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.

Midazolam premedication in ileocolic intussusception: a retrospective multicenter study.

Ileocolic intussusception is a common cause of bowel obstruction. When spontaneous reduction does not occur, non-operative management through enema reduction is necessary. Despite the evidence indicating that sedatives favor success in the reduction, their use is still not a common practice. To determine if midazolam (MDZ) before enema improves the rate of procedure success, we retrospectively reviewed charts of patients admitted to two Italian pediatric emergency departments. Outcome measures were the success rate of the enema, recurrence, and need for surgery. Patients were grouped according to the use of MDZ or not, before hydrostatic reduction attempt. We included 69 and 37 patients in the MDZ and non-MDZ groups, respectively. The two groups did not differ in demographics, clinical characteristics, and ultrasound findings. Intussusception reduction after the first enema attempt occurred in 75% (MDZ group) and 32.4% (non-MDZ group) of patients (P < .001); 27.9% (MDZ group) and 77.8% (non-MDZ group) of patients underwent surgery (P < .001). Among them, spontaneous reduction of intussusception during the induction of general anesthesia occurred in 31.6% and 42.9% of patients, respectively (P .43). Multivariate logistic regression analysis showed that only MDZ had a positive effect on the result of the enema (OR 7.602, 95%CI 2.669-21.652, P < .001). CONCLUSION: Procedural sedation with MDZ for enema reduction of intussusception can increase the success rate and lead to a better management of patients. WHAT IS KNOWN: • Despite the evidence of the usefulness of sedatives in the reduction of intussusception, their use is still not a common practice. WHAT IS NEW: • Midazolam during enema reduction of intussusception can increase the success rate and consequently lead to better management of patients.

Nuss Technique for Pectus Excavatum in Adult Patients: Cosmetic Satisfaction and Improvement of Quality of Life in a Single-Center Experience.

Objectives: Since its introduction, the Nuss minimally invasive procedure for pectus excavatum (PE) repair (MIRPE) has become the method of choice. The current study describes our experience of PE correction in adults, with particular focus on postoperative outcomes, pain, quality of life, and patients' satisfaction. Methods: We enrolled for this observational study n = 93 adult patients from 2011 to 2018. The Haller index was used to quantify PE severity. Pulmonary function tests and cardiac examinations were performed preoperatively; we developed a standardized surgical technique and postoperative treatment, including follow-up at 3, 12, and 24 months after surgery and 6 months after bar removal. We also evaluated the quality of life and the satisfaction with the cosmetic result after the procedure with standardized questionnaires. Results: No operative or perioperative deaths occurred nor life-treating complications. Thirteen complications occurred in 12 patients, with a total complication rate of 14% (n = 13/93). Pain intensity decreased in the follow-up [pain score visual analog scale at 3 months: median 1 (0-8); 12 months: median 1 (0-5); and 24 months: median 1 (0-4)]. Better or much better quality of life after the Nuss procedure was observed: n = 79 (84.1%) at 3 months, n = 80 (86%) at 12 months, and n = 85 (91.4%) at 24 months. After 2 years of observation, more than 90% of patients described improvement in their quality of life and satisfaction with the cosmetic results. Only a very small group of patients suffered from pain in the follow-up. Conclusion: Our results demonstrate that the MIRPE procedure is safe and can be performed with excellent results in adults both for improvement of quality of life and for satisfaction with cosmetic results.

Uniportal Video-Assisted Thoracic Surgery in a Pediatric Hospital: Early Results and Review of the Literature.

Background: Uniportal video-assisted thoracic surgery (U-VATS) is an implemented technique in adult surgery that may aid to extend offer the benefits of thoracoscopy to a wide number of pediatric patients. Materials and Methods: Consecutive cases treated between July 2019 and July 2021 were retrospectively analyzed. Simultaneously, a MEDLINE systematic search was conducted. Results: Twelve patients (median age 13 years, median weight 44.5 kg) underwent 4 major procedures (n = 2 lobectomy, n = 2 segmentectomy) and 11 minor procedures (n = 1 bronchogenic cyst resection, n = 4 apical wedge resections and pleurodesis for pneumothorax, n = 4 wedge resections for lung nodules, and n = 2 debridement for empyema). The median observed operative time was 77 minutes. We recorded one conversion to biportal VATS. No intraoperative complications or 30-day morbidity-mortality was reported. A rate of 40% adverse postoperative events was observed (Clavien-Dindo grade I-IVa). Visual analog scale for postoperative pain recorded a median value of 0 on days 1, 2, and 3. The systematic review provided 15 full-text articles reporting 76 pediatric interventions (4 major and 72 minor procedures); among them, 1 biportal conversion, 3 mild postoperative complications, and 1 redo surgery are presented. Conclusions: As emerged from the literature review, U-VATS remains scarcely adopted by pediatric surgeons. Its feasibility is supported by the four reported major lung resections plus the four cases added on by our series. Thanks to a more rapid learning curve over conventional VATS, the uniportal technique could be accessible to a wider number of centers.

