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.

Background: Preputial calculi are rarely encountered in childhood.Case characteristics: A 5-year-old boy with symptoms of chronic balanoposthitis. Observation: A preputial stone was documented and removed at circumcision. Outcome: Uneventful postoperative recovery. Message: In children, association between phimosis and neurologic impairment represent predisposing condition for preputial stone formation.

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/m 2 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.

The present study presents the experience gained in the Newborn Intensive Care Unit (NICU) of ‘Maria S. Curie’ Emergency Clinical Hospital for Children in Bucharest (Romania) after performing a series of bedside surgery interventions on newborns with congenital diaphragmatic hernia (CDH). We conducted a retrospective analysis of the data for all patients operated on-site between 2011 and 2020, in terms of pre- and post-operative stability, procedures performed, complications and outcomes. An analysis of a control group was used to provide a reference to the survival rate for non-operated patients. The present study is based on data from 10 cases of newborns, surgically operated on, on average, on the fifth day of life. The main reasons for operating on-site included hemodynamical instability and the need to administer inhaled nitric oxide (iNO) and high-frequency oscillatory ventilation (HFOV). There were no unforeseen events during surgery, no immediate postoperative complications and no surgery-related mortality. One noticed drawback was the unfamiliarity of the surgery team with the new operating environment. Our experience indicates that bedside surgery improves the likelihood of survival for critically ill neonates suffering from CDH. No immediate complications were associated with this practice.

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Background and Objectives: Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. Materials and Methods: A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Results: Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). Conclusions: Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.

Independent Cushing's adrenocorticotropic hormone (ACTH) syndrome can have several causes, including adrenal carcinoma or simple adrenal hyperplasia. Although the distinction between malignant and benign can be effectively made through imaging investigations, in the situation where there are bilateral formations, their hormonal activity is impossible to appreciate via non-invasive techniques. The present study includes the presentation of a clinical case on the basis of which a literature review was made. The clinical case pertains to a 32-year-old patient with ACTH-independent Cushing's syndrome and bilateral adrenal tumor formations leading to the utility of adrenal venous sampling to avoid bilateral adrenalectomy. A literature review was subsequently conducted focusing on articles pertaining to the PICO criteria in order to describe: The diagnosis of adrenal tumor masses, the decision on the type of surgery and most importantly, the impact of adrenal venous sampling in avoiding surgical resection. These interventions severely limit the patients' quality of life.

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.