Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0262471 (ENT)
 5,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node biopsy produced a histological diagnosis compatible with Rosai-Dorfman disease. Later he developed an acute red proptotic eye. He had severe proptosis due to an enlarged lacrimal gland. He refused surgical excision of the tumour, which is suggested if there is an ocular adnexal involvement. Conservative treatment with systemic steroid resulted in the resolution of lacrimal gland swelling, nasal sinusal mucosal thickening and cervical lymphadenopathy. Previous studies have shown that patients with Rosai-Dorfman syndrome are often black males1 and require surgery.
Orbit 2001 Sep
PMID:Rosai-Dorfman syndrome affecting the lacrimal gland. 1204 17

The aim of this study was to illustrate the surgical techniques and utility of stereotactic or image-guided navigation in the management of lacrimal drainage obstruction in congenital arhinia-microphtalmia syndrome and review the relevant literature. Image-guided combined external and endoscopic dacryocystorhinostomy was performed in a female, aged 16 years with congenital partial arhinia and ipsilateral microphthalmus. The lacrimal sac was bypassed to the contra lateral nasal cavity through a septal window. The surgical procedure was performed using the intra-operative optical image-guided Nav 1 PicoTM ENT navigation system with real-time intra-operative instrument geometry. Different phases of the surgical technique, adjunctive endoscopic procedures, intra-operative anatomical guidance, and utility at crucial phases of surgery were noted. A review of the literature was performed pertinent to arhinia and navigation guided lacrimal surgeries. Lacrimal bypass into the contra lateral nasal cavity even through a malformed septum is possible in partial arhinia syndromes. Detailed preoperative evaluation including 3D imaging studies, navigation guided planning of risk structures with intra-operative distance control and construction of meticulous surgical roadmaps were found to be essential factors in successful outcomes. At six months follow up after surgery, there was a complete and contiguous healed mucosal anastomosis with lacrimal system patent on irrigation and resolution of epiphora. Combined external and endoscopic approach is useful in partial arhinia syndromes. Image guidance is a very useful adjunctive tool that facilitates safe and precise surgery in the management of such complex lacrimal surgeries.
Orbit 2017 Jun
PMID:Image-guided lacrimal drainage surgery in congenital arhinia-microphthalmia syndrome. 2827 4

In ophthalmology, there have been few reports of botulinum toxin type-A (BTX-A) injection into the lacrimal gland to treat epiphora. In ENT, adductor and abductor (ABSD) spasmodic dysphonia are often treated with BTX-A injections into the respective overacting vocal cord muscles. We describe a 53-year old male with Parkinson's disease who did not respond to BTX-A injections to either the lacrimal gland, for epiphora secondary to Parkinsonian-related blink lagophthalmos, or posterior cricoarytenoid (PCA) muscles for ABSD. Subsequent BTX type-B (BTX-B) injections into the lacrimal gland remarkably improved his epiphora. BTX-B injections into the PCA muscle also greatly improved his dysphonia. We describe the first reported case of (1) BTX-B injection into the lacrimal gland for epiphora, (2) use of Botox in treating epiphora due to blink lagophthalmos/reduced blink frequency secondary to Parkinson's disease, (3) BTX-B use in treating ABSD, and (4) association between ABSD and Parkinson's disease.
Orbit 2019 Jun
PMID:Botulinum toxin-B injection into the lacrimal gland and posterior cricoarytenoid muscle for the treatment of epiphora and abductor spasmodic dysphonia secondary to Parkinson's disease. 2993 26