8-year experience of esophageal atresia repair in Sarvar children hospital [Mashhad- IRAN]

Esophageal atresia [EA] is a congenital anomaly treated by surgical reconstruction. Some early postoperative complications may be encountered we assessed complications following EA repair in a large series of neonates with EA / TEF and in hospital mortality among a large series of our cases. 243 patients with EA / TEF that were treated operatively in Sarvar Children's Hospital from 2002 to 2010 were studied. Early post-operative complications in the ICU and surgery ward until hospital discharge were assessed. Mean age was 3.4 +/- 2.76 days. Primary repair was performed in 83.5% Mean hospital stay was 12.5 +/- 12.81 days. Respiratory problems and food intolerance were the most common early complications. In-hospital mortality rate decreased significantly during the last 8 years [from 17.6% to 4.7%]. Acceptable results and a growing survival rate were observed in this series of patients and we anticipate better results with improvements in minimally invasive methods

Fibrous hamartoma of infancy, report of two cases

Fibrous hamartoma of infancy [FHI] is a rare, benign soft tissue tumor that typically occurs within the first two years of life. It has a specific histologic appearance comprising of three different mesenchymal tissues with variable proportions in an organoid fashion. The clinical course is typically benign with excellent prognosis. We report two cases of this rare lesion and review its cilinicopathologic characteristics The first case was a 15-month-old girl who had a subcutaneous mass in the right axillary region and the other one was an 18-month-old boy with a mass on the medial surface of his right ankle. The masses were successfully excised. After 6 and 30 months follow up no recurrence occurred. Although the clinical and imaging findings of FHI are quite similar to those of malignant soft tissue tumor, histologic characteristics of this tumor will guide to the definite diagnosis that will prevent aggressive and mutilating procedures

Omental-mesenteric myxoid hamartoma mimicking malignancy in a 14-month-old child [a case report]

The omental-mesenteric myxoid hamartoma [OMMH] is a very rare lesion, mainly seen in children and characterized by multiple omental and mesenteric nodules, which may be confused with malignant neoplasm. Microscopically, these lesions consist of a richly vascularized myxoid stroma with plump mesenchymal cells. This lesion has a benign clinical course without recurrence during follow up. We present a 14-month-old boy that was referred with history of abdominal distension, fever and vomiting for 3 months. Enhanced computed tomography [CT] revealed a huge well-demarcated hypodense and spherical mass which displaced bowel loops without obvious penetration to the intestinal walls. Histological and immunohistochemical examinations confirmed the diagnosis of OMMH. No evidence of recurrence was noted during 3 years follow up. OMMH is a very rare lesion and because of its aggressive appearance, differential diagnosis with malignancy is warranted. The clinical picture of our case also led to high suspicion of malignancy. However by consideration of histological and immunohistochemical findings we could achieve the correct diagnosis

Congenital embryonal rhabdomyosarcoma with prenatal onset

Rhabdomyosarcoma [RMS] is the single most common type of soft tissue sarcoma in children and adolescents but it is extraordinarily rare in neonates. Extremity RMS comprises 20% of all sites, occurs more commonly in the leg than in the arm and accounts for 9% of all RMS cases. According to our review, this is the second case of RMS on day one of life with congenital, antenatal feature, and postnatal progressive clinical course of a large tumor of the hand [pretreatment staging T2bN1M0] with embryonic histological subtype and unfavorable prognosis. The patient is a term newborn boy with huge mass in the right hand and palpable lymph node in subaxillar region. Congenital embryonal rhabdomyosarcoma is a rare form of sarcomas with congenital in nature, antenatal feature and post natal progressive clinical course of sarcomas of extremities in newborn infants