Robotic-assisted versus conventional laparoscopic ICG-fluorescence lymphatic-sparing palomo varicocelectomy: a comparative retrospective study of techniques and outcomes.

PURPOSE: This study aimed to compare techniques and outcomes of robotic-assisted varicocelectomy (RAV) and laparoscopic varicocelectomy (LV). METHODS: The medical records of 40 patients, who received RAV and LV over a 2-year period, were retrospectively analyzed. Palomo lymphatic-sparing varicocelectomy using ICG fluorescence was adopted in all cases. Three 5-mm trocars were placed in LV, whereas four ports, three 8-mm and one 5-mm, were placed in RAV. The spermatic vessels were ligated using clips in LV and ligatures in RAV. The two groups were compared regarding patient baseline and operative outcomes. RESULTS: All patients, with median age of 14 years (range 11-17), had left grade 3 varicocele according to Dubin-Amelar. All were symptomatic and 33/40 (82.5%) presented left testicular hypotrophy. All procedures were completed without conversion. The average operative time was significantly shorter in LV [20 min (range 11-30)] than in RAV [34.5 min (range 30-46)] (p = 0.001). No significant differences regarding analgesic requirement and hospitalization were observed (p = 0.55). At long-term follow-up (30 months), no complications occurred in both groups. The cosmetic outcome was significantly better in LV than RAV at 6-month and 12-month evaluations (p = 0.001). The total cost was significantly lower in LV (1.587,07 €) compared to RAV (5.650,31 €) (p = 0.001). CONCLUSION: RAV can be safely and effectively performed in pediatric patients, with the same excellent outcomes as conventional laparoscopic procedure. Laparoscopy has the advantages of faster surgery, smaller instruments, better cosmesis and lower cost than robotics. To date, laparoscopy remains preferable to robotics to treat pediatric varicocele.

Use of combined cerebral and somatic renal near infrared spectroscopy during noncardiac surgery in children: a proposed algorithm.

Cerebral near infrared spectroscopy (NIRS) monitoring has been extensively applied in neonatology and in cardiac surgery, becoming a standard in many pediatric cardiac centers. However, compensatory physiological mechanisms favor cerebral perfusion to the detriment of peripheral tissue oxygenation. Therefore, simultaneous measurement of cerebral and somatic oxygen saturation has been advocated to ease the differential diagnosis between central and peripheral sources of hypoperfusion, which may go undetected by standard monitoring and not mirrored by cerebral NIRS alone. A clinical algorithm already exists in cardiac surgery, aimed to correct intraoperative cerebral oxygen desaturations. A similar algorithm still lacks in noncardiac pediatric surgery. The goal of this paper is to propose a clinical algorithm for the combined use of cerebral and somatic NIRS monitoring during anesthesia in the pediatric population undergoing noncardiac surgery. A panel of experienced pediatric anesthetists developed the algorithm that is based on the clinical experience and intraoperative observations. It aims to lessen the current variability in interpreting NIRS measurement. Multisite NIRS monitoring was achieved applying one pediatric sensor to the forehead for cerebral tissue perfusion reading and a second one to the decumbent lumbar region for recording somatic renal tissue perfusion. The algorithm describes a sequence of acts aimed to identify the putative cause of intraoperative organ tissue desaturation and suggests clinical interventions expected to restore adequate tissue perfusion. It is composed of two arms: the main arm includes patients with an observed decrease in cerebral perfusion (CrO2), the second one includes those with a stable CrSO2 with declining RrSO2. Described also are five clinical cases of infants and neonates in whom pathological alterations of organ perfusion were detected using intraoperative multisite NIRS monitoring, portrayed in the accompanying figures (Annex).

Minimally invasive fetal surgery for myelomeningocele: preliminary report from a single center.