Thyroglossal duct cyst: Factors affecting cosmetic outcome and recurrence.

BACKGROUND: Thyroglossal duct cyst (TDC) is the most common congenital abnormality in the neck in children. The purpose of this study was to perform a comprehensive review of all cases of TDC surgically treated at a single institution and to evaluate the factors that influence the rate of recurrence, and the aesthetic outcome of the surgery on follow up. METHODS: All cases of TDC surgically treated at the Department of Pediatric Surgery at Meyer Hospital from January 2005 to December 2016 were selected. Charts from 248 patients were reviewed and risk factors for recurrence evaluated. A questionnaire was submitted to the patients' parents to determine if postoperative complications were present  and standardized neck pictures were requested, to evaluate the cosmetic result . Microsoft Office Excel 2007 for Windows and Graphpad Prism 6 were used for data management and statistical analysis. RESULTS: Simple cyst excision and post-inflammatory fibrosis (P < 0.05) were assessed as important risk factors for the recurrence of TDC. Recurrence rate on Sistrunk procedure was 5%. Variables such as post-inflammatory fibrosis before surgery (P < 0.001), the positioning of a drain (P < 0.01) and the development of recurrence (P < 0.001), negatively influenced the cosmetic result. No thyroglossal duct cyst carcinoma and no long-term postoperative complications were observed. CONCLUSIONS: Recurrence rates were higher in patients who underwent simple cyst excision instead of the Sistrunk procedure, as already reported in literature. Presence of post-inflammatory fibrosis and positioning of the drain at surgery were associated with higher rates of recurrence, as well as worse cosmetic outcome.

A Novel Objective Approach to the External Measurement of Pectus Excavatum Severity by Means of an Optical Device.

BACKGROUND: Current approaches to quantifying the severity of pectus excavatum require internal measurements based on cross-sectional imaging. The aim of this study is to exploit a novel index evaluated on the external surface of the chest with a three-dimensional (3D) optical scanner. METHODS: Fifty-one children (41 male, 10 female) between 2 and 17 years of age were evaluated with a 3D optical scanner. Pectus excavatum severity was calculated by using an ad hoc instant 3D scanner and defining an automatic procedure to generate an optical 3D correction index (CI3D). For the latter, an ideal threshold was derived from a statistical analysis, and five blind surveys were collected from pediatric specialists on chest wall deformities. The CI3D was then correlated with blind clinical assessments of PE severity. RESULTS: The cutoff thresholds were determined to optimally discriminate between six degrees of severity of PE patients by a correlation analysis. The correlation coefficient obtained by matching the CI3D with the average subjective severity shows that the proposed method outperforms traditional approaches. CONCLUSIONS: The optical 3D index has a good match with the average subjective assessment in distinguishing patients with mild to severe PE. This innovative approach offers several advantages over existing indices, as it is repeatable and does not require cross-sectional imaging. The index might be particularly suitable for monitoring the efficacy of nonoperative treatment and, in the future, for designing an optimal personalized usage of therapeutic devices.

Regenerative Surgery in the Treatment of Cosmetic Defect Following Nuss Procedure.

BACKGROUND: In the past decade, the minimally invasive repair of pectus excavatum (MIRPE) has become the treatment of choice for severe pectus excavatum (PE), proving acceptable to excellent cosmetic results. Recently, autologous fat grafting (FG) has been identified in aesthetic and reconstructive surgery to ideally handle volume and contour defects. We report our experience about FG in the treatment of residual minor defects after MIRPE and a proposal for a new indication of this largely adopted technique. MATERIALS AND METHODS: From April 2012 to April 2015, 127 patients underwent bar removal. At outpatient check, a questionnaire was adopted to investigate aesthetic outcome prior and after surgery (3 months postoperation); scoring options varied from 4 to 0 [4, excellent; 3, very good; 2, good; 1, acceptable; and 0, unacceptable]. In patients scoring less than "good," we proposed FG at the same operating session of bar removal. The donor areas were the abdomen, trochanteric region, and inner thigh. The tissue was gently collected through a thin cannula, filtrated, and then promptly injected into the defect. RESULTS: Eleven patients (8.8%) have been selected for FG. No complications have been detected during the follow-up period (range 1-36 months). In three cases, we performed further FG procedure to achieve an optimal result. Questionnaire reported an increased mean score from 1.8 to 2.7. CONCLUSION: In our early experience, FG has provided satisfactory cosmetic results. With the present report we advocate FG as a minimally invasive "tool" to achieve better defect correction after MIRPE. These preliminary results suggest that FG could be a worthwhile resource in treating chest wall malformations.

