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.
We describe an uncommon clinical presentation of caudal duplication syndrome and features of caudal regression syndrome in a female infant with complex urogenital and colorectal duplication associated with lipomyleomeningocele and left lower limb hypoplasia. A staged surgical treatment plan was carried out to maintain fecal and urine continence, potential fertility, and cosmetic appearance.
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.
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