OBJECTIVE: Recent trials have shown the safety and benefits of fetoscopic treatment of myelomeningocele (MMC). The authors' aim was to report their preliminary results of prenatal fetoscopic treatment of MMC using a biocellulose patch, focusing on neurological outcomes, fetal and maternal complications, neonatal CSF leakage, postnatal hydrocephalus, and radiological outcomes. METHODS: Preoperative assessment included clinical examination, ultrasound imaging, and MRI of the fetus. Patients underwent purely fetoscopic in utero MMC repair, followed by postoperative in utero and postnatal MRI. All participants received multidisciplinary follow-up. RESULTS: Five pregnant women carrying fetuses affected by MMC signed informed consent for the fetoscopic treatment of the defect. The mean MMC size was 30.4 mm (range 19-49 mm). Defect locations were L1 (2 cases), L5 (2 cases), and L4 (1 case). Hindbrain herniation and ventriculomegaly were documented in all cases. The mean gestational age at surgery was 28.2 weeks (range 27.8-28.8 weeks). Fetoscopic repair was performed in all cases. The mean gestational age at delivery was 33.9 weeks (range 29.3-37.4 weeks). After surgery, reversal of hindbrain herniation was documented in all cases. Three newborns developed signs of hydrocephalus requiring CSF diversion. Neurological outcomes in terms of motor level were favorable in all cases, but a premature newborn died due to CSF infection and sepsis. CONCLUSIONS: The authors' preliminary results suggest that fetoscopic treatment of MMC is feasible, reproducible, and safe for mothers and their babies. Neurological outcomes were favorable and similar to those in the available literature. As known, prematurity was the greatest complication.

Tracheal Diameter and Respiratory Outcome in Infants with Congenital Diaphragmatic Hernia Treated by Fetal Endoscopic Tracheal Occlusion.

AIM: To evaluate tracheal diameters and their clinical impact in patients with congenital diaphragmatic hernia (CDH) after fetal endoscopic tracheal occlusion (FETO). METHODS: Patients born with CDH between January 2012 and August 2016 were divided into two groups: noFETO and FETO. Tracheal diameters at three levels (T1, carina, and maximum tracheal dilation) on chest X-ray at 1, 3, 6, 12, 24, and 36 months of follow-up, requirements of invasive and noninvasive respiratory support, the incidence of respiratory infections, and results of pulmonary function tests (PFT) were compared. RESULTS: A total of 71 patients with CDH were born in the study period, and there were 34/41 survivors in the no-FETO group (82.9%) and 13/30 in the FETO group (43.3%). The maximum tracheal diameter was significantly greater in the FETO group at all ages. No differences were observed in the diameters at T1 and the carina, in the requirements of invasive and noninvasive respiratory support, and in the incidence respiratory infections. At the PFT (6-12 months), the FETO group presented higher respiratory rates (46.1 ± 6.2 vs. 36.5 ± 10.6, p = 0.02). No differences in PFT results were found between the groups after the 1st year of life. CONCLUSIONS: The FETO procedure leads to persistent tracheomegaly. However, the tracheomegaly does not seem to have a significant clinical impact.

International Study of the Epidemiology of Paediatric Trauma: PAPSA Research Study.

OBJECTIVES: Trauma is a significant cause of morbidity and mortality worldwide. The literature on paediatric trauma epidemiology in low- and middle-income countries (LMICs) is limited. This study aims to gather epidemiological data on paediatric trauma. METHODS: This is a multicentre prospective cohort study of paediatric trauma admissions, over 1 month, from 15 paediatric surgery centres in 11 countries. Epidemiology, mechanism of injury, injuries sustained, management, morbidity and mortality data were recorded. Statistical analysis compared LMICs and high-income countries (HICs). RESULTS: There were 1377 paediatric trauma admissions over 31 days; 1295 admissions across ten LMIC centres and 84 admissions across five HIC centres. Median number of admissions per centre was 15 in HICs and 43 in LMICs. Mean age was 7 years, and 62% were boys. Common mechanisms included road traffic accidents (41%), falls (41%) and interpersonal violence (11%). Frequent injuries were lacerations, fractures, head injuries and burns. Intra-abdominal and intra-thoracic injuries accounted for 3 and 2% of injuries. The mechanisms and injuries sustained differed significantly between HICs and LMICs. Median length of stay was 1 day and 19% required an operative intervention; this did not differ significantly between HICs and LMICs. No mortality and morbidity was reported from HICs. In LMICs, in-hospital morbidity was 4.0% and mortality was 0.8%. CONCLUSION: The spectrum of paediatric trauma varies significantly, with different injury mechanisms and patterns in LMICs. Healthcare structure, access to paediatric surgery and trauma prevention strategies may account for these differences. Trauma registries are needed in LMICs for future research and to inform local policy.

