Thoracoscopic Resection of Pulmonary Lesions in Israel: The Mentorship Approach.

Background: Most congenital pulmonary airway malformations (CPAMs) are detected antenatally. The majority of newborns are asymptomatic. Patients are prone to subsequent respiratory complications and to a lesser extent malignant transformation remains concerning. In Israel, until 2013, pediatric surgeries were performed by thoracotomy. To minimize its morbidity, we introduced thoracoscopy using a mentorship approach. We present our experience with thoracoscopic resections coordinated by the mentorship of a pediatric worldwide leader in his field and compare our results with resections performed by thoracotomy. Materials and Methods: A retrospective review of records of children operated between 2013 and 2020 was conducted. Data were compared using t-test for quantitative variables. Results: Fifty patients were operated by thoracoscopy with a median age of 4 years, a thoracoscopic lobectomy performed in 68%. There was no conversion with a median length of stay (LOS) of 3½ days. Thirty patients were operated by thoracotomy by a thoracic surgeon with a median age of 3.5 years. A lobectomy was performed in 87% with a median LOS of 7 days. Conclusions: Thoracoscopic lobectomy is a technically demanding procedure with a long learning curve, strongly related to the low volume of cases. The role of a mentorship program in acquiring those surgical skills is crucial through standardization of the technique applied and supervised by the mentor. Early thoracoscopy for congenital pulmonary lesions at an early age can be achieved with a low conversion rate and minimal complications creating a change in the paradigm of practice when considering surgery for CPAM in Israel.

Multiple magnet ingestion in children: A problem on the rise.

AIM: Ingestion of multiple magnets is increasing these last 15 years in children. They have resulted in numerous reports of serious gastrointestinal complications such as bowel obstruction, ischaemia, necrosis, perforation and fistula formation and even led to death. The increasing number of world-wide reports of complications secondary to magnet ingestion and a frequently delayed diagnosis point to a lack of awareness about these risks among medical care-givers in our country and parents in general. METHODS: We reviewed retrospectively all cases of multiple magnet ingestion that required a gastro-intestinal or surgical procedure for removal from 2009 to 2020. RESULTS: Five children underwent gastroscopy removal and three colonoscopy removal of the magnets. Five patients required surgical (laparotomy or laparoscopy) removal of multiple magnets with intestinal perforations. CONCLUSIONS: We propose an updated management algorithm for multiple magnet ingestion to highlight awareness among primary physicians and parents of the presenting circumstances and symptoms as well as the potential complications associated with multiple magnet ingestion.

Is fundoplication mandatory in children with neurological impairment undergoing gastrostomy?

AIM: Percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG) are common procedures in children with neurological impairment (NI) with swallowing difficulties. Pulmonary aspirations are a major concern and performing concomitant or delayed fundoplication is still controversial, especially among these patients. The aim of our study was to review our experience with fundoplication performed concomitantly with gastrostomy or later and to evaluate patient outcomes. METHODS: This is a retrospective, cohort study including all paediatric patients who underwent SG or PEG with or without Nissen fundoplication at Schneider Children's Medical Center of Israel between the years 2007 and 2018. Patients' clinical and surgical data were recorded and analysed. RESULTS: Between 2007 and 2018, 345 patients underwent SG or PEG. Of these, 89 patients underwent fundoplication. Of the patients who underwent PEG/SG, 158 (45.8%) were neurologically impaired. Most of the patients who underwent fundoplication (n = 69, 77.5%) were NI patients (P = 0.0001). NI patients with refractory seizures showed almost no improvement in terms of relief of gastro-oesophageal reflux disease symptoms following fundoplication (P = 0.0001) compared to NI patients without refractory seizures. CONCLUSION: Our findings suggest that in NI patients a concomitant fundoplication is not mandatory and is not efficacious in preventing gastro-oesophageal reflux disease in patients with refractory seizures.

