Journal of Medical Science and Technology

1 – 6.

Authors: Jolanta Walasz, Teresa Matthews-Brzozowska, Maja Matthews-Kozanecka, Dorota Cudziło

General muscle hypotonia, including hypotonia of the oro-facial muscle complex, is one of the characteristics of Down syndrome. To eliminate this disorder, oro-facial therapy with the use of a palatal plate is employed. The aim of this study is to assess the influence of the types and positioning of stimulation elements in palatal plates on the tongue position and mimetic muscles in children with Down syndrome. The study was conducted on 50 children with Down syndrome between the ages of 3 months and 2 years (22 boys, 28 girls) who were treated by means of palatal plate therapy in the Department of Maxillofacial Orthopaedics and Orthodontics at the Poznan University of Medical Sciences. The study analysed the responses received from the parents and the orthodontist to the questions included in a questionnaire developed for this study as well as the photographic documentation produced during every appointment over a period of two years. In total, 90 palatal plates were made for the children with stimulation elements in the form of a cylinder with a "roller" or a movable bead. The plates were replaced on average every 6 months in order not to inhibit the growth of the jaw. The study found that in 50 children who underwent palatal plate oro-facial therapy there was an improvement in the visual appearance of the mimetic muscles and tongue retraction, as well as improved lip closure. None of the children experienced deterioration in their condition. The stimulator in the form of a cylinder with a "roller" had a greater influence as regards improvements in the appearance of the mimetic muscles and lip closure, whereas a movable bead threaded on a wire and secured to the plate produced a greater improvement as regards tongue retraction. The positioning of the stimulation element closer to the alveolar ridges rendered better therapeutic results in the youngest children, whereas placing it in a central or posterior position produced better results in the older children.

10 – 12

Authors: Eyad Safadi, Aser Hrout, Zahi Majali, Nisreen Alkurdi, Sana'a Sulieman Al-Ali

Objective is to evaluate our clinical experience in dealing with recurrent respiratory papillomatosis (RRP) at Queen Rania Al-Abdullah Hospital for children. A total number of 9 patients (8 males, one female) were diagnosed by means of history; physical examination and flexible nasopharyngolaryngoscope .Patients age at time of presentation were 2-10 years. The presenting symptom was just hoarseness of voice in 5 patients (group 1). Hoarseness and dyspnoea on exertion in 3 patients (group 2) and respiratory distress in only one patient (group 3). All 9 patients had been treated by microdebrider excision using apnoic technique. In 3 of patients (group 2) we injected MMR vaccine intralesional and we injected sometimes MMR vaccine and sometimes Cidofovir intralesional in only the most severe case (group 3). In group 1 and 2 the post operative symptom free period was 6-8 months. In group 3 the symptom free period was 4-5 weeks when MMR was injected and 10-12 weeks when Cidofovir was injected. Recurrent Respiratory Papillomatosis (RRP) is a benign neoplasm caused by HPV, it affects mainly children, it has high recurrence rate with remissions. Best results are achieved using microdebrider and Cidofovir intralesional injection.

19 – 23.

Authors: Najeh Alomari, Asma Aref Idamat

Gastroesophageal reflux disease (GERD) is common in pediatric age group due to incomplete maturation of the antireflux mechanism. Vomiting and regurgitation are frequent manifestations in pediatric age group and are important for consideration when they adversely affect their nutritional status and recurrent chest infection due to aspiration. Medical treatment may not control (GERD), so surgical treatment is the remaining choice to control GERD and late complications. In this study, we present our experience in laparoscopic Nissen fundoplication for the management of (GERD) in children over 5 year period, acceptability, safety, efficacy and outcome. The data of all patients undergoing fundoplication for (GERD) at Queen Rania Hospital for Children / King Hussein Medical Center were retrospectively reviewed of prospectively collected data from April 2008 to 2013. Ages ranged from 3 months to 15 years. All laparoscopic fundoplications were performed by a single surgeon. Patients were evaluate for the indications for surgery, diagnostic modalities, duration of surgery, peroperative complications, pain management , hospital stay , post operative course, morbidity, mortality and the need for re operation. We used the Enseal vessel sealing device and hook diathermy through 5 and 3 mm ports for dissection and division of short gastric vessels and excision of the hernia sac. Over the last 5 years, 80 laparoscopic Nissen fundoplication for GERD were performed, 12 patients had GERD associated with thoracic stomach, 3 patients had esophageal achalasia underwent cardiomyotomy & fundoplication, 14 patients had neurological impairment. Weight ranged from 3, 5 to 82 kg. Mean age 5 years, range (3 months – 15 years), 50 males and 30 females. The indications for surgery were thoracic stomach and Barret esophagus due to (GERD), other indications were severe esophageal ulceration, stricture, recurrent bleeding, para-esophageal hernia and recurrent aspiration pneumonia. Five patients had previous repair of esophageal atresia. Mean operating time was 120 min (range 45 -240 minutes). The mean hospital stay was 2 days, range from 1 to 4 days. There were no intra-operative and post-operative complications. One conversion to open fundoplication was required due to technical fault of the CO2 insufflator. Six patients had laparoscopic gastrostomy insertion in addition to Nissen fundoplication. Blood transfusion was not required in any case and no mortality. There was no need for re do surgery. The vast majority of patients showed significant respiratory improvement as well as control of emesis and hematemesis. This study shows that laparoscopic Nissen fundoplication for GERD in children is rapidly becoming the procedure of choice for surgical correction because of the advantages of reduced discomfort and decreased hospitalization. It is feasible, effective and safe technique. Laparoscopic Nissen fundoplication operation times in children reduced by experience. The surgical skills are important to improved outcomes include adequate length of intraabdominal esophagus, minimal hiatal dissection and tension-free wrap. The results are superior to the traditional open fundoplication. Laparoscopic Nissen fundoplication should be considered the gold standard for anti reflux procedures. The length of hospital stay and convalescence is short and hence rapid return to normal activity is expected with less analgesia requirements. Follow-up examination verified perfect clinical, radiological and endoscopic findings. The cosmetic, endoscopic and functional results were excellent with very good patients and family satisfaction.

30 – 42

Authors: Anthony Kodzo-Grey Venyo

Primary angiosarcomas of the urinary bladder are very rare in view of this their clinical and pathological features have not been extensively described. Literature on primary angiosarcoma of the urinary bladder was reviewed performing an extensive search using various internet search engines for case reports, case series and review papers on primary angiosarcoma of the urinary bladder excluding metastatic angiosarcomas. Primary angiosarcomas of the bladder are rare tumours which are more commonly found in men and quite often present with haematuria, cystoscopy reveals raised lesions in the trigone / dome. Microscopic examination of the tumour tends to show anastomosing vascular channels lined by plump hyperchromatic cells as well as solid and epitheliod cytology. Typically the tumour is locally invasive invading the muscularis propria. The tumour stains positively with CD31, CD34 and can be focally positive especially in cases of epitheliod tumours. Few cases have so far been reported and on the whole previous reports have indicated poor prognosis. However, recent anecdotal reports have indicated that multi-modality treatment with radical cystectomy and adjuvant combination chemotherapy and radiotherapy may result in long term survival. Primary angiosarcomas of the urinary bladder tend to affect middle-aged and elderly men. Anecdotal reports have indicated longer term survival with multi-dal treatment strategy unlike previous reports which on the whole indicated poor prognosis. There is no consensus opinion regarding their treatment. There is therefore the need for a multi- centre trial to determine the best treatment options that would improve the prognosis for these tumours.