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August 1992, Volume 42, Issue 8

Review Articles


Fatema Jawad  ( 7/6, Rimpa Plaza, M. A. Jinnah Road, Karachi. )

LAGYNGEAL ANGIOMYOMA. Esmer, N., Uyar, Y., Ekinci, C. Pak.J. Otolaryngo!., 1991;7:26-28.
The case of a 28 years old young man diagnosed as laryngeal angiomyoma is presented. The patient came in with a history of difficulty in swallowing of one year duration. Hoarseness and dyspnoea developed since 2 months. lie was hospitalized and underwent immediate tracheotomy due to the breathing distress. indirect laryngoscopy revealed a small greyish white mass lined with smooth mucosa nearly obstructing the laryngeal lumen. The left arytenoid was fixed. There was no cervical lymphadenopathy. Clinical examination, laboratory studies and x-rays revealed no abnormalities. His­topathology of the biopsy material taken from the laryngeal mass gave a diagnosis of angiomyoma. Micro ­laryngoscopy performed under general anaesthesia revealed the mass to be attached to the left ventricle. It was excised, fixed and stained Squamous epithelium lined the mass. There were numerous vascular structures and smooth muscle with the nuclei showing motosis. The patient was decannulated after 4 weeks. No recurrence has been observed in 5 years follow-up; Angiomyoma or vascular leiortiyoma is usually solitary, occurs in old age and very rarely in the larynx. These tumours are slow growing encapsulated and develop from smooth muscle tissues containing numerous vessels having no internal and external elastic layers. Treatment of the tumours is surgical. Bleeding is always encountered and can be controlled by cauteriza­tion and pressure. In the past decade laser surgery of the larynx is used as a choice as it provides haemostasis. Prognosis is good and no recurrence has been reported.
TOXIC EFFECTS OF CISPLATIN ON OLFACTION. Son!, N.K., Bajjaj, ILK. Pak.J.Otolaryngol., 199 1;7:23-2 5.
Thirty patients with advanced malignancy and on treatment with cisplatin were assessed for impairment of olfaction. Cisplatin, a cytotoxic drug with potent anti-neoplastic activity is potentially toxic too. Side effects as nephrotoxicity, bone marrow, depression and gastrointestinal disorders are prominent features. Distur­bances of special senses as hearing and vision have been reported. As one case developed olfactory changes during cisplatin therapy, the study was undertaken to assess this complication too. The blast inhalation method was used to determine the grade of olfactory impairment in the 30 cases on cisplatin therapy. Six test bottles containing coffee, menthol, methylsalicylate, turpentine oil, amylacetate and ammonia were used alongwith 2 control bottles containing water. Every bottle was completely masked. Each patient was asked to close one nostril and with the other to inhale with a forceful blast. The procedure was repeated if the patient failed to recognise the odour and if after the thircL attempt there was no success it was classed as complete anosmia. The test was done first before starting chemotherapy and then weekly and those patients who had impairment were followed-up for 9 months. 30 normal individuals were also studied in the sameen Vironment as controls. Nine cases in all showed some degree of anosmia with one having complete loss of smell. Cisplatin after intravenous administration binds with DNA and causes fragmentation of its molecules. This proves that atrophy of olfactory receptors is one cause of the anosmia. Olfactory nerve involvement is another probable cause leading to complete anosmia. Impairment of smell usually occurs 4 to 5 weeks after cisplatin therapy and olfactory activity returns to normal in 10 to 12 weeks after stopping the drug in mold cases only. The study proved cisplatin to be an olfacto­toxic drug. More work on this aspect is however necessary.
HAND-SCHULLER-CHRISTIAN DISEASE - A CASE REPORT. Anwar, M., Khan, M.M., Asif T., Saleem, M., Khan,A.H. and Ahmad, M. Rawal Med. J., 1991;19:40-43.
The case of a 3 years old baby girl diagnosed as hand-schuller- christian disease is presented. This is one of three diseases grouped under histiocytosis. The patient was brought in with bilateral painful swelling in the temporal region of the skull, intermittent fever, weakness and pallor. The swellings were tender and proptosis of the right eye was present. Osteolytic lesions of the skull were noted on the x- ray and a scan showed erosion of the skull bones due to a probable brain tumour. There was moderate anaemia with slight leucocytosis. No hepatosplenomegaly or lym­phadenopathy was noted. Repeat x-ray of the skull after a few months showed multiple map- like rarefied areas of bone destruction involving multiple areas in the skull. The left mandible gave an appearance of floating teeth in the premolar region. The peripheral blood film revealed microcytosis, hypochromia, anisocytosis, poikilocytosis and mild thrombocytosis. Bone marrow aspiration showed proliferation of large- mononuclear cells with eccentric nuclei and some haemophagocytic activity. Histopathol­ogy of the local skull lesion showed proliferation of mature histiocytes with foamy cytoplasm and indented nucleuli. Some exhibited erythrophagocytosis. Multi-nucleated touton giant cells, focal aggregates of lym­phocytes and focal areas of haemorrhages and necrosis were also present. Cholesterol clefts were seen. All the findings together gave a diagnosis of hand­schuller- christian disease. Urine specific gravity was normal which ruled out diabetes insipidus. Prednisolone 25 mg daily was started which showed marked improvement by reducing the swellings. The proptosis regressed and general health improved. Histiocytosis-X includes hand-schuller-christian disease, letterer-siewe disease and eosinophilic granuloma. Males are affected twice as much as females. ­The hand-schuller-christian disease usually presents as a triad by lytic skull lesions, cxophthalmos and diabetes insipidus. The jaws are involved due to deposits pf foamy histiocytes to sore, swollen, necrotic gingivae and loose teeth. The prognosis is good in FISCD with resolution of the lesions either spontaneously or by chemotherapy. Drugs used are corticosteroids and vincristin given alone or combined.
OCULAR ADNEXAL LYMPHOMA - CASE REPORt Ahmed, M., Zafar, T., Khan, A., Saeed, S. and Ahmed, M. Rawal Med. J., 1991;19:46-48.
The case of a 32 years old male patient with bilateral upper eyelid swelling since 5 months and diagnosed as malignant lymphoma is presented. Local examination revealed masses in the upper lids which were rubbery and mobile with no proptosis. A biopsy done was reported as malignant lymphoma, diffuse large cell type. Physical examination revealed an enlarged firm right sub mandibular gland, the histopathology of which was again diffuse large cell type of lymphoma. The bone marrow trephine biopsy showed an atypical infiltrate combination therapy with cyclophosphamide, adria­mycin, vincristine, prednisolone alongwith radiotherapy was administered which lead to regression of eyelid swelling. Lymphoma rarely presents as an orbital or adnexal lesion. It has to be differentiated from reactive or inflammatory tumours which is done by histopathology, immuno-histochemicál and surface marker studies. Cytochemistry and electromicroscopy are helpful in differentiating lymphoma from granulocytic sarcoma, melanotic melanoma and undifferentiated carcinoma. Most of the malignant lymphoid lesions arc non­ Hodgkins lymphomas. 75 percent of the patients already have systemic involvement at the time of presentation. Irradiation is the treatment of choice for localised lesions and chemotherapy is given if systemic lymphoma is identified. Rapid relief is had in - combining the two therapies.

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