients may have a better lung outcome despite an initial unfavorable picture. Prenatal diagnosis is essential in identifying infants with congenital diaphragmatic hernia, especially those cases at higher risk for the worse outcomes, to optimize their clinical and surgical management.

Improving standard of pediatric surgical care in a low resource setting: the key role of academic partnership.

BACKGROUND: An epidemiological transition is interesting Sub-Saharan Africa increasing the burden of non-communicable diseases most of which are of surgical interest. Local resources are far from meeting needs and, considering that 50% of the population is less than 14 years of age, Pediatric surgical coverage is specially affected. Efforts are made to improve standards of care and to increase the number of Pediatric surgeons through short-term specialist surgical Missions, facilities supported by humanitarian organization, academic Partnership, training abroad of local surgeons. This study is a half term report about three-years Partnership between the University of Chieti- Pescara, Italy and the University of Gezira, Sudan to upgrade standard of care at the Gezira National Centre for Pediatric Surgery (GNCPS) of Wad Medani. Four surgical Teams per year visited GNCPS. The Program was financed by the Italian Agency for Development Cooperation. METHODS: The state of local infrastructure, current standard of care, analysis of caseload, surgical activity and results are reported. Methods utilized to assess local needs and to develop Partnership activities are described. RESULTS: Main surgical task of the visiting Team were advancements in Colorectal procedures, Epispadias/Exstrophy Complex management and Hypospadias surgery (20% of major surgical procedures at the GNCPS). Intensive care facilities and staff to assist more complex cases (i.e. neonates) are still defective. Proctoring, training on the job of junior surgeons, anaesthetists and nurses, collaboration in educational programs, advisorship in hospital management, clinical governance, maintenance of infrastructure together with training opportunities in Italy were included by the Program. Despite on-going efforts, actions have not yet been followed by the expected results. More investments are needed on Healthcare infrastructures to increase health workers motivation and prevent brain drain. CONCLUSIONS: The key role that an Academic Partnership can play, acting through expatriated Teams working in the same constrained contest with the local workforce, must be emphasized. Besides clinical objectives, these types of Global Health Initiatives address improvement in management and clinical governance. The main obstacles to upgrade standard of care and level of surgery met by the Visiting Team are scarce investments on health infrastructure and a weak staff retention policy, reflecting in poor motivation and low performance.

Evidence for oxidative and not reductive stress in the aged rabbit heart.

Reductive stress, which is due to a paradoxical excess of antioxidants such as reduced glutathione (GSH) and GSH-related enzymes associated with decreased oxidant levels, has emerged as a pathogenetic mechanism of myocardial damage in pathological conditions such as protein aggregation cardiomyopathy. Notably, in the aged heart a cardiomyopathy-like pathology occurs leading to myocardial dysfunction. Whether reductive stress, or instead its counterpart oxidative stress, is operative in the aged mammalian heart needs to be elucidated also for the different therapeutic implications of such redox stress conditions. In the present investigation, we assessed GSH and the specific enzymatic activities of γ-glutamylcysteine synthetase (γ-GCS), glutathione reductase (GSSG-Red) and selenium-dependent glutathione peroxidase (GSH-Px) as endogenous antioxidants, together with oxidized glutathione (GSSG) and the glutathione redox ratio (GSH/GSSG), in the aerobically perfused hearts of aged rabbits (about 4.5 years old) and young adult control rabbits (3-4 months old). We also assessed in the aged and control hearts H2O2 and catalytically active low molecular weight iron (LMWI) as oxidant forces, as well as fluorescent damage products of lipid peroxidation (FDPL) and protein carbonyls (PC) as biomarkers of lipid and protein oxidation. Moreover, the effects of 4.5 mM N-acetylcysteine (NAC) as reducing thiol antioxidant were studied on hemodynamic parameters and lipid peroxidation in the perfused hearts of the aged and control rabbits. The levels of GSH and of the GSH/GSSG ratio were lower, and those of GSSG higher, in the aged than in the control hearts. The aged hearts were also characterized by decreased activities of the antioxidant enzymes γ-GCS, GSSG-Red and GSH-Px, as well as by heightened levels of H2O2, LMWI, FDPL and PC, highlighting the occurrence of aging-dependent oxidative stress. Associated with such biochemical alterations, hemodynamic dysfunction occurred in the aged rabbit hearts, as evidenced by lowered developed pressure (DP) and enhanced end-diastolic pressure (EDP) with decreased coronary flow (CF). Remarkably, NAC administration significantly improved DP and EDP, and lowered lipid peroxidation, electively in the aged hearts. In conclusion, oxidative and not reductive stress is operative in the aged rabbit heart, whose hemodynamic dysfunction is improved by NAC together with reduction in myocardial lipid peroxidation.