Aerodigestive Clinics as Emerging Pediatric Care Model: The First 100 Patients in Israel.

BACKGROUND: Aerodigestive clinics are run by interdisciplinary medical and surgical teams, and provide complex care coordination and combined endoscopies. OBJECTIVES: To describe the design and patient population of the first pediatric aerodigestive center in Israel. METHODS: A retrospective single-center cohort study was conducted describing patients followed in the aerodigestive clinic of Schneider Children's Medical Center of Israel, a tertiary pediatric hospital, between its inception in January 2017 and June 2020. RESULTS: During the study period, 100 patients were seen at the combined respiratory and digestive (NoAM) clinic, with a total of 271 visits. Median age at first assessment was 29.5 months (range 3-216). Fifty-six patients (56%) had esophageal atresia and tracheoesophageal fistula. Thirty-nine patients had an identified genetic disorder, 28 had a primary airway abnormality, 28 were oxygen dependent, and 21 were born premature. Fifty-two patients underwent triple endoscopy, consisting of flexible bronchoscopy, rigid bronchoscopy, and gastroscopy. In 33 patients, esophageal dilatation was necessary. Six patients underwent posterior tracheopexy at a median of 6 months of age (range 5 days to 8 years) all with ensuing symptom improvement. The total mean parental satisfaction score on a Likert-type scale of 1-5 (5 = highest satisfaction) was 4.5. CONCLUSIONS: A coordinated approach is required to provide effective care to the growing population of children with aerodigestive disorders. The cross fertilization between multiple disciplines offers a unique opportunity to develop high quality and innovative care. Outcome measures must be defined to objectively measure clinical benefit.

Not only appendicitis: rare appendix disorders manifesting as surgical emergencies in children.

Acute appendicitis is the most common cause of acute abdominal pathology in children. However, other rare non-inflammatory non-neoplastic disorders involving the appendix may manifest as surgical emergencies. This study aimed to describe these atypical entities and present representative cases. The database of a tertiary children's medical center was reviewed for all the patients aged 0-18 years who underwent urgent appendectomy between June 2014 and December 2019, for rare disorders of the appendix unrelated to inflammatory or neoplastic processes. Of 1367 patients who underwent appendectomy, 1345 were operated urgently or emergently. Of these, six, all males, mean age 32.6 months (range 0.7-76), underwent appendectomy for rare surgical complications that involved the appendix. These included torsion of the appendix (2), a strangulated internal hernia through an appendicular ring (1) or through a mesoappendix gap (1), an incarcerated appendix in an acute hernia sac (1), and appendiceal intussusception (1). In all cases, the role of the appendix in the pathologic process was unexpected and came as a surprise to the surgeon. During a median follow-up of 4.2 months (range 1-8 months), one patient underwent relaparotomy for small bowel obstruction 4 weeks after the original procedure.Conclusion: The appendix in children can be the source of rare pathological disorders that present as surgical emergencies. Familiarity with these entities may aid in achieving accurate preoperative diagnosis and contribute to surgical team orientation on exploratory laparotomy. However, correct diagnosis is often only established during timely surgical intervention.

Emergency or urgent splenectomy in children for non-traumatic reasons.