Sternal Cleft and Pectus Excavatum: A Combined Approach for the Correction of a Complex Anterior Chest Wall Malformation in a Teenager.

Congenital sternal cleft is a rare chest wall malformation. Because of the flexibility of the chest in infants, surgical repair should be performed by primary closure in the neonatal period. In adolescents and adults, different techniques have been suggested to overcome the lack of sternal bone tissue. We describe a very rare case of an 18-year-old woman with a complete bifid sternum associated with pectus excavatum for whom a satisfactory cosmetic and functional result was obtained by adequate surgical planning, which entailed a combination of two standardized surgical techniques.

A simplified method to pass the bar through the mediastinum in the Nuss technique.

In the minimally invasive repair of pectus excavatum, as reported by Nuss, the introducer is inserted into the thoracic cavity, making its way through the mediastinum, and emerges through a left intercostal space. Then, 2 umbilical tapes are tied to the introducer tip as a guide to pass into this tunnel the curved bar with the concave side up. When fat tissue is present in the anterior mediastinum or bars with notched ends are used, passage of the umbilical tape could be challenging and eventually lead to bleeding. In this report, we describe a different and simple technique to allow this passage, from left to right, in a very safe and effective way.

Is a shorter bar an effective solution to avoid bar dislocation in a Nuss procedure?

BACKGROUND: A variety of expedients to minimize bar dislocation in the Nuss procedure has been reported. The aims of this study were to create a mathematical model to define mechanical stresses acting on bars of different lengths in the Nuss procedure, and to apply this model to clinical scenarios. METHODS: Finite element model analyses were used to outline the mechanical stresses and to mathematically define different cases. Data from a group of patients with procedures carried out using standard Nuss criteria (NC group; bars half an inch shorter than the distance between the mid-axillary lines) were compared with data from a second group treated by applying model-based suggestions (MS group; bars approximately 3 inches shorter than the distance between the mid-axillary lines). RESULTS: Mean patient age in the NC group (48 cases) was 16.4 years old (84% males). The mean operating time was 57 minutes, and the mean bar length was 14.19 inches. There were 5 cases (10.4%) of bar dislocation. Mean patient age in the MS group (88 cases) was 16.2 years old (87% males). The mean operating time was 43 minutes and the mean bar length was 11.67 inches. There was only 1 bar dislocation, a reduction from 10.4% (NC) to 1.1% (MS) odds ratio 0.0989 (confidence interval 0.0112 to 0.8727), p = 0.0373. CONCLUSIONS: A shorter Nuss bar reduces tension on the sutures applied at bar extremities. This leads to enhanced bar stability and a reduced risk that the bar will flip. The use of a shorter Nuss bar may reduce the incidence of bar dislocation.

Adnexal torsion in children and adolescents: new trends to conservative surgical approach -- our experience and review of literature.

The purpose of this study is to discuss the surgical treatment for ovarian torsion in children and adolescents with a focus on the procedures of adnexal conservation surgery and its frequency in the literature of the last 10 years. We retrospectively reviewed the medical charts of 127 operative ovarian lesions including 30 ovarian torsions (23.6%) treated in two pediatric centers over a 10-year period. Age at presentation, presenting symptoms, diagnostic studies, surgical procedure and pathological findings were analyzed. Mean age was 13.7 years. Conservative surgery has been performed in 46.7% of the cases and laparoscopic approach in 40%. Ovarian torsion occurred in 56.7% on ovaries with functional lesion, in 23.3% on normal adnexa and in 20% on ovaries with benign neoplasm. The article includes a literature review (2000-2010) and a statistical analysis which shows a slow increase in conservative surgery from 28 to 45%. Laparoscopic surgery accounts for 23.5%. Literature review shows 40.5% normal adnexa, 33.2% non-neoplastic lesions, 25.3% benign neoplasms and 1% malignant neoplasms. The surgical treatment of children and adolescents presenting adnexal torsion should be practiced as an emergency and it should be more conservative as possible in order to maximize the future reproductive potential.

Chest fast MRI: an imaging alternative on pre-operative evaluation of Pectus Excavatum.

BACKGROUND: Standard imaging methods in evaluating chest wall deformities, such as Pectus Excavatum (PE) in paediatric and adolescent patients, include baseline 2-view chest radiography and chest CT scan. Only few studies to date investigated the value of fast MRIin the pre operative assessment of patient affected by PE. OBJECTIVE: To evaluate the efficacy of chest fast MRI in pre-operative management of patient affected by PE. To obtain the Haller Index (HI) and Asymmetry Index (AI) from chest fast MRI protecting patients from radiation exposure. MATERIALS AND METHODS: We analyzed the data of 42 consecutive patients with severe PE who underwent minimally invasive repair between March 2007 and March 2010. All 42 patients received chest fast MRI, but only the first 5 in view of the results, were studied also with chest ultrafast CT scan. In both examinations, data at the deepest point of the depression were collected. RESULTS: Severity indices of the deformity using HI and AI, collected from CT scan and fast MRI in the first 5 patients, were comparable. In the remaining 37 fast chest MRI offered good images of the chest wall deformities with no radiation exposure, detailing anatomical information such as displacement and rotation of the heart or great vessels anomalies. CONCLUSION: This study suggests the use of chest MRI in pre operative workup for patients with PE to obtain severity indices (Haller Index and Asymmetry Index avoiding radiation exposure to paediatric patients.

