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.

Rectal Bleeding in Infants: Diagnostic Work-up and Management.

Lower gastrointestinal bleeding is an alarming symptom in pediatrics, especially in infancy. However, it is commonly secondary to benign and self-limiting conditions, such as anal fissures, infections, and allergies; more rarely it is caused by more serious disorders, such as necrotizing enterocolitis, very early onset inflammatory bowel diseases, and vascular malformations. The present review aims at summarizing the different clinical conditions presenting with rectal bleeding in infancy and provides an evidence-based diagnostic work-up for the clinical management of patients with this occurrence.

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.

Use of Meckel's diverticulum as a temporary diverting ostomy.

Meckel's diverticulum is among the most common congenital defects of the gastrointestinal tract, and is associated with a total lifetime risk of complications around 4%. While debate on prophylactic resection of incidental Meckel's diverticulum continues, there have been reports of its successful use for urinary diversion and reconstruction. In contrast, its use as a means of fecal diversion has been described anecdotally. Herein, we describe our technique of temporary fecal diversion using Meckel's diverticulum as reliable conduit for stoma formation in a toddler. The stoma functioned well until continuity of bowel was restored and diverticulum resected safely. We trust that our limited experience will encourage other colleagues to test the inventive use of Meckel's diverticulum as a potentially safe and effective option to fit in the surgical armamentarium for temporary fecal diversion. KEY WORDS: Anastomotic leak, Fecal diversion, Ileostomy, Meckel's diverticulum, Stoma.