Left extralobar pulmonary sequestration and a right aorto-topulmonary vein fistula in a newborn : a 3-mm thoracoscopic monolateral approach

An extralobar pulmonary sequestration (EPS) associated with a contralateral aorto-to-pulmonary vein fistula is rare. We report the case of a female newborn with left EPS fed by an artery originating from the distal thoracic aorta and, symmetrically on the controlateral side, an artery shunting in the inferior right pulmonary vein. Echocardiography showed dilatation of the left atrium. On the 34th day since birth (weight 4500 g), the patient was operated on thoracoscopically. The EPS was closed with a 3-mm sealing system, divided and removed. A window in the mediastinal pleura was created, and the origin of the fistula was identified and sealed. The postoperative course was uneventful. The patient was discharged on Day 4 with no echocardiographic signs of persistence of the fistula and of the congestive heart failure. This is the first case report of a thoracic large systemic circulation-to-pulmonary vein fistula causing heart failure associated with EPS. The thoracoscopic monolateral approach and the availability of 3-mm instruments guaranteed a maximum level of minimal invasiveness

Possible Approach to Esophageal Lung with Long Tracheobronchial Gap

Esophageal lung is a rare bronchopulmonary foregut malformation characterized by an anomalous origin of one of the main bronchi which arises from the esophagus. Less than 30 cases are reported in the literature. Therefore, there are no standardized guidelines for the treatment of this condition. We report a case of right esophageal lung diagnosed in a neonate. The patient was treated with thoracoscopic closure of the ectopic main bronchus in the neonatal period, followed by delayed pneumonectomy at 5 months of age. No prosthetic substitute was implanted in the ipsilateral hemithorax after pneumonectomy. The patient is now 4 years old and doing well, postpneumonectomy syndrome was never observed. Our strategy and the possible alternatives are discussed here

duodenal hematoma and pancreatitis complicating endoscopic intestinal biopsy in a boy with noonan syndrome

Duodenal intramural hematoma is a rare condition, mostly described in children and young adults that can be a complication of duodenal biopsy, especially in patients with predisposing hemorrhagic diathesis. It can determine secondary pancreatitis because of ampullary hematoma. Noonan Syndrome (NS) is an autosomal dominant disorder characterized by short stature, typical facial dysmorphisms, congenital heart defects and other anomalies such as bleeding problems which have been reported in up to 55% of patients. We herein report a case of duodenal hematoma with pancreatitis developed after endoscopic biopsy in a boy who was initially suspected of having celiac disease on the base of his short stature and growth retardation. Afterwards a more careful past medical history collection and objective examination revealed characteristic features of NS which could have been picked-up in advance, thus avoiding an investigation, such as the duodenal endoscopic biopsy, which in NS patient is potentially more risky

Respiratory morbidity in children with repaired congenital esophageal atresia with or without tracheoesophageal fistula

Congenital esophageal atresia with or without tracheoesophageal fistula (CEA \ub1 TEF) is a relatively common malformation that occurs in 1 of 2500-4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA \ub1 TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula. Long-term respiratory morbidity is grouped into four categories according to the body section or function mainly involved: upper respiratory tract, lower respiratory tract, gastrointestinal tract, and aspiration and dysphagia. The reasons for the persistence of respiratory morbidity to adulthood are not univocal. The malformation itself, the acquired damage after the surgical repair, various co-morbidities, and the recurrence of lower respiratory tract infections at an early age can contribute to pulmonary impairment. Nevertheless, other conditions, including smoking habits and, in particular, atopy can play a role in the recurrence of infections. In conclusion, our manuscript shows that most children born with CEA \ub1 TEF survive into adulthood, but many comorbidities, mainly esophageal and respiratory issues, may persist. The pulmonary impairment involves many underlying mechanisms, which begin in the first years of life. Therefore, early detection and management of pulmonary morbidity may be important to prevent impairment in pulmonary function and serious long-term complications. To obtain a successful outcome, it is fundamental to ensure a standardized follow-up that must continue until adulthood