Emergency splenectomy is rarely performed since a widespread consensus exists towards conservative management of splenic injury. However, in selected conditions, mainly hematological, there is a role for emergency or urgent splenectomy. This study aims to retrospectively review these cases and discuss outcome in relation to the pre-existing splenic pathologies. Between 2000 and 2015, 12 patients, five girls, and seven boys, with a median age of six years (3 months-13.11 years), underwent emergency or urgent splenectomy for non-traumatic conditions. All patients had major associated disorders; mainly hematological (11 cases) including hemolytic anemia with pancytopenia (1), sickle cell anemia (1), AML (1), ALL (2), CML (1), T cell lymphoma (1), Burkitt lymphoma (1), and ITP (3). One patient had a microvillous inclusion disease. Indications for splenectomy included diffuse resistant splenic abscesses (4), intracranial hemorrhage (4) or hypersplenism (3) with refractory thrombocytopenia, and spontaneous splenic rapture (1). Nine patients improved following surgery but three died, owing to massive intracranial hemorrhage (1) and severe respiratory failure (2) despite aggressive management.Conclusions: Rarely, an emergency splenectomy is required in complex settings, mostly refractory hematological conditions, in a deteriorating patient when all other measurements have failed. A multidisciplinary team approach is mandatory in the treatment of these complex cases. What is known • Conservative treatment is advised for splenic injury. • Many hematological disorders are responsible of splenic pathology. What is new • Emergency splenectomy in children for reasons other than trauma is a treatment of last resort that should be performed in a multidisciplinary context. • The outcome of emergency splenectomy in children for reasons other than trauma depends on the underlying medical condition.

Current management of lipoblastoma.

Lipoblastoma is a rare and benign tumor arising from embryonal fat cells. It is generally diagnosed in children younger than 3 years of age and can occur in the extremities or on the trunk. We present our series of 10 children with lipoblastoma treated at Schneider Children's Medical Center of Israel between 2011 and 2016. Six boys and four girls underwent tumor resection at a median age of 2 years and 3 months (range 5 months to 5.6 years). Locations were trunk (6), groin (2), perineum (1), and omentum (1). Follow up ranges from 1 to 5 years. Two patients had a local recurrence and required a second resection 2 years (perineal) and 6 years (trunk) after the first surgery without further recurrence at 1.9 and 2.9 years, respectively. CONCLUSION: Higher awareness of lipoblastoma enables optimal imaging strategies and resection. Long follow up is required due to local recurrences. The treatment of choice consists of complete, but non mutilating surgical resection. What is Known: • Lipoblastoma is a rare benign tumor of fatty tissue affecting children • Treatment consists of surgical resection What is New: • MRI is the modality of choice for follow up • Ten-year long-term follow up is required due to late recurrence.

Hypertrophic pyloric stenosis following repair of esophageal atresia and tracheo-esophageal fistula.

Two cases of hypertrophic pyloric stenosis (HPS) developed after a few weeks of repair of an esophageal atresia and tracheo-esophageal fistula (EA and TEF). Both cases were dealt successfully with laparoscopic pyloromyotomy.

Androgen insensitivity syndrome: risk of malignancy and timing of surgery in a paediatric and adolescent population.

BACKGROUND: Management of female phenotype XY disorders poses a series of problems for the treating clinician. Even after a series of investigations and imaging modalities, there are lingering doubts about the exact nature of the disease and the correct management option. Optimal timing and necessity for removal of their testes have been a debated issue by physicians. There is a generally accepted opinion among physicians that the risk of malignancy in androgen insensitivity syndrome (AIS) is considerably lower than with other intersex disorders and occurs at a later age. OBJECTIVE: The highlight of this presentation is to reinforce the value of laparoscopic gonadectomy in management of AIS in correlation with data suggesting higher risk of malignancy. PATIENTS AND METHODS: A retrospective review of 11 phenotypic females with XY karyotype was carried out. The patients were evaluated by a diagnostic protocol which included clinical, hormonal, sonographic and cytogenetic examinations. Patients/parents were counselled by the team concerning the different treatment modalities and contrary to the assigned gender, laparoscopy was offered to them. Uneventful bilateral gonadectomy was performed in all the patients and gonads submitted for histopathological examination. RESULTS: A total of 11 patients (mean age, 10.4 ± 4.1 years), including six with complete AIS and five with partial AIS (PAIS) were reviewed. In two patients with PAIS (18.1%), histopathology revealed malignancy (bilateral seminoma and gonadoblastoma) and in an additional patient, a benign hamartoma was found. Literature evidence suggests that AIS female phenotype patients retaining their testes through puberty have a 5% chance for developing malignant tumours. Reviewing our results in correlation with literature, we found that PAIS patients may harbour a higher risk of malignancy. CONCLUSIONS: In complementation to hormonal tests and cytogenetic techniques, laparoscopic gonadectomy is required to complete the diagnostic work up for AIS as it also adds a final therapeutic approach with low risk and huge benefit. Since laparoscopy is now a well-tolerated and widely accepted gold standard, it should be included in routine management for patients with AIS. Risk of malignancy in PAIS should be investigated in larger cohort of these patients.

