Thymectomy in black children with juvenile myasthenia gravis

Fifteen black children with juvenile myasthenia gravis presented to our institution over a 10-year period at ages ranging from 18 months to 7 years, 4 males and 11 females. Twelve presented with progressive, generalised weakness and 3 had bulbar manifestations. Ocular signs were absent in 2 patients. All 15 patients were placed on pyridostigmine bromide (Mestinon) and 1 also required steroids and plasmapheresis. Medical therapy only was instituted in 5 patients, of whom 2 came to surgery, 2 died, and 1 was lost to follow-up. Thymectomy was performed in 12 children (including the 2 with failed medical therapy) via a median sternotomy with no mortality or morbidity related to the operation. The timing of surgery was less than 3 months in 75% of the children. Thymic hyperplasia was noted in 10 glands and 2 were reported as normal. Of the children in the operative group, 83% are on minimal medication or in total remission. Better results were noted in young patients with early thymectomy and diseased glands.     

Rectal adenocarcinoma following cranio-spinal radiotherapy for cerebellar medulloblastoma

The authors present a case of a 16-year-old female diagnosed with rectal adenocarcinoma 10 years after receiving cranio-spinal radiotherapy for a cerebellar medulloblastoma. While the risk of a second malignancy is recognised to be increased in children previously treated with radiotherapy, rectal adenocarcinoma is a rare presentation. A child presenting with symptoms of weight loss and a change in bowel habit in a patient who has previously received radiotherapy should alert practitioners to the possibility of a colorectal malignancy.     

Superior mediastinal teratoma containing well-differentiated bowel

A previously fit and well 14-month-old-girl presented with a 2-month history of worsening cough and wheeze. Chest radiograph revealed a widened mediastinum and thoracic CT showed a large mixed density mass in the superior mediastinum, consistent with a mediastinal teratoma. Her tumor markers were within the normal range. The mass was resected and found to be a mature cystic teratoma. Surprisingly, well-formed bowel-like structures were present, containing all bowel wall layers and having a ganglionated myenteric plexus. The identification of complete sections of bowel in this context is a rare finding and to the best of our knowledge has not been published previously for the mediastinal teratoma. The possibility of secondary neoplasia developing in these areas is a complicating factor.     

Challenges in developing paediatric surgery in Sub-Saharan Africa

The aim of the present study is to report on the difficulties in developing paediatric surgery in a teaching hospital in Tanzania. The methods are as follows: (1) information on the demography and health services of Tanzania were obtained from the Ministry of Health and the 1998 consensus report; (2) hospital data was obtained from the Medical Director’s Office and analysed; (3) the current delivery of surgical services for children at the Tumaini University Hospital and Kilimanjaro Christian Medical Centre (KCMC) is reported; (4) the local, national and international support for the development of surgical services for children is noted; (5) the teaching, training and research programmes are proposed. The results showed that (1) Tanzania has a population of 31 million with a total health expenditure of 10%. There is 1 doctor for 23,000 inhabitants and 1 hospital bed per 940. The infant mortality is 173 per thousand life births. (2) The bed capacity at KCMC is 500 with a staff of 40 consultants, 294 nurses, 246 health attendants and 38 clinical officers. (3) Forty-two percent of admissions to the surgical ward and 50% of surgical outpatients are children. Surgical newborns are cared for in the special care baby unit and there are no neonatal or paediatric ventilators. (4) Support to develop surgical services for children has been pledged for locally, nationally and internationally; however, delivery of the service has not had the equivalent momentum. (5) The proposed teaching and training programme has been approved by the University. There is a need for surgical services for children with encouraging support for this venture; however, the challenges remain in delivering the service.     

Lesser-curve ischaemic necrosis mimicking a gastric ulcer--a complication of highly selective vagotomy. A case report

Ischaemic necrosis of the lesser curve of the stomach is thought to be a specific but rare complication of highly selective vagotomy. While it commonly presents as a free perforation, it may also present as sloughing of the mucosa only, a gastric fistula or a sealed perforation of the lesser curve mimicking a chronic gastric ulcer. We describe a case in which ischaemic necrosis of the lesser curve presented as a chronic gastric ulcer from a sealed perforation.     

Blunt splenic trauma in children

Splenic trauma in 96 patients over an 11-year period is reviewed. Almost 80% of injuries resulted from motor vehicle accidents in which the patients was a pedestrian, and over 80% of patients sustained multiple injuries. Splenic injury was confirmed by isotope scanning at laparotomy. Non-operative management was feasible in 87.5% of the children, with no mortality or morbidity. In patients requiring surgery, splenic salvage was possible in 75%.     

Somatostatin for intractable postoperative chylothorax in a premature infant

A premature infant of 31 weeks gestation underwent repair of an oesophageal atresia, distal tracheo-oesophageal fistula and anal stenosis. A lymphatic leak was noted at the time of surgery. Chylous drainage persisted and an intravenous infusion of somatostatin was begun. The volume of chyle drained fell dramatically within the first 24 h and was negligible by the 5th day of treatment. No reaccumulation of the chylothorax was seen after the cessation of somatostatin. To our knowledge this is the youngest reported child in whom somatostatin has been used successfully in treating a postoperative chylothorax.     

Pre-sternal mass with a congenital sternal defect: a rare presentation

Anterior chest wall and anterior mediastinal infections are very rare in children with no predisposing thoracic surgery. Congenital sternal defects occur as a result of incomplete ossification of the sternal bone, and show a wide range of severity. We present the case of a 15-month-old boy who presented to the A&E department with an anterior chest-wall mass. After investigation, it was found to be an infective mass, with extension into the anterior mediastinum, through a previously undiagnosed 5 mm congenital sternal defect identified peri-operatively. After antibiotic therapy and surgical management the patient made complete recovery with no recurrence or complications within 6 months of review.     

Renal abscess in Papillion–Lefèvre syndrome

A 5-year-old female with Papillon–Lefèvre syndrome presented with renal mass. A radiological diagnosis of malignancy was made; however, partial nephrectomy revealed granulomatous disease indicative of chronic infection. Although liver abscess is an emerging complication in patient with Papillon–Lefèvre syndrome, this case represents the first renal abscess described in such patients.