Successful extracorporeal membrane oxygenation after incidental azygos vein cannulation in a neonate with right-sided congenital diaphragmatic hernia interruption of the inferior vena cava and azygos continuation

Incidental azygos vein cannulation has been reported in a few cases of neonatal extracorporeal membrane oxygenation (ECMO). This complication is described in the literature mainly in pathological conditions wherein increased central venous pressure dilates the superior vena cava (SVC), i.e., right congenital diaphragmatic hernia (CDH) or pulmonary hypertension. Azygos vein cannulation should always be suspected in cases of impaired venous return and circuit failure. Although rare, it hinders proper venous aspiration of the ECMO circuit and generally requires repositioning or replacement of the venous cannula or conversion to central cannulation. In this report, we describe a newborn with severe right CDH who required ECMO assistance, wherein incidental cannulation of the azygos vein resulted in successful functioning of the circuit because of the concomitant presence of isolated interruption of the inferior vena cava and azygos continuation. To the best of our knowledge, this is the first report of successful neonatal ECMO despite azygos vein cannulation in a patient with such rare physiology

Intravenous thrombolysis in the emergency department for the treatment of acute ischaemic stroke

BACKGROUND AND AIMS: Thrombolytic therapy with intravenous recombinant tissue plasminogen activator (rt-PA) improves outcome in patients with ischaemic stroke treated within 3 h of symptom onset, but its extended implementation is limited. A pilot study was designed to verify whether evaluation of patients with acute ischaemic stroke and their treatment with intravenous rt-PA in the emergency department (ED), followed by transportation to a semi-intensive stroke care unit, offers a safe and effective organisational solution to provide intravenous thrombolysis to acute stroke patients when a stroke unit (SU) is not available. METHODS: After checking for inclusion and exclusion criteria, ED doctors contacted the stroke team with a single page, located family members and urgently obtained computed tomography scan and laboratory tests. A stroke team investigator clinically assessed the patient, obtained written informed consent and supervised intravenous rt-PA in the ED. After treatment, the patient was transferred to the SU for rehabilitation and treatment of complications, under supervision of the same stroke team investigator. RESULTS: 52 patients were treated with intravenous rt-PA within 3 h of symptom onset. 20 patients (38%) improved neurologically after 24 h, the number increased to 30 (58%) after one week. At 3 months 22 patients had a favourable outcome (43%). The 3-month mortality rate was 12%. Symptomatic cerebral haemorrhage was observed in two patients (4%). CONCLUSIONS: Intravenous rt-PA administration in the ED is an effective organisational solution for acute ischaemic stroke when an SU is not established

Labeling male anorectal malformations: objective evaluation of radiologic imaging before surgery

PurposeProne cross-table lateral x-ray (CTLxR) and colostogram aid surgical planning for anorectal malformations (ARMs) without perineal fistulas. We suggest objective imaging tools to classify ARMs.MethodsThree observers prospectively evaluated CTLxR and colostograms of male ARM patients (2012–2022) without perineal fistulas. The level of the rectal pouch was estimated with pubococcygeal (PC) and ischiatic (I) lines. On CTLxR, we described the “pigeon sign”, defined as the rectal pouch ending with a beak-like image, suspicious for a rectourinary fistula. ARM was defined as rectobulbar when the rectal pouch was below the I line, rectoprostatic when between PC and I lines, and rectovesical when above the PC line. Concordance was assessed with Fleiss' kappa. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the “pigeon sign” were calculated.ResultsThirteen patients were included in this study. The interobserver agreement on CTLxR was 69.2% (k = 0.54) on pouch ending, 84.6% (k = 0.69) on the “pigeon sign”, and 76.9% (k = 0.69) on diagnosis; concordance between observers and intraoperative diagnosis was 66.6% (k = 0.56). The “pigeon sign” had 75% sensitivity, 100% specificity, 100% PPV, and 50% NPV. The interobserver agreement on colostograms was 84.6% (k = 0.77) on pouch ending and 89.7% (k = 0.86) on diagnosis; concordance between observers and intraoperative diagnosis was 92.3% (k = 0.90).ConclusionPC and I lines and the “pigeon sign” are useful tools in examining CTLxR and colostograms. Adequate CTLxR interpretation may modify surgical strategy