Prenatal and accurate perinatal diagnosis of type 2 H or ductular duplicate gallbladder.

BACKGROUND: Double gallbladder is a rare biliary anomaly. Perinatal diagnosis of the disorder has been reported in only 6 cases, and in 5 of them the diagnosis was based on ultrasound imaging only. However, the ultrasound technique alone does not provide a sufficiently precise description of cystic ducts and biliary anatomy, an information that is crucial for a correct classification and for a possible future surgery. CASE PRESENTATION: At 21 weeks of gestational age of an uneventful pregnancy in a 38 year old primipara mother, a routine ultrasound screening detected a biliary anomaly in the fetus suggestive of a double gallbladder. A neonatal abdominal ultrasonography performed on postnatal day 2 confirmed the diagnosis. On day 12 the newborn underwent a Magnetic Resonance Cholangiopancreatography (MRCP) that clearly characterized the anatomy of the anomaly: both gallbladders had their own cystic duct and both had a separate insertion in the main biliary duct. CONCLUSIONS: We report a case of early prenatal suspected duplicate gallbladder that was confirmed by a neonatal precise diagnosis of a Type 2, H or ductular duplicate gallbladder, using for the first time 3D images of Magnetic resonance cholangiopancreatography in a newborn. An accurate anatomical diagnosis is mandatory in patients undergoing a possible future cholecystectomy, to avoid surgical complications or reoperations. Therefore, in case of a perinatal suspicion of a double gallbladder, neonates should undergo a Magnetic resonance cholangiopancreatography. A review of the Literature about this variant is included.

Sequence variants identification at the KCNQ1OT1:TSS differentially Methylated region in isolated omphalocele cases.

BACKGROUND: Omphalocele is a congenital midline ventral body wall defect that can exist as isolated malformation or as part of a syndrome. It can be considered one of the major and most frequent clinical manifestation of Beckwith-Wiedemann Syndrome (BWS) in case of loss of methylation at KCNQ1OT1: Transcription Star Site-Differentially Methylated Region (TSS-DMR) or in presence of CDKN1C mutations. The isolated form of the omphalocele accounts approximately for about the 14% of the total cases and its molecular etiology has never been fully elucidated. METHODS: Given the tight relationship with BWS, we hypothesized that the isolated form of the omphalocele could belong to the heterogeneous spectrum of the BWS associated features, representing an endophenotype with a clear genetic connection. We therefore investigated genetic and epigenetic changes affecting BWS imprinted locus at 11p15.5 imprinted region, focusing in particular on the KCNQ1OT1:TSS DMR. RESULTS: We studied 21 cases of isolated omphalocele detected during pregnancy or at birth and identified the following rare maternally inherited variants: i) the non-coding variant G > A at nucleotide 687 (NR_002728.3) at KCNQ1OT1:TSS-DMR, which alters the methylation pattern of the imprinted allele, in one patient; ii) the deletion c.624-629delGGCCCC at exon 1 of CDKN1C, with unknown clinical significance, in two unrelated cases. CONCLUSIONS: Taken together, these findings suggest that KCNQ1OT1:TSS-DMR could be a susceptibility locus for the isolated omphalocele.

Comparative retrospective study on the modalities of biopsying peripheral neuroblastic tumors: a report from the Italian Pediatric Surgical Oncology Group (GICOP).