Conversion Rate in Pediatric Robotic-Assisted Surgery: Looking for the Culprit.

Introduction: Robotic-assisted surgery (RAS) is increasingly used in adulthood but its application in pediatric population is limited. We report our initial experience in pediatric RAS, focusing on conversions to analyze their causes. Methods: All pediatric patients who underwent RAS between June 2015 and April 2019 were included, analyzing demographics, comorbidities, previous surgery, and intraoperative surgical and anesthetic parameters. A three-arms robotic technique was used in all cases. Additional laparoscopic ports were added, when needed. The surgical team did not change during the program, whereas the anesthesiology team varied. Results: Thirty-nine patients (23 females, 16 males; mean age ± SD = 9.33 ± 4.73 years [range = 1-16]; mean weight ± SD = 35.2 ± 20.0 kg [range = 9-85]) underwent 40 different procedures (18 gastrointestinal, 15 urogynecological, 5 oncological, and 2 miscellaneous). Three procedures (7.5%) were converted to open surgery for inadequate working space (two marked bowel distension and one insufficient hepatic retraction). Converted patients were of significant lower age (mean ± standard error of mean [SEM] = 2.97 ± 1.03 versus 9.83 ± 0.77 years, P = .01) and lower weight (mean ± SEM = 11.83 ± 1.74 versus 35.47 ± 3.16 kg, P = .03). The two groups did not differ statistically for duration of facial mask ventilation before intubation (mean ± SEM = converted 10.67 ± 2.33 versus completed 10.31 ± 0.91 minutes), neuromuscular block dosage (rocuronium; mean ± SEM = converted 0.46 ± 0.06 mg/kg versus completed 0.62 ± 0.03 mg/kg) and in the type of bowel preparation (mechanical and/or pharmacological). Discussion: Conversion rate in initial pediatric RAS program is acceptable. In children, the need for conversion is mainly because of inadequate working space, particularly in smaller children, but it seems not to be influenced by measurable anesthetic factors or different regimen for bowel preparation.

Holmium: Yttrium-aluminum-garnet laser for endoscopic decompression of ureterocele in the first months of life: A comparison with electrosurgery.

INTRODUCTION: Few case series report the use of holmium: yttrium-aluminum-garnet (Ho:YAG) laser to decompress ureterocele (UC) in pediatric population, and only two studies compared its outcomes with electrosurgery. This study aims to compare outcomes of Ho:YAG laser transurethral endoscopic puncture (TUP) versus electrosurgery TUP of UC in the 1st month of life, analyzing incidence of secondary surgery, redo TUP, and iatrogenic vesicoureteral reflux (VUR). PATIENTS AND METHODS: A retrospective study of patients treated by TUP of UC from 2008 to 2017 was performed. Those undergoing Ho:YAG laser TUP were included in Group A, those undergoing electrocautery TUP were included in Group B. Data were compared using Fisher's exact test. RESULTS: Group A included seven patients (mean follow-up 4 years). Two required a redo TUP. Two had preoperative VUR, which resolved after TUP. Two developed VUR after TUP, which resolved spontaneously. No secondary surgery was required. Group B included nine patients (mean follow-up: 9.5 years). One required a redo TUP. Preoperative VUR was detected in 4/9 and persisted after TUP in 2. Three developed post-TUP VUR, which persisted. Five required further surgery because of persistent and symptomatic VUR. Secondary surgery was significantly lower after Ho:YAG laser compared to electrocautery TUP (P < 0.05). The incidences of both redo TUP and postoperative VUR were not significantly different between the two groups (P = ns). CONCLUSION: Ho:YAG laser TUP seems to be safe and effective in the decompression of obstructive UCs and maybe advantageous over electrocautery puncture. However, further studies with larger cohort are needed to corroborate our preliminary results.

