Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

Female splenogonadal fusion: A PLEA for conservative management.

Splenogonadal fusion in female patients is seldom reported. We describe a 6-month-old girl who represents the youngest living female with splenogonadal fusion reported to date. The lesion was diagnosed as an incidental finding during screening abdominal ultrasonography performed for a vulvar infantile hemangioma. A tail-like structure with splenic echotexture connecting a normally located spleen and the left ovary was detected and better characterized by MRI. We also reviewed the pertinent literature on managing this usually asymptomatic condition, especially in female patients. Greater professionals' awareness of this benign anomaly is paramount to avoid the unnecessary removal of an otherwise normal gonad.

Early Surgery Versus Watchful Waiting Strategy for Infantile Abdominoscrotal Hydrocele.

BACKGROUND: Infantile abdominoscrotal hydrocele is generally managed with early surgery. However, whether these patients actually benefit from an initial watchful waiting strategy is yet to be determined. METHODS: This single-center, comparative observational analysis included 36 consecutive patients (9 bilateral cases) diagnosed between January 1998 and December 2019. Initial 16 patients (21 lesions) underwent surgical repair shortly after diagnosis (Group A) at a mean (SD) age of 8 (4) months. Remaining 20 patients (24 lesions) underwent initial nonoperative management (Group B). Overall follow-up was 82.74 (63.84) months. RESULTS: Six lesions of Group B spontaneously regressed at a mean age of 23.8 (7.8) months. Remaining 18 hydroceles underwent surgical management at a mean age of 18.7 (12.6) months. Early postoperative morbidity was high (43%) in both groups (Clavien-Dindo grade I-II). During follow-up, there were 1 recurrent hydrocele and 1 testicular atrophy, both events occurring in Group A patients. Group B patients required a higher need for concomitant ipsilateral orchidopexy (61% versus 14%; p = 0.001), and developed a higher rate of ipsilateral inguinal hernia (39% versus 5%; p = 0.01), occurring within 8.3 (3.53) months of surgery. On multivariable analysis, nonoperative management was associated with unplanned second surgery (odds ratio 5.5, 95% CI 1.25-24.17, p = 0.02), regardless of the type of hydrocelectomy performed. CONCLUSIONS: Nonoperative management provides effective spontaneous resolution in about 25% of infantile abdominoscrotal hydrocele. Clinicians must balance the potential benefits of such strategy against the impact of pressure induced detrimental effects on the success of future surgery when dealing with longstanding lesions. LEVEL OF EVIDENCE: III.

The Role of Manual Detorsion in Pediatric Testicular Torsion During the Global COVID-19 Pandemic: Experience From 2 Centres.

OBJECTIVE: To evaluate the role of emergency manual detorsion as first line management for testicular torsion in the context of the COVID-19 pandemic. METHODS: This retrospective observational study includes 90 pediatric patients ≤14 years old with diagnosis of testicular torsion made at 2 tertiary centers between October 2020 and June 2022. Variables examined included age, presentation delay, surgical wait time, number of attempts at manual testicular detorsion, and manual testicular detorsion success. All patients finally underwent surgery, including contralateral testicular fixation. Outcomes included predictors of successful manual detorsion, testicular findings at surgery, and operation time. RESULTS: Mean (SD) age at diagnosis was 11.51 (2.64) years. Mean presentation delay was 11.76 (13.79) hours. Detorsion was attempted in 72 (80%) patients, resulting successful in 58 (80.5%). Surgical wait time after successful manual detorsion was 22.85 (16.94) hours. On multivariable analysis, successful manual detorsion was associated with a presentation delay<6 hours (odds ratios [OR] 0.154, 95% confidence intervals (CI) 0.036-0.655, P = 0.01) and absence of scrotal edema (OR 0.171, 95% CI 0.038-0.769, P = 0.02). Vice versa, a heterogeneous echo-texture (OR 0.57, 95% CI 0.007-0.461, P = 0.007) and absent blood flow on Doppler ultrasound scan (OR 0.256, 95% CI 0.067-0.971, P = 0.045) were significantly associated with the likelihood of manual detorsion failure. CONCLUSION: In our experience, manual detorsion provided safe and effective emergency treatment for pediatric testicular torsion, especially in absence of edema and when presentation delay is <6 hours. This maneuver should be more widely attempted immediately after diagnosis as temporizing rescue.

Prevalence, management, and outcome of cryptorchidism associated with gastroschisis: A systematic review and meta-analysis.