BACKGROUND: Peripheral neuroblastic tumors are the most common extracranial solid neoplasms in children. Early and adequate tissue sampling may speed up the diagnostic process and ensure a prompt start of optimal treatment whenever needed. Different biopsy techniques have been described. The purpose of this multi-center study is to evaluate the accuracy and safety of the various examined techniques and to determine whether a preferential procedure exists. METHODS: All children who underwent a biopsy, from January 2010 to December 2014, as a result of being diagnosed with a peripheral neuroblastic tumor, were retrospectively reviewed. Data collected included patients' demographics, clinical presentation, intraoperative technical details, postoperative parameters, complications, and histology reports. The Mann-Whitney U and Fisher's exact tests were used for statistical analysis. RESULTS: The cohort included 100 patients, 32 of whom underwent an incisional biopsy (performed through open or minimally invasive access) (Group A), and the remaining 68 underwent multiple needle-core biopsies (either imaging-guided or laparoscopy/thoracoscopy-assisted) (Group B). Comparing the two groups revealed that Group A patients had a higher rate of complications, a greater need for postoperative analgesia, and required red blood cell transfusion more often. Overall adequacy rate was 94%, without significant differences between the two groups (100% vs. 91.2% for Group A and Group B, respectively, P = 0.0933). CONCLUSIONS: Both incision and needle-core biopsying methods provided sub-optimal to optimal sampling adequacy rates in children affected by peripheral neuroblastic tumors. However, the former method was associated with a higher risk of both intraoperative and postoperative complications compared with the latter.

Fetoscopic Endoluminal Tracheal Occlusion in Fetuses with Severe Diaphragmatic Hernia: A Three-Year Single-Center Experience.

OBJECTIVE: To report on our experience in the prenatal treatment of severe congenital diaphragmatic hernia (CDH) by fetoscopic endoluminal tracheal occlusion (FETO). METHODS: Between 2012 and 2014, FETO was performed at our center in 21 cases of CDH considered to be severe based on sonographic measurement of observed/expected lung-to-head ratio (O/E LHR) and side of the defect. We reported pre- and postoperative ultrasound findings, procedure-related complications, pregnancy outcome and survival at 1-3 years of age. RESULTS: The median gestational age (GA) at balloon insertion was 28.1 weeks (range 26.0-31.1) and the median GA at delivery 34.7 weeks (range 31.6-39.0); delivery before 32 and 34 weeks occurred in 2 (9.5%) and 7 (33.3%) cases, respectively. Postnatal survival at 1-3 years of age in the 17 cases with isolated unilateral CDH was 47.1%. The percentage difference between pre-balloon removal O/E LHR and pre-FETO O/E LHR was significantly higher in survivors compared to neonates who died (40.8 vs. 21.2%, respectively; p < 0.05). CONCLUSIONS: In this study, FETO was associated with an infant survival of 47% in cases with isolated unilateral severe CDH. The post-FETO increase in O/E LHR was higher in fetuses that survived compared to those who died.

2q33.1q34 Deletion in a Girl with Brain Anomalies and Anorectal Malformation.

2q33 deletions are considered to constitute a distinct clinical entity (Glass syndrome or 2q33 microdeletion syndrome) with a characteristic phenotype. Most patients have moderate to severe developmental delay, speech delay, a particular behavioural phenotype, feeding problems, growth restriction, a typical facial appearance, thin and sparse hair, tooth abnormalities, and skeletal anomalies. Here, we report on a patient with a 2q33.1q34 deletion spanning 8.3 Mb of genomic DNA. Although her clinical features are very reminiscent of the 2q33 microdeletion syndrome, she also presented with brain and anorectal malformations. Based on the present and published patients with 2q33 deletions, we suggest that the critical region for the Glass syndrome may be larger than initially proposed. Moreover, we suggest that brain abnormalities might be an additional feature of the 2q33 microdeletion syndrome, but that anorectal malformation is likely not a key marker.

Treatment of Severe Hypervolemic Hyponatremia in a Child With Pneumonia.

A 21-month-old boy came to our attention because of pneumonia. His weight increased before presentation, and his blood test results showed hyponatremia (116 mEq/L), low plasma osmolarity (241 mOsm/L), and high urine osmolarity (435 mOsm/L). He was treated with 0.9% sodium chloride solution and intravenous furosemide, and sodium levels rose up to 135 mEq/L in 36 hours. No standard treatment is available for severe hyponatremia in children. The use of vaptans in pediatric patients is described in literature, but it lacks evidence about safety and effectiveness. We suggest that furosemide administration plus salt replacement is effective in restoring normal values of plasma sodium concentration in severe euvolemic and hypervolemic hyponatremia.