Contrast-enhanced ultrasonography (CEUS) in the follow-up of pediatric abdominal injuries: value and timing.

AIMS: Primary aim was to investigate the value and safety of contrast-enhanced ultrasonography (CEUS) during follow-up (FU) of splenic, hepatic and renal post-traumatic injuries in a pediatric population. Secondary aim was to extrapolate appropriate timing of FU-CEUS. METHODS: In a retrospective study, post-traumatic parenchymal injuries diagnosed with CT or CEUS, were subjected to non-operative management and followed with CEUS. RESULTS: Forty-six patients were enrolled, with isolated or combined injuries, for a total of 30 splenic, 15 hepatic and 12 renal injuries. At admission 42/46 patients underwent CT and 4/46 underwent CEUS. During FU a total of 65 CEUS were performed: 16 within 72 h to check delayed active bleeding or parenchymal rupture; 24 between 5 and 10 days post admission, to pose indication to active mobilization or to discharge; 21 between 20 and 60 days post admission to document complete healing of the lesion or pose indication to discharge in most severe injuries. No complications related to CEUS were encountered. CONCLUSIONS: CEUS is valuable and safe to follow patients with post-traumatic abdominal injuries, even if further data are needed for renal injuries. We propose a tailored approach based on injury grade and clinical course: in the first 3 days only in case of delayed bleeding or rupture suspect; between 5 and 10 days post trauma to ensure a safe active mobilization and/or pose indication to discharge, and over 20-30 days post trauma to pose indication to discharge in most severe injuries or document complete healing and permit return to sport activities.

Management of Spontaneous Pneumothorax in Children: A Systematic Review and Meta-Analysis.

INTRODUCTION: Management of primary spontaneous pneumothorax (PSP) is mainly based on adults. Data are controversial with regards to its management in children. We aimed to assess: (1) the length of hospital stay (LOS) between conservative management (i.e., observation with O2 administration), aspiration/chest drain, and surgical management; (2) the risk of recurrence after nonsurgical treatment versus surgery; (3) the risk of recurrence in the presence of bullae. MATERIALS AND METHODS: Using a defined search strategy, three independent investigators identified all the studies on the management of PSP in children. Case reports, opinion articles, and gray literature publications were excluded. The study was conducted according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. A meta-analysis was performed using RevMan 5.3. Data are expressed as mean ± SD. RESULTS: Of 3,089 abstracts screened, 95 full-text were analyzed, 23 were included in the quantitative analysis, and 16 were included in the meta-analysis (1,633 patients). LOS was similar between conservative and surgical management (6.2 ± 0.8 days vs. 5.9 ± 1.4 days; p = ns). Recurrence of PSP was significantly higher among children with a nonsurgical management (32%) versus those surgically treated (18%; p = 0.002). The incidence of recurrence was slightly higher in patients managed by aspiration/chest drain (34%) compared with those with a conservative management (27%; p = 0.05). Risk of recurrence in patients with or without documented bullae was not significantly different (26 vs. 38%, respectively; p = ns). CONCLUSION: Given the lack of a standardized management of pediatric PSP, the present study seems to demonstrate a better outcome in children treated with surgery as first-line of management. LEVEL OF EVIDENCE: This is a Level III study.

Serum Steroid Profiling by Liquid Chromatography-Tandem Mass Spectrometry for the Rapid Confirmation and Early Treatment of Congenital Adrenal Hyperplasia: A Neonatal Case Report.

