Laparoscopic management of Rhabdomyosarcoma of common Bile duct, Case report.

INTRODUCTION: Embryonal Rhabdomyosarcoma (ERMS) is a malignant soft tissue musculoskeletal tumor which constitutes about 0.06% of all malignancies affecting children. Biliary tract ERMS is still rare, though it is considered the most common cause of malignant obstructive jaundice in children. CASE PRESENTATION: A report of a 2-year-old boy, who was presented with recurrent episodes of scleral icterus of three months duration, is added to the related literature. His labs went with obstructive jaundice and the radiological investigations were consistent with a diagnosis of choledochal cyst. The found mass was suspected to be an ERMS of common bile duct and turned out to be so by the histopathology. He was managed totally by laparoscope, both excision and hepaticojejunostomy reconstruction, which is an extremely uncommon entity. CONCLUSION: Common Bile Duct Rhabdomyosarcoma is rare and diagnosis at this anatomical site is difficult. Our case highlights the feasibility of laparoscopic resection and hepaticojejunostomy reconstruction with very good results at 16-month follow up and parents' gratitude as well.

Congenital massive hiatus hernia type IV; initial experience with laparoscopic repair in young infant.

Congenital massive hiatus hernia (CMHH) is an uncommon disorder during childhood. It can be associated with grave complications especially if presented in the highest grade; type IV, when the hernia contains other intra-peritoneal organ beside the stomach through a large hiatus defect. The insidious form of clinical presentation can be deceptive in diagnosis and may mimic congenital diaphragmatic hernia or other chest pathologies. The basic principle of surgical repair is to reduce the herniated organs, excise the hernia sac, and repair the crural defect and to add anti-reflux procedure with or without gastropexy. Traditionally, this has been done by open approach. Nowadays, the minimally invasive approach is the preferred method of treatment. A sixteen-month-old boy with history of recurrent respiratory symptoms was diagnosed with CMHH type IV for which laparoscopic repair was performed. Few reports in using minimally invasive technique in the management of CMHH in the pediatric age group are present in the literature, to the best of our knowledge type IV had never been described in young infants. We present a new case repaired by laparoscope in a young infant with CMHH type IV from the Middle East.

Thoracic wall reconstruction for primary malignancies in children: short- and long-term results.

AIM: Primary thoracic wall malignancy is a rare and diverse entity in children. Surgical treatment commonly involves major chest wall resection causing large defects requiring complex reconstruction. In adults, the use of alloplastic and/or xenogenic materials and muscle flap repair is well established. However, literature provides only little information on procedures in children. We report our experience in 8 consecutive children who underwent chest wall resection and reconstruction with regard to surgical treatment and outcome. PATIENTS AND METHODS: Retrospective study of all children with primary malignant chest wall tumors requiring rib resection and reconstruction with prosthetic material performed in our institution between November 2002 and April 2010. Endpoints were postoperative complications and long-term results, focusing on scoliosis defined radiologically by the Cobb angle. RESULTS: 8 children (7 male, 1 female) with a median age of 10.6 (4.1-18.9) years underwent resection of thoracic wall tumors. A mean number of 3 (1-5) ribs were resected. Stability was obtained using rigid prosthetic material (STRATOS™ titanium bar) in 2 patients and/or non-rigid prosthetic material (Goretex® patch in 6 patients, Vicryl® patch in 3 patients, Tutopatch® in 1 patient). A muscular flap was added in 5 patients. Postoperative complications included superficial wound infection (n = 2) and dislocation of a titanium bar necessitating removal in 1 patient. No infections of the prosthetic material were observed. No perioperative mortality occurred. At a mean follow-up of 37.5 (4-97) months, 6 patients were alive. 2 patients died due to early tumor recurrence. Mild scoliosis (Cobb angle 10-20°) was detected in 2 of the surviving patients (33%). CONCLUSION: Surgical reconstruction after resection of malignant thoracic wall tumors using non-rigid prosthetic material is safe and effective in pediatric patients, whereas rigid prosthetic material might dislocate. Scoliosis represents a long-term complication after chest wall reconstruction and should be monitored during routine follow-up.

Our experience with single lung ventilation in thoracoscopic paediatric surgery.