Survey on the management of anorectal malformations (ARM) in European pediatric surgical centers of excellence.

PURPOSE: Various management strategies for anorectal malformations (ARM) have been proposed. The aim of this study was to assess the current management in centers of excellence in Europe. METHODS: An online survey on the pre- and post-operative concepts, surgical techniques, and the management of complications was sent to the representative experts of 28 selected European centers of pediatric surgery with special expertise in the treatment of ARM. RESULTS: The survey was completed by 25 experts from 14 countries. To assess the level of the rectal pouch in newborns 60% of participants perform a prone cross-table X-ray and 52% ultrasound. If an ostomy is required, 84% create divided Peña stomas. Primary repair in the newborn period is performed in females with rectoperineal (92%), rectovestibular (60%), and no fistula (32%), and in males with rectoperineal (92%), rectourinary (17%) and no fistula (38%). For 68 % of surgeons, the PSARP is the preferred surgical approach for "low" malformations. Laparoscopically assisted pull-throughs are routinely performed by 48% of experts for ARM with bladderneck and 28% for rectoprostatic fistula. 88% perform postoperative dilations. CONCLUSIONS: The management of ARM in Europe is very heterogeneous. High-quality clinical studies are needed to provide scientific evidence for the optimal treatment strategies.

Comparison in the incidence of anorectal malformations between a first- and third-world referral center.

PURPOSE: Aim of study was to evaluate the differences in incidence and presentation of anorectal malformations (ARMs) between selected Pediatric Surgery Divisions in the Republic of South Africa (ZAR) and Italy. METHODS: A retrospective cohort study involved analysis of clinical records of patients with ARM born between 2005 and 2012. Type of ARM, maternal age, birth weight, gestational age, presence of associated anomalies and delayed diagnosis were analyzed. RESULTS: 335 patients were included in this study. Of note, statistically significant differences between the African and European patient groups were observed in a male predominance in the ZAR patient population. In addition, female recto-perineal fistulas were diagnosed in significantly more Italian patients than in ZAR. Furthermore, a more advanced maternal age and a lower gestational age was noted in the European cohort with a minimal delay in initial diagnosis as opposed to the African counterpart. Both centers reported recto-perineal fistula as the most common malformation in male patients. CONCLUSION: With the exception of perineal fistulas in females, the incidence of specific subtypes of ARMs was similar in the two groups. This may be of importance when extrapolating European study conclusion to the South African setting.

Severe and isolated headache associated with hypertension as unique clinical presentation of posterior reversible encephalopathy syndrome.

BACKGROUND: Posterior reversible encephalopathy syndrome is a potentially reversible clinicoradiologic syndrome characterized by headache, mental confusion, visual disturbances and seizures associated with posterior cerebral lesions on radiological imaging. Prompt treatment of this condition is mandatory to avoid severe irreversible complications. CASE PRESENTATION: We report a 9-year-old boy with arterial hypertension and headache as unique clinical presentation of posterior reversible encephalopathy syndrome. CONCLUSIONS: Severe and isolated headache associated with arterial hypertension can be the unique clinical presentation of posterior reversible encephalopathy syndrome. This syndrome must be considered even in absence of all typical symptoms to prevent the progression of a potentially life threatening condition.

Classification and management of rectal prolapse after anorectoplasty for anorectal malformations.

