Abstract: Introduction: Bernard–Soulier syndrome is a rare autosomal recessive disease that causes a deficiency of glycoprotein Ib, the receptor for von Willebrand factor, which is important for clot formation. It is estimated to occur in fewer than 1 per 1 million persons. The differential diagnosis includes von Willebrand disease, immune thrombocytopenic purpura, May–Hegglin anomaly, thrombocytopenia-absent radius syndrome, grey platelet syndrome, and other inherited giant platelet disorders. Case Presentation: A 30-year-old Turkish woman was admitted to the Department of Heamatology for evaluation of thrombocytopenia. Because she had repeated epistaxis during admission, she was assessed to evaluate for haemorrhagic diathesis. She had been diagnosed with immune thrombocytopenic purpura and given steroid therapy at different times. Peripheral blood smear was characterized by neutrophils 75%, lymphocytes 20%, monocytes 5%, giant platelets and platelets forming three to four clusters, a normal red blood cell morphology. A decreased ristocetin-induced platelet aggregation was detected in low and high concentration. On the basis of these findings, Bernard–Soulier syndrome was screened with flow cytometry and genetic mutation. CD41a 86.2%, CD42a 92.9%, CD42b 92.5%, and CD61 87.8% were detected in flow cytometry. Normal platelet GPIb/IX levels by flow cytometry turned down the suspected diagnosis. genetic analysis, we have applied to our patient and her parents. We detected same mutation in patient and her mother. Laboratuar parameters and flow cytometry of mother did not support diagnosis of Bernard–Soulier syndrome. Conclusion: Bernard–Soulier syndrome should be considered before a young patient is diagnosed with immune thrombocytopenia purpura and peripheral blood smear should be examined for giant platelets.

Abstract: Congenital nasopharyngeal teratoma or epignathus is a very rare congenital neoplasms, it may be mature, immature or malignant. We report the case of a 04-day old female neonate, with a teratoma of the nasopharyngeal area. Examination showed a polypoid mass protruding from the nasopharynx measuring 3 x 2 x 2 cm, covered on one side with numerous fine and coarse hair. Surgical excision of the mass was performed under general anesthesia on day 4 after birth. Pathology revealed a well differentiated mature teratoma. The patient had no complication in the postoperative period.

Abstract: Moyamoya (meaning a hazy puff of smoke) disease is a rare, idiopathic, persistent, occlusive cerebrovascular disease involving bilateral progressive stenosis or occlusion of a terminal portion of the internal carotid artery, or a proximal portion of the anterior cerebral arteries and the middle cerebral arteries. There are irregular perforating vascular networks (moyamoya vessels), seen in the base of the brain, which produce magnetic resonance images of this ‘puff of smoke’ condition. The association of moyamoya disease and congenital heart disease such as Coarctation of aorta, ventricular septal defect and tetralogy of fallot has been previously reported. Here we report a case of 15 yr old female child presented with seizure episode and was diagnosed as having moyamoya disease with ebstein anomaly. To the best of our knowledge this is the first case report of this co-occurrence of moyamoya disease with ebstein anomaly.

Abstract: Submitral aneurysm is a congenital out pouching of the left ventricular wall invariably occurring adjacent to the posterior mitral leaflet. This rare congenital cardiac anomaly was initially described among the African population and is considered rare in the other parts of world. The clinical picture is dominated by congestive cardiac failure in the presence of mitral regurgitation. Echocardiography provides precise non-invasive diagnosis. The literature is reviewed to increase the awareness of clinicians, especially novice echocardiographers about this rare cardiac anomaly when coming across congestive cardiac failure with mitral regurgitation in young population.

Abstract: POEMS disease is a rare paraneoplastic syndrome resulting from an underlying plasma cell disorder. We present a unique case of POEMS disease with predominant clinical symptoms of diarrhea and paresthesias.

Abstract: Skeletal abnormalities of cervical region or in craniocervical region are of interest to the anatomists, orthopaedicians, neurologists, neurosurgeons and even orthodontists. These abnormalities may result in severe neck pain, decreased neck mobility, muscular weakness and sensory deficits of both upper limbs and sudden unexpected death. During the routine osteology teaching, it was observed that the axis vertebra is fused with the third cervical vertebra. It was observed that body, laminae and pedicles of C2 and C3 were completely fused on both anterior and posterior aspects. The features of these fused cervical vertebrae were analysed and the specimen was photographed from different aspects. This is a condition of block vertebra which has embryological importance and clinical implications.

