Rapunzel syndrome is a rare presentation of a trichobezoar, with 24 cases having been reported in literature so far. This syndrome has been reported in 23 female and 1 male patient with a mean age of 10.8 years. The patients commonly present with abdominal pain, nausea, vomiting and signs of obstruction. The distal end of the bezoar may be in the jejunum, ileum or the colon. We evaluate the various cases reported and discuss the various modes of presentation of this uncommon syndrome, and also present a series of 3 cases, all females, aged 16, 18 and 21 years, and had a trichobezoar in the stomach with a tail extending into the ileum.
Of 258 cases of dacryocystorhinostomy performed on children in the period September 1981 to September 1991, 130 were for simple, unresolved congenital nasolacrimal duct obstruction. Other indications for surgery included punctal agenesis, lacrimal fistula, post-traumatic and post-inflammatory canalicular obstruction. Of 177 children without canalicular pathology, 171 (96%) were relieved of symptoms with one operation, without canalicular intubation. Of 81 cases with canalicular disease, 55 of 70 (79%) who underwent DCR plus canalicular intubation, and 10 of 11 who underwent DCR plus Lester-Jones tube, were substantially improved with one operation. No child required peroperative or postoperative blood transfusion. Dacryocystorhinostomy in childhood, in experienced surgical hands, is a safe procedure, achieving relief of symptoms in most cases, particularly in the absence of canalicular disease.
Therefore, it becomes important for the right handed surgeons to modify their techniques and establish a proper hand eye coordination to adapt to the mirror image anatomy of the Calot's triangle in a patient of situs inversus totalis.
Medullary thyroid carcinoma (MTC) is associated with amyloid deposition in the surrounding tissues. MTC-positive tumor thyroid tissues surgically removed from patients were used in our study to extract amyloid. We tested the MTC extracts for the presence of amyloid by measuring fold enhancement of thioflavin T fluorescence. Transmission electron microscopic study and atomic force microscopy of MTC patient extracts revealed typical amyloid fibrils. Matrix-assisted laser desorption ionization-time of flight mass spectrometric analysis demonstrated full-length calcitonin as the constituent of the MTC amyloid from seven patients. Our results unequivocally demonstrated that full-length calcitonin is the sole constituent of amyloid in MTC.
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