A maChine and deep Learning Approach to predict pulmoNary hyperteNsIon in newbornS with congenital diaphragmatic Hernia (CLANNISH): Protocol for a retrospective study

Introduction Outcome predictions of patients with congenital diaphragmatic hernia (CDH) still have some limitations in the prenatal estimate of postnatal pulmonary hypertension (PH). We propose applying Machine Learning (ML), and Deep Learning (DL) approaches to fetuses and newborns with CDH to develop forecasting models in prenatal epoch, based on the integrated analysis of clinical data, to provide neonatal PH as the first outcome and, possibly: Favorable response to fetal endoscopic tracheal occlusion (FETO), need for Extracorporeal Membrane Oxygenation (ECMO), survival to ECMO, and death. Moreover, we plan to produce a (semi)automatic fetus lung segmentation system in Magnetic Resonance Imaging (MRI), which will be useful during project implementation but will also be an important tool itself to standardize lung volume measures for CDH fetuses. Methods and analytics Patients with isolated CDH from singleton pregnancies will be enrolled, whose prenatal checks were performed at the Fetal Surgery Unit of the Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (Milan, Italy) from the 30th week of gestation. A retrospective data collection of clinical and radiological variables from newborns' and mothers' clinical records will be performed for eligible patients born between 01/01/2012 and 31/12/2020. The native sequences from fetal magnetic resonance imaging (MRI) will be collected. Data from different sources will be integrated and analyzed using ML and DL, and forecasting algorithms will be developed for each outcome. Methods of data augmentation and dimensionality reduction (feature selection and extraction) will be employed to increase sample size and avoid overfitting. A software system for automatic fetal lung volume segmentation in MRI based on the DL 3D U-NET approach will also be developed. Ethics and dissemination This retrospective study received approval from the local ethics committee (Milan Area 2, Italy). The development of predictive models in CDH outcomes will provide a key contribution in disease prediction, early targeted interventions, and personalized management, with an overall improvement in care quality, resource allocation, healthcare, and family savings. Our findings will be validated in a future prospective multicenter cohort study

Single-stage surgical correction of anorectal malformation associated with rectourinary fistula in male neonates

INTRODUCTION: The treatment of children affected by anorectal malformations (ARM) is characterized by some unsolved problems. The three-stage surgical correction has been known to be most effective in preventing complications, but recently new approaches have been proposed. We describe our experience with the newer approaches. METHODS: Twenty three male newborns, affected by ARM and recto-urinary fistula, were treated in 2 different centers in 8 years. Nineteen neonates (birth weight 2.4 - 3.5 kg) received a primary posterior sagittal anorectoplasty (PSARP) at the Department of Pediatric Surgery of the Chittagong Medical College Hospital (group 1). Four term neonates (birth weight 2.9 - 3.4 kg) received a primary pull-through with combined abdomino-perineal approach at the Pediatric Surgery Department of Fondazione C\ue0 Granda of Milan (group 2). RESULTS: Among patients of Group 1, 11 patients had a recto-bulbar fistula and 8 a recto-prostatic fistula. Among the Group 2, 2 had a recto-bulbar fistula and 2 a recto-prostatic fistula. The site of fistula was decided at the time of surgery. In Group 1, 5 post-surgical complications were recorded (26%); 1 child died of sepsis, 3 had dehiscence and 1 stenosis, which resolved with dilatation. In Group 2, the only post-operative complication of small rectal prolapse resolved spontaneously after a few months on follow-up. Group 2 patients were followed-up in a dedicated multidisciplinary colorectal center. CONCLUSIONS: Primary repair of ARMs with recto-urinary fistula is a feasible, safe and effective technique in the neonatal period. A combined abdominal and perineal approach seems to guarantee better results. A dedicated team is mandatory, both for the surgical correction and for a long-term follow-up