INTRODUCTION: Data on the feasibility and effects of single lung ventilation (SLV) in children are scarce. We conducted a retrospective study on the feasibility of SLV during video-assisted thoracoscopic surgery (VATS) in children and adolescents undergoing major thoracic procedures. METHODS: A retrospective chart review of all records from patients who underwent VATS at our institution from 2000 to 2010 was done. Patients receiving SLV were analysed in detail. Endpoints of the analysis were conversion to open thoracotomy (frequency and reasons), postoperative duration of ventilation, and pulmonary complications such as radiologically confirmed atelectasis and pneumonia. RESULTS: 74 out of 305 patients (24%, 43 boys, 31 girls) with a mean age of 9.4 years (56 days-18 years) and mean weight of 34 kg (4.5-76 kg) had SLV. Lung resection was done in 43 (58%), pleural surgery in 17 (23%), a combination of both in 7 (9%), and mediastinal procedures in 7 (9%). 11 patients (15%) required conversion of VATS to open surgery, mostly because of problems with exposure of the operative field (73%). 32 patients (43%) were extubated immediately after the operation, whereas 8 (11%) required ventilation for more than 24 h. The mean intensive care unit stay was 1.6 days. 18 patients (24%) developed radiologically confirmed atelectasis, and 1 patient (1%) required bronchoscopic clearance. Pneumonia occurred in 1 case (1%) and was successfully treated with antibiotics. CONCLUSION: SLV is feasible in children and adolescents undergoing VATS for a broad spectrum of procedures. However, despite SLV, the conversion rate in our series was 15%. The main reason for conversion was problems with exposure of the operative field. The complication rate for SLV was low. Atelectasis developed in every fourth patient but usually resolved spontaneously, and intervention to achieve ventilation was rarely indicated.

A complex variation in the superficial palmar arch.

In this article we describe a unique and complex variation in the arterial pattern of the left hand of a female cadaver. The following variations were found in this case: a) persistent median artery of the palmar type, terminating in the hand as the princeps pollicis and radialis indicis arteries; b) the ulnar artery giving only two common palmar digital arteries; c) the second digital palmar artery without division into two digital branches and instead supplying only the radial side of the ring finger; d) absence of the first common digital artery with the contiguous sides of the second web space supplied by the first palmar metacarpal artery from the deep palmar arch; e) early bifurcation of the median nerve proximal to the flexor retinaculum.

Nuclear zinc in the three lobes of the rat prostate gland.

The prostate gland concentrates Zn more than any organ in the body and Zn is increased and decreased in benign prostatic hyperplastic and adenocarcinoma of the prostate, respectively. Zn is a key structure in zinc finger proteins and hence is involved in cell proliferation and differentiation. The role of Zn may be attributed to a zinc binding protein, metallothionein (MT), which can be induced by certain metals, e.g. Cd. Induced MT may be involved in Zn metabolism and transport in the prostate gland. X-ray microanalysis was used to detect quantitatively the Zn in the nuclei of the glandular epithelium of the gland. It was found that Zn concentration was elevated and reduced in the lateral and dorsal lobes, respectively, after Zn treatment, but the Zn concentration was elevated in the lateral lobe after cadmium treatment. The ventral lobe did not show any substantial change in Zn concentration after either treatment. These results suggest that the three lobes of the gland respond differently to treatment. The variations in the response may reflect different mechanisms of Zn transport and metabolism in the three lobes of the rat prostate. Binding Zn to MT may indicate the involvement of MT in the metabolism and transport of zinc, an effect which may be modified by treatment.

Accumulation of the proteolytic marker peptide ubiquitin in the trophoblast of mammalian blastocysts.

Ubiquitination is a universal protein degradation pathway in which the molecules of 8.5-kDa proteolytic peptide ubiquitin are covalently attached to the epsilon-amino group of the substrate's lysine residues. Little is known about the importance of this highly conserved mechanism for protein recycling in mammalian gametogenesis and fertilization. The data obtained by the students and faculty of the international training course Window to the Zygote 2000 demonstrate the accumulation of ubiquitin-cross-reactive structures in the trophoblast, but not in the inner cell mass of the expanding bovine and mouse blastocysts. This observation suggests that a major burst of ubiquitin-dependent proteolysis occurs in the trophoblast of mammalian peri-implantation embryos. This event may be important for the success of blastocyst hatching, differentiation of embryonic stem cells into soma and germ line, and/or implantation in both naturally conceived and reconstructed mammalian embryos.

Immunocytochemical localization of metallothionein in the rat prostate gland.

Metallothionein (MT), which binds zinc and other metals, was localized within the rat prostatic complex at light and electron microscopic levels utilizing immunocytochemistry. Two groups of mature, male Sprague-Dawley rats were injected with cadmium chloride subcutaneously (2 mg/kg body weight) to induce the synthesis of MT, and were sacrificed 48 h postinjection. From the first group, prostatic tissue from the three lobes was prepared for light microscopy. The indirect peroxidase antiperoxidase procedure was used for MT localization using rabbit anti-MT as the primary antibody. The three lobes of the rat prostate demonstrated a positive reaction for MT, but among the lobes variations in the intensity and localization of the reaction product occurred. Tissues from the second group of animals were prepared for electron microscopy. Using the indirect immunogold labeling technique with anti-MT, MT localization was confirmed in the rough endoplasmic reticulum, secretory vesicles, secretory products, and subepithelial connective tissue. These localizations suggest that MT binds zinc both intra- and extracellularly, where it may function in zinc storage and metabolism.