Navigating the transition: a multidisciplinary approach to inflammatory bowel disease in children.

PURPOSE: A multidisciplinary approach to Inflammatory Bowel Disease (IBD) has recently demonstrated a positive impact in pediatric patients, reducing dropout rates and facilitating the transition to adult care. Our study aims to evaluate how this approach influences disease activity, dropout rates, and transition. METHODS: We conducted a longitudinal observational study including all patients diagnosed with IBD during pediatric-adolescent age, with a minimum follow-up period of 12 months. For each patient, endpoints included therapeutic approach, need for surgery and transition features. RESULTS: We included 19 patients: 13 with Ulcerative Colitis (UC) and 6 with Crohn's disease (CD). Most patients required multiple lines of therapy, with over 50% in both groups receiving biological drugs. Compliance was good, with a single dropout in each group (10, 5%). The need for surgery was significantly higher in the CD group compared to the UC group (16% vs. 7.7%, p < 0.01). Mean age at transition was significantly higher in the UC group compared to the CD group (19.2 ± 0.7 years SD vs. 18.3 ± 0.6 years SD, p < 0.05). CONCLUSIONS: In our experience, the multidisciplinary approach to IBD in transition-age patients appears effective in achieving clinical remission, offering the potential to reduce therapeutic dropouts.

Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia - why is transitional care mandatory?

PURPOSE:  to review recent literature concerning long-term health issues and transitional care in esophageal atresia (EA) patients. PubMed, Scopus, Embase and Web of Science databases were screened for studies regarding EA patients aged more than or equal to 11 years, published between August 2014 and June 2022. Sixteen studies involving 830 patients were analyzed. Mean age was 27.4 years (range 11-63). EA subtype distribution was: type C (48.8%), A (9.5%), D (1.9%), E (0.5%) and B (0.2%). 55% underwent primary repair, 34.3% delayed repair, 10.5% esophageal substitution. Mean follow-up was 27.2 years (range 11-63). Long-term sequelae were: gastro-esophageal reflux (41.4%), dysphagia (27.6%), esophagitis (12.4%), Barrett esophagus (8.1%), anastomotic stricture (4.8%); persistent cough (8.7%), recurrent infections (4.3%) and chronic respiratory diseases (5.5%). Musculo-skeletal deformities were present in 36 out of 74 reported cases. Reduced weight and height were detected in 13.3% and 6% cases, respectively. Impaired quality of life was reported in 9% of patients; 9.6% had diagnosis or raised risk of mental disorders. 10.3% of adult patients had no care provider. Meta-analysis was conducted on 816 patients. Estimated prevalences are: GERD 42.4%, dysphagia 57.8%, Barrett esophagus 12.4%, respiratory diseases 33.3%, neurological sequelae 11.7%, underweight 19.6%. Heterogeneity was substantial (> 50%).   Conclusion: EA patients must continue follow-up beyond childhood, with a defined transitional-care path by a highly specialized multidisciplinary team due to the multiple long-term sequelae. WHAT IS KNOWN: • Survival rates of esophageal atresia patients is now more than 90% thanks to the improvements in surgical techniques and intensive care, therefore patients' needs throughout adolescence and adulthood must be taken into account. WHAT IS NEW: • This review, by summarizing recent literature concerning long term sequelae of esophageal atresia, may contribute to raise awareness on the importance of defining standardized protocols of transitional and adulthood care for esophageal atresia patients.

Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.

Minimally-invasive surgery in paediatric oncology: proposal of recommendations.

The Authors report a proposal of recommendations concerning Minimally Invasive Surgery (MIS) in Paediatric Oncology. Since the exact role of MIS in Paediatric Oncology is still not completely defined, a restrict panel of Italian Paediatric Surgeons, some interested in Oncologic Surgery, others in MIS, prepared a schematic document, mainly founded on literature data, to provide Paediatric Surgeons with recommendations useful to approach paediatric tumours with MIS. The final draft was approved by the Italian Group of Paediatric Oncologic Surgeons. The Authors summarize the feasibility of MIS, when performed with different purposes (biopsy / resection) and timing (initial / delayed surgery) for the most common solid tumours in children. The oncologic criteria must be always followed with MIS as well as with "open" surgery.

Completely isolated enteric duplications cysts: a survey of four cases.

Enteric duplication cysts are uncommon congenital abnormalities with epithelial lining. They are cystic or tubular structures intimately attached to a portion of the gastrointestinal tract; they are usually located on the mesenteric site of the digestive tract sharing common blood supply. Isolated cystic duplications are an extremely rare variant with their own blood supply: in literature only five cases have been reported. We present our four cases series of this uncommon anomaly.

Two unusual cases of pharyngeal hairy polyp causing intermittent neonatal airway obstruction.

Hairy polyp of the pharynx is an uncommon developmental malformation that is most frequently seen as a penduculated tumour in the neonate. The clinical presentation is characterized by the presence of a polypoid mass protruding through the mouth as 'a second tongue' causing respiratory distress. Two patients are presented with this condition.

Thoracoscopic surgery in the management of pediatric malignancies: a multicentric survey of the Italian Society of Videosurgery in Infancy.

