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A patient with Down’s syndrome, with dermatofibrosarcoma protuberans, was intended for adjuvant radiotherapy. The lesion was on the parietal region of the head of the patient. Given the proximity of the lesion to the brain, the curvature of the lesion, and potential complications of anaesthesia for a Down’s syndrome patient, brachytherapy was the appropriate treatment. Anaesthesia complications for patients with Down’s syndrome are airway infections, atlanto-occipital dislocation and bradycardia.
Method:
Instead of sedating the patient in order to prepare a mould applicator, a 3D-printed model of the patient’s head was used. This allowed us greater time to prepare the applicator in a more relaxed environment.
Result:
The fit of the mould applicator on the patient was satisfactory. Minimum air gaps were observed. The treatment could be completed with sedation only.
Conclusion:
We were able to achieve an equivalent dose of 44·69 Gy in 5 sessions of brachytherapy, significantly reducing the anaesthesia sessions and the associated risks. A drawback of 3D printing is that it takes several hours to print the model.
To describe the cardiac lesions seen in children with trisomy 21, the outcome of these children and rates of access to corrective surgery at the Bustamante Hospital for Children.
Methods
A 10-year retrospective review of the records of trisomy 21 patients with cardiac lesions referred to the Bustamante Hospital for Children was conducted.
Results
A total of 76 patients were enrolled in the study, 30 (40%) males and 46 (60%) females; among these 110 cardiac lesions were detected. A total of 20 (26%) patients died, 48 (63%) survived, and for 8 (11%) the status was unknown. The most common lesion was the atrioventricular septal defect, which accounted for 41 (37%) of all the lesions and occurred as a single defect in 24 (53%) patients. At presentation, 33 (46%) patients had one or more medical complication; 30 (91%) had cardiac failure, 10 (30%) had pneumonia and 5 (15%) had evidence of systemic pulmonary arterial pressures. Cardiac catheterisation was recommended for 43 (56%) patients but only 10 (23%) had the procedure done. Surgery was recommended for 60 (79%) patients; of these 6 (10%) patients had the procedure done. The median time of survival was 88 months (7.3 years). The age of presentation was not found to significantly affect outcome.
Conclusion
Trisomy 21 patients with cardiac lesions have high morbidity and mortality. This morbidity and mortality could be reduced if surgical intervention was offered routinely.
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