Congenital adrenal hyperplasia (CAH) describes a group of autosomal recessive disorders of steroid biosynthesis, in 95% of cases due to 21-hydroxylase deficiency. The resulting hormonal imbalances lead to increased 17-hydroxyprogesterone and androgens levels, at the expense of decreased concentrations of glucocorticoids and, in some cases, of mineralocorticoids. A variety of clinical presentations accompany a range of severities, which are described as different forms of CAH, and are the result of these hormonal imbalances. The incidence of CAH worldwide is approximately 1 in 15,000 live births, and is population-dependent; thus, its inclusion in neonatal screening tests is widely discussed. Diagnosis of CAH is based on the quantification of 17-hydroxyprogesterone, usually by immunoassay, which has low specificity and high false-positive rates, resulting in a relatively high demand for a second-tier confirmation test. We report a case of a newborn recognized as female at birth, but showing ambiguous genitalia and other CAH clinical features, including hypernatremia, in the first days of life. In addition to the classical assays, liquid chromatography-tandem mass spectrometry was used to determine the serum steroid profile, allowing for the accurate and simultaneous quantification of seven steroids in the same analysis. Such an application immediately revealed an alteration in the levels of specific steroids related to CAH, leading to an early intervention by hormone replacement therapy. Subsequently, the diagnosis of classic CAH due to 21-hydroxylase deficiency was further confirmed by molecular testing.

Yq Microdeletion in a Patient with VACTERL Association and Shawl Scrotum with Bifid Scrotum: A Real Pathogenetic Association or a Coincidence?

VACTERL association is defined by the occurrence of congenital malformations: vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula with esophageal atresia, radial and renal dysplasia, and limb defects. No genetic alterations have been discovered except for some sporadic chromosomal rearrangements and gene mutations. We report a boy with VACTERL association and shawl scrotum with bifid scrotum who presented with a de novo Yq11.223q11.23 microdeletion identified by array CGH. The deletion spans 3.1 Mb and encompasses several genes in the AZFc region, frequently deleted in infertile men with severe oligozoospermia or azoospermia. Herein, we discuss the possible explanation for this unusual genotype-phenotype correlation. We suggest that the deletion of the BPY2 (previously VCY2) gene, located in the AZFc region and involved in spermatogenesis, contributed to the genesis of the phenotype. In fact, BPY2 interacts with a ubiquitin-protein ligase, involved in the SHH pathway which is known to be implicated in the genesis of VACTERL association.

Zinner Syndrome in Pediatric Age: Issues in the Diagnosis and Treatment of a Rare Malformation Complex.

Zinner syndrome (ZS) is the association of congenital seminal vesicle cysts and ipsilateral upper urinary tract anomalies, such as multicystic displastic kidney (MCDK). This condition is rare in pediatric age and both diagnosis and treatment are challenging. The aim of this study was to analyze the issues in diagnosis, management, and treatment of ZS in pediatric age. The medical records of two patients with ZS were examined. Furthermore, a review of the literature on this topic in pediatric age was performed. In our experience the diagnosis of ZS was incidentally achieved in the first months of life, as a consequence of studies performed for a prenatal diagnosis of MCDK. The first patient presented unspecific and transient symptoms, the second infant was completely asymptomatic. They were conservatively treated, with a long-term follow-up planned at least until adolescence. Fifty cases of ZS in pediatric age have been reported in the literature up to now. Only 12/50 were diagnosed in the first year of life. The diagnosis was demanding, as the clinical presentation was unspecific and the results at imaging studies needed a differential diagnosis with other retrovesical masses. More than 80% of these cases were asymptomatic at long-term follow-up. Therefore, a conservative management of ZS has been accepted for asymptomatic or poorly symptomatic patients, with occasional, transient, and unspecific symptoms, such as urinary tract infections or orchyepididimytis. As the surgical management is challenging, it is proposed only in those symptomatic patients. In conclusion, ZS is rare in pediatric age. However, it should be considered in the differential diagnosis of cystic masses within the pelvis in males with ipsilateral renal anomalies. A conservative treatment with a long-term follow-up is a safe option in the management of asymptomatic or poorly symptomatic patients, thus reserving the surgical approach only in those cases with symptoms.