BACKGROUND: Few reports have elucidated the role of minimally invasive surgery (MIS) for pediatric malignancies. This study aimed to review the results of a multicenter study on the management of thoracic tumors in children using MIS. METHODS: A 5-year retrospective review of all MIS procedures for the treatment of pediatric malignancies performed in seven centers belonging to the Italian Society of Videosurgey in Infancy is reported. The data from 145 pediatric oncologic patients (80 girls and 65 boys) ages 30 days to 17 years (median, 7.2 years) were analyzed. Of the procedures performed, 87 were laparoscopies (60%), 55 were thoracoscopies (38%), and 3 were lumboscopies (2%). This study focused only on the results of the 55 thoracoscopic procedures performed for diagnostic purposes in 19 cases (34.6%) and for therapeutic purposes in 36 cases (65.4%). RESULTS: The duration of surgery was 15 to 180 min (median, 65 min). Metastasectomies were performed for various etiologies in 31 of the 55 cases. Of the 55 patients, 5 underwent resection of a mediastinal tumor, and 19 underwent a diagnostic thoracoscopy. During a mean follow-up period of 25.6 months, 2 (3.6%) of the 55 patients experienced perioperative complications. CONCLUSIONS: The role of MIS in tumor resection for children is currently limited, but may be used in individual cases when the preoperative workup shows it to be feasible. Its indication is strictly dependent on the thoracoscopic experience of the surgeon and the tumor site for preoperative imaging techniques. When the indication for thoracoscopy is correct, this approach has high therapeutic applicability (65.4% in our series). Our preliminary experience shows that careful patient selection and an appropriate level of technical skill make thoracoscopy a reasonable and safe option for the treatment of pediatric malignancies.

Italian multicentric survey on laparoscopic spleen surgery in the pediatric population.

BACKGROUND: Some technical aspects of laparoscopic spleen surgery still are debated, although efforts have been made to standardize them. The position of the patient, the approach to the spleen, vessel identification and division, and spleen extraction can vary from center to center. METHODS: This retrospective muticentric study led by the Società Italiana di Videochirurgia Infantile (SIVI) examined indications, surgical details, and complications of laparoscopic spleen surgery in the pediatric population during a 5-year period. RESULTS: The study period from January 1999 to December 2003 (5 years) involved nine centers and included 85 patients with a mean age of 10 years (range, 2-17 years). Hypersplenism or severe hemolysis in cases of hematologic disorders represented the most important indications. More than 90% of the patients underwent total laparoscopic splenectomy. Specific technical details from each center were collected. Intraoperative complications occurred in 19% of the patients (hemorrhage in 8% and technical problems in 14%), and 6% of the patients required conversion to the open approach. No deaths occurred, and no reoperations were required. Postoperative complications were experienced by 2% of the patients. CONCLUSION: Laparoscopic spleen surgery is safe, reliable, and effective in the pediatric population. On the basis of the results, some technical details for laparoscopic spleen surgery can be suggested. The patient is preferably kept supine or lateral, approaching the spleen anteriorly. Moreover, the ilar vessels should be identified selectively and individually, with initial artery division performed to achieve spleen shrinking. Any hemostatic device proved to be effective in experienced hands. Once freed, the spleen is preferably extracted via a suprapubic cosmetic transverse incision (faster, easier, and safer), although a bag can be used. Finally, the size of the spleen does not represent a contraindication for a trained and experienced surgeon. Nevertheless, this parameter must be considered when laparoscopic spleen surgery is planned.

A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

Italian multicenter survey on laparoscopic treatment of gastro-esophageal reflux disease in children.

BACKGROUND: Skepticism is still present today about the laparoscopic treatment of gastro-esophageal reflux (GER) in children. We present the prospective experience and short-term results of eight Italian pediatric surgical units. METHODS: We included all the children with complicated GER, operated after January 1998 by single surgeons from eight different centers. Diagnostic aspects, type of fundoplication, and complications were considered. All the patients were followed for a minimum period of 6 months in order to detect complications or recurrences. RESULTS: 288 children were prospectively included. Mean age was 4.8 years (3 m-14 y). Nissen fundoplication was done in 25%, floppy Nissen in 63%, Toupet in 1.7%, and anterior procedures (Lortat Jacob, Thal) in 10%. Gastrostomy was associated, if neurological impairment or feeding disorders were present. Mean follow-up was 15 months and reoperation was necessary in 3.8% of cases. CONCLUSIONS: This experience underlines that minimal invasive access surgery in children is safe and that the laparoscopic approach is considered in eight centers the golden standard for surgical repair of gastro-esophageal reflux disease maintaining the same indications and techniques of the open approach.

Features and outcome of neuroblastoma detected before birth.

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

[Current status in transplantation of the endocrine pancreas in the treatment of diabetes mellitus 2. Transplantation of fetal pancreas]. / Stato attuale del trapianto di pancreas endocrino nel trattamento del diabete mellito. 2) Il trapianto di pancreas fetale.

