Pediatric esophageal stenoses: Challenges and new surgical device promoting tension-free esophageal anastomosis.

Esophageal stenoses of childhood have a broad spectrum of underlying causes. Their treatment is usually minimally invasive by endoscopic means, but sometimes surgery is necessary in refractory cases. Techniques employed in the surgical treatment of esophageal strictures include resection of the stenotic esophageal segment or esophageal substitution procedures. Esophageal anastomosis has always been a challenge in pediatric surgery. Anastomosis complications are linked to anatomical, biological and technical aspects. Mechanical tension between esophageal ends is an important cause of complications including anastomotic leaks or dehiscence. Eleven cases of esophageal stenoses, surgically treated in the Pediatric Surgery Department of Emergency Clinical Hospital for Children 'Marie S. Curie' by a single team in 5 years, were included in the present study. The results showed that, the main causes of esophageal stenosis were represented by corrosive esophageal injury in five cases, complications of esophageal atresia repair in three cases, congenital esophageal stenosis in two cases and chemotherapy-induced esophageal necrosis in acute lymphoblastic leukemia treatment in one case. The authors also designed and presented a device facilitating esophageal anastomosis under tension. Its principle involved temporary absorption of tension at secure points of the two esophageal pouches and reallocating it in equal amounts following anastomosis while decreasing any stretch-related tissue trauma. In conclusion, this auxiliary tool is beneficial for esophageal anastomosis; however, the standard steps of the esophageal anastomosis procedure should still be considered when necessary.

Antiproliferative therapy with sirolimus and propranolol for congenital vascular anomalies in newborns (Case reports).

We present a series of four newborns diagnosed with complicated congenital vascular anomalies, with different localization: Congenital lymphatic malformation (CLM) on the left hemithorax extending on the left upper limb; congenital hepatic hemangioma (CHH) with important complications in the first 7 weeks of life; Kaposiform hemangioendothelioma (KHE) of the left lower limb complicated with Kasabach Merritt phenomenon (KMM) and most probable diffuse capillary malformation with overgrowth (DCMO). All patients were treated with combined antiproliferative therapy with sirolimus and propranolol. The initial dose of sirolimus was 0.45-0.5 mg/m2 with doses adjusted according to plasmatic levels. Therapeutic intervals of sirolimus were considered at plasmatic levels of 7-12 ng/ml. Our aim was to use the lowest therapeutic dose in order to avoid possible side effects. Propranolol was initiated in doses of 0.5-1.0 mg/kg/day and was increased up to 3.0 mg/kg/day depending on tolerability. Following two months, every patient showed a marked reduction in the size of the mass, improvement in overall appearance or even calcification in the liver vascular tumor. No patient showed life threatening side effects to the treatment. Hypertriglyceridemia was the only side effect noted in all patients. This is in accordance with several international studies, which try to demonstrate the importance of sirolimus in neonatal vascular malformations in monotherapy or combined with different drugs.

Lipoblastoma: Diagnosis and surgical considerations.

Lipoblastoma (LB) and lipoblastomatosis (LBS) are uncommon benign mesenchymal tumors of embryonal fat, occurring almost exclusively in infancy and early childhood. These fast-growing tumors have an excellent prognosis if properly treated. Eight consecutive children having pathologically demonstrated LB treated by the same surgical team were retrospectively reviewed. There were 5 boys and 3 girls between 7 to 36 months (median age 22 months). The localization of the tumors was on the thigh (1 case), abdomen (2 cases), axillary and pectoral region (1 case) paragluteal region (1 case), lumbar area (1 case), inguinal-scrotal (1 case), and in one case, presacral, gluteal and perirectal region (1 case). Five were focal and in 3 cases an infiltrative growth pattern was observed. One case exhibited a gross appearance resembling sacrococcygeal teratoma, with associated Dravet syndrome. No recurrence was noted in our series, after a mean follow-up of 28 months post operatory. Despite its rareness, LB must be kept in mind when diagnosing a rapidly growing fatty mass in children. Even when dealing with very large abdominal LB, complete surgical excision is possible, with an excellent prognosis. Due to the relatively high recurrence rate noted in the literature, particularly in LBS, follow-up is extremely important.

Practical safety in the diagnosis and treatment of congenital isolated tracheoesophageal fistula.

The rareness of H-type tracheoesophageal fistula in conjunction with its unspecific clinical presentation and wide range of anatomical presentation makes its diagnosis and treatment a problematic topic for both ear, nose and throat (ENT) specialists and pediatric surgeons worldwide. Symptoms and clinical signs of H-TOF are easily misleading. Diagnostic methods, most of the times, are dependent on the physician's experience; therefore, various errors may be made. We analyzed our experience in managing H-TOF cases over the last 15 years. Advice and strategies of action for health professionals directly involved in the diagnosis and treatment were identified, but also errors and mistakes while managing 6 cases. We analyzed 'red flags' but also important steps in the practical safety concerning this rare congenital malformation. Choosing the surgical access for division of the fistula throughout the cervical or thoracic approach is sometimes difficult. A scrupulous perioperative planning is mandatory. A dynamic overview of the patient's presentation never underestimating the subtlety of H-TOF presentation should be conducted for its early recognition and achieving best outcomes.

Alimentary tract duplications in children - a 15 years' experience.

Duplications of the alimentary tract are a diverse and complex spectrum of congenital malformations and can be found anywhere along the digestive tract. The management depends on multiple factors, such as age, location, size, macroscopic aspect, and the associated anomalies. This study reflects a 15-year single surgical team experience. We reviewed medical records of 35 consecutive patients presenting alimentary tract duplications, evaluated and managed between 2004 and 2019. The anatomical distribution included: oral structures (two cases), esophageal (three cases), gastric (three patients), jejunoileal (seven cases), ileocecal (12 cases), colonic (six cases), anorectal (one case), and one case of complex tubular duplication of the terminal ileum and entire colon with two anal openings at the perineum. Four patients had antenatal diagnosis, initially asymptomatic, were followed, after birth, with repeated ultrasound examinations for a medium period of 3.8 months. All cases were managed with open surgery. Excision of the lesion with preservation of the gut integrity could be performed in 28 of the cases, while in six cases, enterectomy followed by digestive anastomosis was required. In one complex caudal duplication syndrome, the duplicated tubular colon was left in place. The postoperative complications were gastroesophageal reflux disease (GERD) (two cases), Claude Bernard-Horner syndrome (one case), wound infection (one case), and in one case, massive tongue edema. Clinical findings may be misleading, imaging studies may be uncertain, therefore the surgeon remains to complete de picture with intraoperative findings. In complex duplication cases, a multidisciplinary approach is imperative for the best results.