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.

Intradiaphragmatic pulmonary sequestrations: a surgical challenge. Case series.

Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.

Unexpected Pediatric Uretero-Inguinal Hernia: Case-Report and Literature Review.

Uretero-inguinal hernia (UIH) is a rare condition in children. We present our experience with an unexpected UIH in a male infant with associated anomalies and a literature review of pediatric cases. A full-term male newborn was referred to our hospital for microcolon and right hydroureteronephrosis. Voiding cystourethrography showed grade-IV right vesicoureteral reflux and a dilated, convoluted, ureter protruding in the right inguinal canal, suggesting a right UIH, which was uneventfully repaired at 2 months of life. UIH is a rare type of inguinal hernia in children that pediatric urologists and surgeons should be aware of. In experienced hands, and when preoperatively suspected, surgical management is safe and effective.

Case Report: Congenital Perineal Lipoma Associated With Additional External Genitalia Anomalies.

Perineal lipoma is an uncommon congenital benign tumor sometimes associated with genitourinary or anorectal malformations. Accessory scrotum and accessory labioscrotal fold are infrequent features, often concurrent with perineal tumors. We describe a single institution experience with three consecutive cases of perineal lipoma associated with external genital anomalies, and a literature review.

Long-term lung function in children following lobectomy for congenital lung malformation.

BACKGROUND/PURPOSE: To date, the optimal management of asymptomatic congenital lung malformations (CLMs) is still debated. There is still scant and controversial information regarding the long-term assessment of pulmonary function (PF) after lobectomy in children. The aim of this study is to evaluate PF in children who underwent lobectomy for CLM in infancy, hypothesizing that patients operated during the first year of life retain a normal lung function. METHODS: Children operated between 2005 and 2016 at our institution underwent PF evaluation through spirometry/whole-body plethysmography, forced oscillation technique, and multiple-breath inert gas wash-out. RESULTS: Out of 85 patients who underwent lobectomy at a median age of 5months, 50 met the inclusion criteria, and 28 patients were tested. More than 80% of patients had normal FEV1 and FVC. The mean FEV1, FVC, FEF25-75% values were higher in the patients operated before reaching one year of age. CONCLUSIONS: The long-term outcome after lobectomy was excellent for most patients, as they retained a normal long-term PF. Therefore, for asymptomatic patients, a surgical approach before one year of age to avoid complications such as malignancy and to ensure an optimal PF catch-up could be beneficial. LEVEL OF EVIDENCE: III - Treatment Studies.

The Role of DNA Amplification and Cultural Growth in Complicated Acute Appendicitis.

Bacterial growth of peritoneal fluid specimens obtained during surgical procedures for acute appendicitis may be useful to optimize further antibiotic therapy in complicated cases. DNA amplification represents a fast technique to detect microbial sequences. We aimed to compare the potential of DNA amplification versus traditional bacterial growth culture highlighting advantages and drawbacks in a surgical setting. Peritoneal fluid specimens were collected during surgery from 36 children who underwent appendectomy between May and December 2012. Real-time polymerase chain reaction (RT-PCR) and cultures were performed on each sample. RT-PCR showed an amplification of 16S in 18/36 samples, Escherichia coli (in 7 cases), Pseudomonas aeruginosa (3), Fusobacterium necrophorum (3), Adenovirus (2), E.coli (1), Klebsiella pneumoniae (1), Serratia marcescens/Enterobacter cloacae (1). Bacterial growth was instead observed only in four patients (3 E.coli and 1 P.aeruginosa and Bacteroides ovatus). Preoperative C-reactive protein and inflammation degree, the most reliable indicators of bacterial translocation, were elevated as expected. DNA amplification was a quick and useful method to detect pathogens and it was even more valuable in detecting aggressive pathogens such as anaerobes, difficult to preserve in biological cultures; its drawbacks were the lack of biological growths and of antibiograms. In our pilot study RT-PCR and cultures did not influence the way patients were treated.

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.

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.

Pectus excavatum and heritable disorders of the connective tissue.

Pectus excavatum, the most frequent congenital chest wall deformity, may be rarely observed as a sole deformity or as a sign of an underlying connective tissue disorder. To date, only few studies have described correlations between this deformity and heritable connective tissue disorders such as Marfan, Ehlers-Danlos, Poland, MASS (Mitral valve prolapse, not progressive Aortic enlargement, Skeletal and Skin alterations) phenotype among others. When concurring with connective tissue disorder, cardiopulmonary and vascular involvement may be associated to the thoracic defect. Ruling out the concomitance of pectus excavatum and connective tissue disorders, therefore, may have a direct implication both on surgical outcome and long term prognosis. In this review we focused on biological bases of connective tissue disorders which may be relevant to the pathogenesis of pectus excavatum, portraying surgical and clinical implication of their concurrence.

Rib cartilage characterization in patients affected by pectus excavatum.

Pectus excavatum (PE) is the most frequent anterior chest deformity which may be frequently associated with connective tissue disorders. We performed microscopic analyses to better understand cartilage behavior and obtain clues on its pathogenesis. In 37 PE patients, none with Marfan syndrome, we analyzed costal cartilage by light microscopy, immunohistochemistry and transmission electron microscopy. Control tissue specimens were harvested from four patients without any connective tissue disease. In both control and PE patients, chondrocytes were on the average <15 µm in diameter and occupied <10% of tissue volume; in most cases the extracellular matrix was stained by alcian blue, instead of safranin; no difference between PE and control samples was significant. All samples showed an uneven collagen type II immunolabeling both within the cells and pericellular matrix, and occasionally of the territorial matrix. In all cases numerous cells underwent apoptosis accompanied by matrix condensation as shown by electron microscopy. Our results suggest that matrix composition and the cell number and size of costal cartilage are dependent on the subject and not on the disease; the microscopic organization of cartilage is correlated with the stabilization of the defective shape rather than with the onset of the deformity.

Pectus excavatum and MASS phenotype: an unknown association.

INTRODUCTION: Severe pectus excavatum (PE) is a deep chest wall deformity that generates both a cosmetic damage and a cardiac/respiratory function impairment. Excluding the scarce reports on Marfan's syndrome (MFS) and Ehlers-Danlos's syndrome (EDS), few studies have examined the relation between severe PE and connective tissue disorders. The aim of this study is to verify the clinical significance of such correlation. SUBJECTS AND METHODS: Ninety-two consecutive patients, of whom 79 were males, between 6 and 34 years old, classified as having severe PE, were seen at our institution from June 2005 to September 2010. All patients underwent clinical, ophthalmological, cardiac, and radiological (chest and spine magnetic resonance imaging) screening. The following features were observed: skin stretch marks, scoliosis, joint hypermobility, echocardiographic signs, spinal defects, and myopia. RESULTS: Classical connectivopathies such as MFS or EDS were present in only 5 patients (approximately 5%), whereas a single deformity was present in 4. The largest group (approximately 71%) was represented by phenotypical alterations such as mitral valve prolapse, aortic root enlargement, and skeletal and skin alterations (MASS). Among those patients, the most frequent clinical manifestations were the skeletal ones, followed by skin marks and mitral valve prolapse. CONCLUSIONS: PE showed an evident association with an array of features that we describe as MASS. Although not one of this subgroup of patients has been described with increased aortic root diameter when screened (a feature widely present in MFS patients), they probably would require a thorough and longer follow-up than those affected by isolated PE because of the potential occurrence of severe cardiovascular complications such as aneurysms and dissection, which are major causes of morbidity and mortality in MFS.