PURPOSE: To suggest a classification, describe the risk factors and management of rectal prolapse after anorectoplasty for anorectal malformations (ARMs). METHODS: We classified prolapse as minimal (rectal mucosa visible with Valsalva manoeuvre), moderate (prolapse <5 mm without Valsalva), evident (>5 mm without Valsalva) and compared patients with and without prolapse within our ARM-population. RESULTS: Among 150 patients, 40 (27 %) developed prolapse: 25 minimal, 6 moderate, 9 evident. Prolapse affected 33 % of males (9 % of perineal fistulas, 38 % of bulbar, 71 % of prostatic, 60 % of bladder neck and 13 % without fistula) and 21 % of females (9 % of perineal, 30 % of vestibular, 50 % of cloacas, and 25 % without fistula). Risk factors for prolapse were: tethered cord (40 vs 24 %), vertebral anomalies (39 vs 24 %), laparoscopic-assisted anorectoplasty (LAARP) (75 vs 25 %), and colostomy at birth (49 vs 9 %). Redo anorectoplasty was not associated with prolapse. Symptoms were present in 11 patients (28 %): in 7 % with minimal, 33 % with moderate and 77 % with evident prolapse. Nine patients (2 moderate, 7 evident) underwent surgical correction. CONCLUSION: Severe ARMs, tethered cord, vertebral anomalies, colostomy, and LAARP predispose to rectal prolapse. Classifying prolapse allows to predict symptoms and need for surgical correction, and to compare outcomes among different centers.

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Anastomotic strictures and endoscopic dilatations following esophageal atresia repair.

PURPOSE: To identify risk factors that can predict prevalence of anastomotic strictures (AS) following esophageal atresia (EA) repair. METHODS: Of 46 consecutive patients with EA managed at our institution between 2004 and 2012, 35 underwent esophageal anastomosis and were included in this retrospective longitudinal study. Routine endoscopy was performed 1 month after surgical repair. According to stricture index (SI), endoscopically calculated as SI = (D - d)/D, where D is the diameter of the esophageal pouch and d the stricture diameter, population was divided into Group 1, SI ≤ 0.1 (no evidence of stricture); Group 2, 0.3 > SI > 0.1 (mild stricture); Group 3, SI ≥ 0.3 (high-grade stricture). Trends of subsequent endoscopic esophageal dilatations were compared between the groups using Wilcoxon-Mann-Whitney or Pearson's tests. Cox regression analysis was performed to estimate the hazard ratio. RESULTS: Gastro-esophageal reflux disease (P = 0.04), tension on the anastomosis (P = 0.02) and long-gap form (P = 0.008) have an increased risk of developing AS. SI at 1 month after surgery correlates with the average number of future dilatations: Group 2 and 3 compared to Group 1 required more dilatations (hazard ratio 2.291 and 12.765). CONCLUSION: AS remain frequent complications of esophageal surgery, especially in specific subgroups of patients. SI at 1 month after surgery could already predict the severity of the stricture and the need for subsequent endoscopic esophageal dilatations.

Combined Pre- and Postnatal Minimally Invasive Approach to a Complex Symptomatic Congenital Pulmonary Airway Malformation.

Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that usually remains asymptomatic during the fetal and neonatal period. However, it can occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms at birth (such as respiratory distress) and may require urgent surgical intervention. Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of gestation, a male neonate was born (weight 2,850 g). The TAS spontaneously displaced during delivery, causing an open pneumothorax (PNX), initially treated with a drainage. His condition gradually worsened, requiring ventilatory support. Computed tomography (CT) scan showed different giant cysts in the context of the right lower lobe, left mediastinal shift, and compression of the rest of the lung. An urgent surgical management was required. A thoracoscopic right lower lobectomy was performed at 10 days of life (weight 2,840 g). The postoperative course was uneventful; the child remained totally asymptomatic and showed a good recovery. To the best of our knowledge, this is the first reported case of open iatrogenic PNX following TAS positioning and the second of neonatal thoracoscopic lobectomy in a newborn weighting less than 3 kg. The purpose of this report is to indicate that minimally invasive surgery is feasible, safe, and effective for the resection of CPAM, even in small newborns.

Congenital Lung Malformations: Pulmonary Function Assessment Before and After Surgery.

BACKGROUND: The management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact. METHODS: A retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate). Results were assessed as z-score (normal ±1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. RESULTS: Thirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: -0.32 (-2.12; +1.56); post: -0.18 (-1.62; +2.43)). Patients with extralobar sequestration had pre-operative high median RR, which improved after sequestrectomy (pre: +1.99 (-2.49; +7.43); post -0.22 (-3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: -2.15 (-9.75; +0.91); post: -0.35 (-6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (-2.27 (-6.12; +5.26)). CONCLUSIONS: Patients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. LEVEL OF EVIDENCE: III, Treatment study.