The role of testicular volume in adolescents with varicocele: the better way and time of surgical treatment.

PURPOSE: We report varicocele prevalence in adolescents. Surgical treatment has been proposed in adolescents with relevant testicular disproportion to avoid fertility problems in adulthood. We prospectively analyzed the testicular volume variation in adolescents with varicocele and hypoplastic testis. MATERIALS AND METHODS: In a 2-year period we selected 54 consecutive pediatric patients with a median age of 14.5 years (range 13 to 16) who had left varicocele using certain criteria, including testicular volume discrepancy greater than 20%, no previous inguinal-testicular surgery and no symptoms. Adolescents were divided into 2 groups, including 27 who underwent surgical correction with lymphatic sparing microsurgical varicocelectomy (intervention) and 27 who were only observed (control). After surgery or at first observation patients were evaluated clinically and by ultrasound at 3, 6 and 12 months. Testicular volume was estimated by the prolate ellipsoid formula. RESULTS: We noted significant improvement in testicular volume with less than 20% disparity between the 2 gonads in 23 patients (85.2%) in the intervention group and in 8 controls (29.6%). Two recurrences (7.4%) were reported in the intervention group, each in an adolescent with increased testicular volume. CONCLUSIONS: Our study confirms significantly increased testicular volume in many surgically treated boys and shows that physiological catch-up growth occurs in adolescents with varicocele without treatment. Considering critically results in each group, in select cases clinical and ultrasound followup is indicated before intervention due to a possible spontaneous decrease in testicular asymmetry. Further histopathological studies are needed to identify the relationship between testicular hypoplasia, irreversible damage and future fertility problems to determine which adolescents should be treated.

Multidisciplinary surgical approach to a surviving infant with sirenomelia.

Sirenomelia is an extremely complex and rare malformation with different degrees of lower-extremities fusion associated with gastrointestinal, musculoskeletal, vascular, cardiopulmonary, and central nervous system malformations. In the English literature, there are only 5 reports of infants surviving with this condition. In our case, a 2540-g female infant was born with normal vital signs, no facial dysmorphism, and a complete soft tissue fusion of the lower limbs, from perineum to ankles. Radiologic examinations revealed an intestinal atresia and a single pelvic kidney, with a unique ureter, 2 femurs, 2 tibias, 2 fibulas, and 2 feet (simpus dipus). At 7 months of age, a multidisciplinary surgical team achieved complete separation of the lower limbs, with independent vascular and nerve supplies. At the time of this writing, the infant was 28 months old and had a regular growth curve. Many future reconstructive surgeries have been planned to achieve an acceptable quality of life for this infant.

Total oesophagogastric dissociation: experience in 2 centres.

BACKGROUND/PURPOSE: Neurologically impaired (NI) children have an increased incidence of gastroesophageal reflux and many will require surgery. METHODS: The case notes of 50 NI children who underwent total oesophagogastric dissociation (TOGD) were reviewed. Thirty-four were done as a primary procedure, and 16 were rescues for failed fundoplications. RESULTS: There was no operative mortality. Morbidity consisted of 1 subphrenic collection, 1 oesophagojejunal dehiscence and 2 stenoses that responded to dilatation, and 2 bowel obstructions. In 1 case, partial gastric resection was needed because of transhiatal herniation of stomach. Gastrostomy feeding was established by 3 to 5 days. The mean hospital stay was 10.9 days. At 4 months to 11 years of follow-up, there was no recurrence of reflux. Children who could swallow enjoyed oral feeds. Their general health and weight SD scores improved. Food aspiration, chest infections, and hospitalizations were reduced, with an improvement in quality of life. There were 5 late deaths in the "primary" and 7 in the "rescue" group from deterioration in their original condition. CONCLUSION: Total oesophagogastric dissociation is a safe and versatile procedure without immediate mortality and limited surgery-related morbidity. Review of our practice suggests TOGD should be considered as a primary procedure in severely NI children with gastroesophageal reflux and significant oropharyngeal incoordination and dependence on enteral tube feeding. Rescue TOGD carries a greater morbidity because of previous surgery with consequent difficult dissection, poor oesophageal tissue, and higher incidence of vagal nerve injury.

Fatal aortoesophageal fistula in two cases of tight vascular ring.

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.