Management of primary spontaneous pneumothorax in children.

OBJECTIVE: To examine the role of CT scans and early surgical intervention in the management of pediatric patients with primary spontaneous pneumothorax (PSP). METHODS: Retrospective cohort study. RESULTS: The authors identified 46 cases with 70 episodes of pneumothorax. The recurrence rate among conservatively treated patients was 50% both after the first and the subsequent episode. Recurrence rate in cases with and without blebs on CT was comparable. Initial episodes were treated with supplemental oxygen (n = 18) and chest tube drainage (n = 18), and 10 patients underwent video-assisted thoracoscopic surgery (VATS). The recurrence rate was significantly lower following surgical intervention compared with other therapy, and morbidity was comparable with that in patients who needed chest tube drainage. CONCLUSIONS: Recurrence after the first episode of PSP in children is frequent and is difficult to predict by CT findings. VATS is safe and effective in preventing recurrences. Surgical intervention may be an attractive alternative in patients who require chest tube drainage for the first episode of PSP.

Fibrous hamartoma of infancy.

Fibrous hamartoma of infancy is a rare benign soft tissue tumor that usually occurs within the first 2 years of life. We report 3 cases of fibrous hamartoma of infancy seen over a 10-year period. Treatment is curative by local excision with a low local recurrence rate. Fibrous hamartoma of infancy should be considered in the differential diagnosis of soft tissue masses in children because it is always benign and subject to nonaggressive treatment.

The management of long gap esophageal atresia.

BACKGROUND: Present management of esophageal atresia has enabled the survival rate to approach 95%. Controversy remains concerning the many options for the surgical management of long gap esophageal atresia without tracheoesophageal fistula and represents the difficulty of this pathology. In the last couple of years, we have had a nonexplained outbreak of cases of long gap esophageal atresia without tracheoesophageal fistula. This article reports our experience in the management of these children. MATERIAL AND METHODS: It is a retrospective study of all cases of long gap esophageal atresia without tracheoesophageal fistula managed in our institution since 1992, focusing on the antenatal period, delivery with weight and term, the associated malformations, the initial management, and the definitive surgery. Mann-Whitney U test was used for statistical analysis. RESULTS: Ten cases (8.7%) of long gap esophageal atresia according to Ladd's classification, 6 during the past 2 years, were taken in charge at Robert Debré Hospital between 1992 and 2002. There were 4 girls and 6 boys. Ten had a prenatal diagnosis of esophageal atresia. The average birth weight was 2496 g (range, 1400-3400 g) with an average term of 36.6-week gestation (range, 31.5-39.6). Delayed reconstruction was done in all children between 41 and 147 days of life (average of 102 days). Six had a direct anastomosis and 4 had a colonic esophagoplasty (3 with an esogastric disconnection during the same procedure). The average follow-up was 60 months (range, 27-133). There was 1 death owing to adenovirus infection at 5 years of age. Four children required a Nissen fundoplication for severe gastroesophageal reflux. At least, 2 children presented an anastomotic stricture which required pneumatic dilatations. CONCLUSION: Treatment options for long gap esophageal atresia generally require several stages over several months. We propose, for their management, a direct anastomosis at 4 months of age whenever it is possible. If not, we use a colonic esophagoplasty with an esogastric disconnection to control the gastroesophageal reflux which is responsible for strictures and respiratory impairment and does not obstruct the aperistaltic tube.