Abstract: 2 cases of Gossypiboma also called Textiloma or Gauzeoma are presented with the attendant management challenges. One of them presented with an intra peritoneal abscess and a fistula necessitating a 2 staged bowel resection and colostomy then closure while the other simulated a complete intra luminal colonic tumor with sub acute intestinal obstruction. Despite the serious medico-legal and psychological issues involved the patients were duly informed. The doctors that did the previous surgeries were also informed. The surgical team is advised to be meticulous with swab counts in order to avoid its occurrence and when it does a high index of suspicion is required for early diagnosis and management. The policy of prevention coupled with use of several adjunct technologies which accounts for sponge use will help to reduce the incidence of gossypibomas. These strategies to avoid its occurrence are also discussed.

Abstract: Aims: To isolate and identify the causative agents for chronic osteomyelitis in patients and understanding their virulence factors for identification of targets for novel drugs and design of new vaccines. Study Design: samples of swab and bone tissues biopsy from chronic osteomylitis patients were collected in order to study the infectious agents by investigating their virulence factors. Place and Duration of study: Twenty five specimens of osteomyelitis were collected from chronic osteomyelitis patients at Surgical Specialist Hospital, Al-Yarmook Teaching Hospital and Baghdad Teaching Hospital during June 2010 to May 2011. Methodology: a form was designed to be filled by each patient, which included information about their age, sex, duration of infection, infection site. Specimens were collected using a sterile cotton swab and bunch of biopsy. The pus was taken before the surgery by swabbing an open infected area and from the deeper part of the infected bone, while, biopsies were taken from osteomyelitis patients during surgical management at the operation theater. Results: from fifty swab and twenty-five bone tissue biopsy samples, the results showed high prevalence of osteomyelitis in male patients with 84% (21), and 16% (4) in females. It was found that chronic osteomyelitis has a high prevalence among males with 84%, and the highest incidence was recorded at the age group of 30-39 years. Cultural, microscopic examination and bioc-hemical characterization of both swab and biopsy specimens, showed that 50% of the isolates were Staphylococcus aureus, 26% Enterobacter cloacae, 14% Pseudomonas aeruginosa, 6% Escherichia coli, and 4% Klebseilla sp. From the results of the antimicrobial susceptibility test using different (11) antibiotic discs against S. aureus, Enterobacter cloacae, Pseudomonas aeruginosa, E.coli, and Klebsiella sp. six isolates of S. aureus were selected and designated as (S1, S3, S4, S7) which showed multiple resistances to antibiotics. Conclusion: It was found that multiple resistances S. aureus isolates were able to produce haemolysin enzyme, capsules, slime layer, biofilm and hyaluronidase enzyme.

Abstract: Stomach injury is one of the most severe types of injuries and among severe injuries of piece abdominal injuries makes 25%. Post traumatic stomach ruptures make 1% of abdominal injuries in isolated abdominal traumas and up to 6% in combined. During 1999-2012 periods we have analyzed 57 observations, of them 12 isolated and 45 combined gastric injuries. Injuries of anterior gastric wall were revealed in 42 patients, of posterior wall in 4 persons with thoracoabdominal wounds and of both walls in 11 patients. Study proved some different surgical treatment tactics of stomach injury have been presented. Complications in the postoperative period developed in 9 (15, 8%) patients with ruptures in all layers of the stomach. Relaparatomy became necessary in 5 patients due to the following indications: ileus (2), pancreatitis (1), bleeding to gastric lumen (1) insolvency of stomach wound sutures. 3 of 5 reoperative patients died, all of them had combined traumas; 2 mortality of shock and blood loss, 1 from pancreatitis and peritonitis. An adequate intervention volume in gastric injuries appears to be two-layer suturing of the wound with previous ligating of bleeding vessels and following decompression of the organ through nasogastral probe.

Abstract: This rare sporadic disease is named after the Greek god Proteus who could change shape. The syndrome has protean manifestations that include partial gigantism of the hands and feet, cerebriform plantar hyperplasia, hemangiomas, lipomas, lipohypoplasia, linear verrucous epidermal nevi, patchy dermal hypoplasia, macrocephaly, hyperostosis, muscular hypoplasia, and hypertrophy of the long bones.