The latest findings support hypotheses that diabetes complications are secondary to metabolic control which current insulin therapy is unable to render constant. Surgical attempts to transplant the pancreas and Langerhans islets in the treatment of diabetes mellitus are reviewed and special attention paid to the use of the foetal pancreas which has proved particularly suitable in preventing microangiopathic complication. Comments are based on a careful review of the literature, made in the light of personal experience.

[Hirschsprung's disease in adolescents and adults]. / Il morbo di Hirschsprung nell'adolescente e nell'adulto.

Megacolon must be considered as a diagnosis in any case of intractable constipation also in the adult. Besides the Hirschsprung's disease and the idiopathic megacolon, which have no apparent organic cause, many other pathologic conditions may produce a marked dilatation of the large bowel. The differential diagnosis depends, after a valuation of the clinical and radiologic features, also from enzymatic, histologic and functional tests. The Hirschsprung's disease can be observed after the infancy in: 1) Patients in whom diagnosis is made only in adult age; 2) Patients with a diagnosis made in infancy or childhood, with moderate symptoms, treated with a conservative methods, who are getting worse; 3) Adult relapsers after a previous inadequate operation. Group II also includes the patients who need an emergency operation, because of a perforation or a volvolus of a dilatated loop. We report three cases of adult Hirschsprung's disease and two adult "idiopathic megacolon". The patients with aganglionic megacolon were admitted to our Hospital respectively for an abdominal palpable mass, a volvolus and incontinence. The two patients with idiopathic megacolon showed the same clinical picture of those with Hirschsprung's disease. We choose a surgical therapy in all five patients, with good long-term results.

[Sickle cell anemia in surgical patients. Significance and problems]. / La falcemia nel paziente chirurgico. Significato e problemi.

Drepanocytosis, a disease notoriously widespread among blacks, is surprisingly frequent in certain Italian islands. Surgical aspects of the disease are discussed: erythrocyte sequestration attacks, pigmentary cholelithiasis, priapism, malleolar ulcers and vaso-occlusive attacks. A knowledge of such aspects is considered fundamental for the purposes of a reasoned differential diagnosis in the field of paediatric pathologies requiring surgery.

[Experimental intrauterine surgery: the creation of a congenital diaphragmatic hernia in a sheep fetus]. / Chirurgia sperimentale endouterina: la creazione di un'ernia diaframmatica congenita nel feto di pecora.

Up to 50% of the neonates operated during the first hours of life for a congenital diaphragmatic hernia die. The presence of a severe lung hypoplasia, which is at the origin of hypoxia, acidosis, increased pulmonary vascular resistance and right to left shunt, explains the poor clinical results, in spite of surgical success and intensive therapy. A modern approach to the problem includes treatment with pulmonary vasodilator drugs, whose effects are still discussed. Perhaps the prenatal recognition of the defect could improve prognosis. Lots of experience are needed to know more on the subject. This could be obtained with an experimental approach on newborn animals with a CDH. The article relates authors experience in the creation of a CDH in a foetus of sheep, by means of a surgical intervention during its intrauterine life.

[Experimental intrauterine surgery. Morphological study of lung development in the sheep fetus with congenital diaphragmatic hernia]. / Chirurgia sperimentale endouterina. Studio morfologico dello sviluppo polmonare nel feto di pecora con ernia diaframmatica congenita.

Experimental comparison of the lungs of 7 sheep foetuses with surgically induced CDH and 7 controls permitted an assessment to be made of the changes that take place in lung growth, generally described as hypoplasia, through a study of their morphology and histology, and the lung: lamb weight ratio. Changes increased in gravity in function of the duration of hernia. They included: reduced alveolar expansion, fewer generations of bronchi and alveoli, and septal thickening. An increase in the smooth muscle component of 5th-6th generation arteries (i.e. resistance) may offer an explanation of the hypertension characteristic of CDH, and the non-reactivity of these vessels in response to vasodilators. It is also suggested that damage to the mesenchyma can be regarded as the sole cause of the changes in lung growth observed in CDH. Early treatment before these changes become irreversible is thus advisable.

[Transplantation of the left lung in a 2-month-old piglet. The Study Group for Lung Transplantation]. / Il trapianto di polmone sinistro nel suinetto di due mesi. Gruppo di Studio per il Trapianto di polmone.

Experimental surgery in animals remains an irreplaceable model for the clinical application of a new technique. We performed a single lung allotransplantation in young pigs. The pigs were organized into groups: 1) 6 to assess the surgical anatomy; 2) 10 to receive left transplantation; 3) 3 to study the pathophysiology of the transplanted lung. The preliminary results demonstrated that the surgical model is useful.

[The reno-calyceal index. Radiological evaluation of pyelocalyceal distensions in children]. / L'index réno-caliciel. Appréciation radiologique des distensions pyélo-calicielles chez l'enfant.

The ratio length of one kidney width of the lower calyx, measured pre and post-operatively, is proposed as an objective index to assess the result of pyeloplasty in surgical treatment of hydronephrosis. Statistical approach on a series of 25 children with unilateral hydronephrosis and normal contralateral kidney has demonstrated the reliability of this index to determine the evaluation of the dilatation in the post-operative period. An increase of the ratio was present in all cases in which the result of surgery, as far as distension was concerned, was satisfactory.