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A full-term male neonate was initially managed for respiratory distress which developed few hours after birth. His initial chest radiograph was normal, and blood culture revealed Group B streptococcal (GBS) sepsis. He subsequently developed progressive right chest opacification that did not improve with medical management. Imaging done few days later revealed right-sided diaphragmatic hernia. The 12-day-old neonate underwent primary repair of the diaphragmatic defect and had an uneventful recovery. This case report intends to highlight this unique association between early onset GBS sepsis and delayed onset of the right congenital diaphragmatic hernia.

Context: Nonurological malignancies in children include a wide variety of tumors. These tumors include primary tumors of the liver, thyroid, lung, gastrointestinal tract (GIT), and adrenals; soft tissue sarcomas (STSs) like rhabdomyosarcoma (RMS) and non-RMS; and finally extragonadal germ cell tumors (GCT). Aims: This article aims at describing the current thinking in the management of these childhood solid tumors. This is critical in view of the recent advances in the elucidation of the molecular, genetic, and biologic behavior of these tumors and how these factors are getting integrated not only in the staging but also in developing a risk-based approach towards the management of these tumors. Materials and Methods: Reference was made to recently published literature from the leading pediatric cancer centers of the world to make a sense of things of the most current thinking in this rapidly expanding field. This will provide surgeons and physicians taking care of these children with a working knowledge in this somewhat challenging field. Conclusions: Treatment results vary from center to center depending on access to resources and following different management protocols. Results have improved for these tumors with the advent of newer chemotherapeutic agents, novel delivery methods of radiation therapy (RT), and improvement in surgical technique. Due to the limited number of patients presenting with these tumors, national and international collaboration of data is critical for all and beneficial to individual treatment centers. This has resulted in better results in the past and will definitely result in still better results in the future.

Background Infantile fibrosarcoma (IFS) is an uncommon soft-tissue sarcoma. Here we review our experience treating this tumor. Patients and methods We retrospectively reviewed records of patients with IFS treated at St. Jude Children’s Research Hospital between 1980 and 2009. Results We identified 15 patients, 8 girls and 7 boys; 13 white and 2 black. Median age at diagnosis was 3 months. Primary sites included the leg ( n  = 3), chest wall ( n  = 2), foot ( n  = 2), and one each in the tongue, occipital region, axilla, parascapular region, arm, forearm, retroperitoneum, and thigh. All patients underwent resection; 11 upfront surgery, and 4 delayed. Complications included loss of the posterior tibial nerve and artery, axillary vein, biceps, pectoralis major, gallbladder, and transverse/sigmoid sinus. Eight received chemotherapy and three radiotherapy. Seven experienced local recurrence and three lung metastasis. Median follow-up was 65 months. At the time of the review, 12 patients were alive and 3 had died. All deaths were in patients older than 1 year at diagnosis with an axial primary site. Conclusions Non-mutilating surgery should be the primary treatment for IFS. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible. Patients with positive surgical margins should receive adjuvant chemotherapy. Radiotherapy is indicated for axial primary sites where complete resection is impossible.

Purpose The purpose of this study was to describe the use of lymphoscintigraphy and sentinel lymph-node biopsy (SLNB) for the management of children with melanoma and sarcomas. We report the experience of two children’s hospitals that utilize this technique to identify sentinel lymph nodes for lymph-node biopsy and dissection. Methods We identified 56 patients (median age 10.8 years) who underwent 58 lymphoscintigraphy procedures. There were 33 patients with melanoma and melanocytic lesions, and 23 with sarcomas. Results Of 58 lymphoscintigraphy procedures, sentinel lymph nodes were identified in 52 (90% success rate). Using the combination of intraoperative blue dye injection and lymphoscintigraphy, the success rate was 95% (55/58). Metastatic disease was found in 14 sentinel lymph nodes (13 patients with melanoma and melanocytic lesions, and 1 patient with rhabdomyosarcoma). Conclusion We have found that lymphoscintigraphy with SLNB is an effective method to identify patients who may benefit from more extensive lymph-node dissection and to identify those patients who are unlikely to benefit from further lymph-node exploration.

Background In 1991, Delaitre reported the first laparoscopic splenectomy (LS). Since then LS has become the procedure of choice to treat hematological diseases requiring splenectomy. The Eastern province of Saudi Arabia is known to have a high incidence of hemoglobinopathies including sickle cell disease (SCD), which is known to be associated with complications necessitating splenectomy and/or cholecystectomy. This report describes our experience with LS and/or laparoscopic cholecystectomy (LC) for children with SCD. Patients and methods The medical records of all children with SCD who had LS and/or LC were retrospectively reviewed for age, sex, indication for splenectomy, operative time, hospital stay, and post-operative complications. The results were compared to a similar group of children with SCD who had open splenectomy (OS) and/or open cholecystectomy (OC). Results Over a period of 3.5 years (January 2005 and June 2008), a total of 45 children had LS with or without LC, 30 (66.7%) of them had SCD. Their age ranged from 2 to 12 years (mean 7 years). There were 16 males and 14 females. In all, LS was done because of recurrent splenic sequestration crisis except one who had a large spleen with multiple infarcts that was causing abdominal pain. The operative time ranged from 1.5 to 9 h (mean 2.75 h). Their hospital stay ranged from 3 to 9 days (mean 4.5 days). There was no mortality. Two patients (6.7%) required conversion to OS due to a large-sized spleen and severe adhesions in one and uncontrolled intra-operative bleeding in the other. The results were compared to a group of 120 children with SCD who had OS only (88) and OS with OC (32). From 1994 to 2006, a total of 55 children had LC only, 47 (26 M:21 F) of them (85.5%) had SCD. Their age ranged from 4 to 15 years (mean 11.4 years). The indications for cholecystectomy were: biliary dyspepsia (20), biliary colic (35), acute cholecystitis (5), obstructive jaundice (5), asymptomatic (6), and biliary pancreatitis (1). There was no mortality, but one (2.1%) required conversion to OC because of severe adhesions and another underwent postoperative exploration because of bleeding from an accessory cystic artery. The results were compared to a similar group of 27 children with SCD who underwent OC. Conclusions With good peri-operative management, LS is feasible and safe in children with SCD and can be done concomitantly with cholecystectomy. Currently, it requires more operative time than the open approach. This is specially so for children with SCD who are known to have a large spleen with severe adhesions. It is, however, superior to OS with regard to duration of hospital stay, cosmetic appearance, post-operative complications, and post-operative recovery. LC is also safe in children with SCD. When compared with OC, it is associated with less post-operative complications, a shorter hospital stay, better cosmetic appearance and a faster recovery.

This report describes a successful outcome in a preterm baby with an esophageal atresia and tracheo-esophageal fistula, who initially underwent a primary esophageal repair; but a persistent nonexpanding lung on the side of surgery led to further investigations. A further diagnosis of an esophageal lung resulted in pneumonectomy and prophylactic placement of an intra-thoracic prosthesis to prevent post-pneumonectomy syndrome. To the best of our knowledge, this is the first report of a prophylactic placement of an intra-thoracic prosthesis in a neonate with the condition of esophageal atresia and tracheo-esophageal fistula and associated esophageal lung.