PURPOSE: Cryptorchidism associated with gastroschisis has been poorly investigated. We aimed to assess the prevalence of this association, and to address the management and outcome of cryptorchidism in this context. MATERIALS AND METHODS: In this systematic review, we searched electronic databases (PubMed, Web of Science, and Google Scholar), without language restrictions from inception to March 31, 2021, for studies on cryptorchidism and gastroschisis co-occurrence. Random effects meta-analysis was used to calculate pooled prevalence estimates of cryptorchidism in gastroschisis and spontaneous testicular descent in this population. RESULTS: Twenty-five studies were included for the systematic review. All were retrospective case reports or series for a total of 175 patients. Pooled prevalence estimate for cryptorchidism in gastroschisis was 19% (95% CI 13-26). Complete data sets for management and outcome analysis were available in 94 patients, involving 120 undescended testes [63 (52.5%) nonpalpable, 34 (28.3%) prolapsed outside the abdominal wall defect, and 23 (19.2%) palpable]. Fifty-five descended spontaneously, with a pooled prevalence estimate of 50% (95% CI 31-69). Twelve testes/remnants were primarily removed or absent, while 49 testes underwent orchiopexy (success rate, 63%). Excluding 4 testes still awaiting orchiopexy, 30 of the remaining 116 (25.8%) testes had a dismal outcome, including testicular loss (19), hypoplasia (8) or recurrent cryptorchidism (3). CONCLUSION: Cryptorchidism in gastroschisis appears to occur more frequently than in the normal population. The overall mediocre testicular outcome seems to reflect the most unfavorable presentation of cryptorchidism in gastroschisis, which is represented by originally intrabdominal or prolapsed testis in the majority of cases. LEVEL OF EVIDENCE: IV.

Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review.

Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years.

Prolonged Indwelling Urethral Catheterization as Minimally Invasive Approach for Definitive Treatment of Posterior Urethral Valves in Unstable Premature Babies.

Premature newborns with posterior urethral valves (PUV) may present with medical conditions taking priority over definitive surgical care. We encountered three of such cases who underwent initial bladder decompression via transurethral catheterization and waited 2-3 weeks until they were fit enough for voiding cysto-urethrography to confirm PUV. An unexpected good urinary flow and negligible residual urine volume were documented during micturition, suggestive of valve disruption induced by insertion and prolonged duration of indwelling urethral catheter drainage. Cystoscopy documented non-obstructing remnant leaflets. Non-operative treatment may be considered as a viable alternative therapeutic option for PUV in tiny babies facing prolonged intensive care unit stay.

Zero-ischemia testis-sparing surgery for probably benign pediatric testicular tumors.

Permanent ischemia-induced testicular damage may occur as early as 30 min in prepupertal rats. With the goal of potentially enhancing testicular function and fertility preservation, we performed testis-sparing surgery (TSS) without ischemia for testicular lesions in select children with negative markers and high likelihood of benignity on ultrasonography. Preliminary experience suggests that off-clamp TSS should be more liberally encouraged, especially in infants and prepubertal children, given their particularly vulnerable spermatic cord elements.

Feasibility, safety and outcome of inguinal hernia repair under spinal versus general anesthesia in preterm and term infants.

BACKGROUND: Inguinal hernia repair (IHR) is a common operation in preterm and term infants. Recently, spinal anesthesia (SA) has been proposed as an alternative to avoid exposure to general anesthesia (GA) during early life. The aim of this study was to compare surgical outcomes of open IHR performed under SA versus GA in neonates and infants, and to detect criteria to predict the success or failure of SA. MATERIALS AND METHODS: This is a 6-year, single center, nonrandomized interventional study (2013-2019). SA was performed with 0.5% bupivacaine. GA was given using propofol, fentanyl, sevoflurane, and laryngeal mask. Patient demographics, operative time, intraoperative events related to surgery or anesthesia, and complications were analyzed at short and long-term follow-up. RESULTS: 68 infants (78 IHR) and 37 infants (44 IHR) received SA and GA at the discretion of the anesthesiologist, respectively. SA failure rate was 9%, and positively correlated with weight at surgery (p = 0.001; rp = 0.38). Conversion from SA to GA occurred in 4 (6%) patients owing to prolonged operative time (43.75 ±â€¯4.8 vs 23.02 ±â€¯11.3 min; p = 0.0006). There were no differences regarding operative time and intra- and postoperative complications among the two groups at mean follow-up of 18.53 ±â€¯21.9 months. CONCLUSIONS: This pilot study confirms that SA is safe, effective and not detrimental to surgical outcome of neonates and infants undergoing IHR. Additionally, it may help further define what patients may have a successful SA. Our experience suggests that SA is especially suitable in infants weighing <4000 g, and conversion to GA correlates with prolonged operative time. LEVEL OF EVIDENCE: Level II.

Laparoscopic patch repair of a Morgagni hernia in Menkes disease.

The association between Morgagni hernia and Menkes disease has not yet been described. Here, we report such a rare association in an 8-year-old boy who presented with subocclusive symptoms. He successfully underwent laparoscopic repair with a patch. The patch was fixed to the anterior abdominal wall by using transfascial sutures with extracorporeal knot tying and to the remaining edges of the diaphragmatic defect by using intracorporeal suturing and spiral tacks. At the 2-year follow-up, the child remained recurrence-free and without gastrointestinal symptoms. The potential relationship between the two conditions and the controversial use of spiral tacks to affix the mesh to the diaphragm are also discussed.

Resection of Inferior Vena Cava Without Reconstruction for Intravascular Intrusion of Wilms Tumor.

Persistent inferior vena cava (IVC) tumor thrombus in Wilms tumor patients represents a management challenge. We describe three pediatric cases with preoperative evaluation documenting complete IVC occlusion and well-developed collaterals. They underwent nephrectomy and tumor thrombus removal accomplished with circumferential resection of the retrohepatic IVC without vascular reconstruction. All patients are asymptomatic and disease-free at 9, 2.5, and 2 years after stopping therapy. Cavectomy without reconstruction is safe and well tolerated in Wilms tumor patients with completely occlusive IVC tumor thrombus. Additionally, when performed en bloc with nephrectomy and with clear margins, cavectomy obviates the need for radiotherapy per protocol.

The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia]. METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes. RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean. CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

Volume and function of the operated kidney after nephron-sparing surgery for unilateral renal tumor.

AIM: We sought to assess the magnitude of functional decline and the natural history of the operated kidney residual function after zero-ischemia nephron-sparing surgery (Z-NSS) in children with unilateral renal tumor (URT). PATIENTS AND METHODS: 50 children were treated for URT at our surgical unit between 1992 and 2016. Of these 12 who underwent Z-NSS were available for the current analysis. Operated kidney function was assessed by 99mTc-dimercapto-succinic acid (DMSA) renal scintigraphy. Operated kidney volume was assessed by renal ultrasonography. RESULTS: A positive correlation between split renal function and split renal volume was found (P = 0.001). The subset of patients with ≥40% preservation of operated kidney function/volume (OKF/V) had no-time dependent changes during adolescence. The subset of patients with <40% OKF/V preservation had a catch-up growth that after puberty reached values not much different from those with ≥40% OKF/V preservation. At 5 years of follow-up, 3 of 5 patients with baseline dysfunction (eGFR between 40.8 and 89.4 ml/min/1.73 m2) presented with a global renal function within normal range. After puberty, all patients presented with global renal function within normal values (eGFR between 95 and 151 ml/min/1.73 m2). CONCLUSIONS: In children with URT who underwent Z-NSS, the pattern of OKF/V recovery suggests that compensatory catch-up growth capacity during childhood minimizes OKF/V decline more than Z-NSS. LEVEL OF EVIDENCE: Level I prognosis study - prospective cohort study with >80% follow-up and all patients enrolled at same time point in disease.

Cardiogenic Necrotizing Enterocolitis: A Clinically Distinct Entity from Classical Necrotizing Enterocolitis.

AIM: The main purpose of this study was to investigate if necrotizing enterocolitis (NEC) has a different presentation and outcome in patients with congenital heart defect (CHD) (cardiogenic NEC) from those without (classical NEC). MATERIALS AND METHODS: A systematic review of the literature on the characteristics of infants with NEC and CHD was performed by three independent investigators using a defined strategy (PubMed, Cochrane, Embase, and Web of Science). A meta-analysis was conducted on studies comparing NEC in infants with CHD and non-CHD infants using RevMan 5.3. RESULTS: Systematic review: Of 7,291 abstracts screened, 126 full-text articles were analyzed and 51 studies were included. NEC had an incidence of 5.1% in CHD infants (7,728/151,046, range 0-24%) and 0.8% in non-CHD infants (26,430/3,256,891, range 0.1-8.9%; p < 0.0001). In very low birth weight infants, NEC occurred in 6.3% of CHD patients (6,361/100,454pts) and in 8.9% of non-CHD (23,201/257,794pts; p < 0.0001). In CHD cases, NEC occurred before cardiac surgery in 48% cases and surgery for NEC was required in 31% infants (2,037/6,683). Meta-analysis: In eight comparative studies, the incidence of NEC was higher in CHD infants (6%, 768/13,145) than in infants with no CHD (0.9%, 32,625/3,354,323pts; p < 0.00001, odds ratio [OR] 1.84, 95% confidence interval [CI] 1.7-1.9). The overall mortality was higher in infants with CHD and NEC (38%, 243/640) than in those without CHD (27%, 6651/24810; p < 0.00001, OR 3.4, 95% CI 2.8-4.1). CONCLUSION: This is the first evidence-based study showing that infants with cardiogenic NEC have different demographics and outcomes than those with classical NEC. The risk of developing NEC and the mortality rate are higher among infants with CHD than in those without. Conversely, the need for intestinal surgery is lower in babies with cardiogenic NEC than in those with classical NEC. Further studies are needed to establish preventative and management interventions that are specific to infants with or at risk of developing cardiogenic NEC.