Haemorrhagic cystic sino-nasal fibrous dysplasia - Springer

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Fibrous dysplasia of bone is a rare disorder. The term fibrous dysplasia was coined by Liechtenstein and Jaffe. (1942) to group together a variety of bony lesionsĀ ...

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Address f o r Correspondence : Dr. Kasim Behranwala 285, Thakurdwar R o a d , R o g a y Apartment, 5 th floor, Mumbai- 400 002

H A E M O R R H A G I C CYSTIC S I N O - N A S A L F I B R O U S DYSPLASIA R. K. S a x e n a ~, S a u r a b h V a r s h n e y 2, J. Singh 3, A. K a u s h a l 4, P. P. B i s h n u s

Key words : Fibrous dysplasia, Cranio-facial resection.

INTRODUCTION Fibrous d y s p l a s i a o f b o n e is a rare disorder. T h e term fibrous dysplasia was coined by Liechtenstein and Jaffe (1942) to g r o u p together a variety o f b o n y lesions such as osteitis osteodystrophia fibrosa and Juvenile P a g e t ' s disease. F i b r o u s d y s p l a s i a is an u n c o m m o n l e s i o n o f obscure aetiology. It is characterised b y r e p l a c e m e n t o f the normal b o n e matrix by fibro-osseous connective tissue, histologically exhibiting varying degree of osseous m e t a p l a s i a . T h e r e are three r e c o g n i s e d f o r m s o f the disease-monostotic, polyostotic and A l b r i g h t syndrome. Facial fibrous dysplasia denotes a fibro-osseous thickening o f the b o n e s o f the face. It is unilateral and can affect several facial b o n e s but it must be differentiated f r o m polyostotic fibrous dysplasia which is a systemic disorder affecting m a n y bones throughout the body. It represents

an arrest o f the maturation o f b o n e formation at the stage of woven bone. There are varying degrees o f fibrosis and in all cases, the lesion is diffuse. Teeth are often present in the lesion. Facial fibrous dysplasia can present as a swelling around the orbit, over the c h e e k or on the maxillary alveolus. In the cranio-facial region, the maxilla is most frequently involved. The mandible, ethmoidal, frontal and sphenoidal regions f o l l o w in the o r d e r o f p r e v a l e n c e . It o e c o m e s a p p a r e n t during c h i l d h o o d and i n c r e a s e s in size w i t h growth but it often ceases to expand after the age o f 20. Case details : A 9 y e a r s old T i b e t a n m a l e c h i l d w a s r e f e r r e d f r o m Department o f Paediatrics with complaints of the left nasal

1Professor and Head, 2Assistant Professor, 3Assistant Professor, 4Senior Resident (ENT), 5Associate Professor (Neurosurgery), Himalayan Institute of Medical Sciences, Jollygrant, Dehradun (U.P.) 248 140

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Fig. I :

Pre-op photograph of patient showing proptosis.

Fig. II :

X-ray PNS (Water's view). A. Pre-opertive ground glass appearance. B. Post operative.

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Fig. III: Pre-op CT Scan coronal section.

blockage, occasional left nasal bleeding, bulging of left eye ball and widening of nasal bridge for the last nine months. The complaints were insiduous in onset without any history of fever, trauma, and local pain. There was no history suggestive of any intracranial extension of the disease. There was no history of any decrease in vision, double vision and any excessive watering from the eyes. On examination, the child was cheerful with average build and nutrition. He had telecanthus, widened nasal bridge on the left. Left eye ball had proptosis (antero-lateral), eye movements were normal in all the directions, vision and visual field perimetry were normal. Anterior rhinoscopy revealed septum was pushed towards right side, touching the right lateral wall, with mucoid discharge from right nostril. Left nostril had a red coloured mass arising from its lateral wall, and completely occluding the cavity (inferior turbinate could not be visualised). Consistency

Fig. IV A&B : Post-op CT scan coronal section.

of the mass was firm and it bled on touch. Rest of the h e a d and n e c k e x a m i n a t i o n , along with s y s t e m i c examination was normal. Opinions of Ophthalmologist and N e u r o s u r g e o n were taken, which gave no additional findings.

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On radiological evaluation, X-ray Paranasal sinuses (occipito-mental view) revealed a ground glass appearance, involving left ethmoids, nasal cavity, maxillary sinus and orbit, suggestive of fibrous dysplasia. CECT Cranium, Orbit and PNS (sequential axial and coronal 5 and 10 mm scans were obtained before and after the administration o f I/V contrast) - there was a large expansile lesion involving the left ethmoid air cells(both anterior and posterior group). Irregularly marginated walls were noted which showed sclerosis and patchy rarifaction of the affected bone. Thick fluid contents with layering were noted within it. Medial wall of the left orbit was also expanded and displacement of the medial and inferior rectus was noted. There was smooth indentation into the retrobulbar region, mild impression on the left optic nerve with partial encroachment on the inferior orbital fissure. Optic canal and superior orbital fissure were also indented by this mass in their proximal course, though there was no complete encroachment of the same. Expansion of the nasal cavity was noted with bowing of the medial wall of the left maxillary sinus. Posteriorly the wall of sphenoid sinus was also indented, with right sphenoid sinusitis. Nasopharyngeal airway, pterygoid plates, parapharyngeal spaces, infratemporal fossa, brain parenchyma, ventricular system, CSF cisterns and posterior fossa were normal. CECT imaging features were suggestive of (monostotic) fibrous dysplasia involving left ethmoid bone with possiblility of infected mucocoele. An attempt was made to do a FNAC from the left nostril mass, which aspirated approximately 40ml of altered blood. All routine haematological, biochemical and radiological examination were normal, (including serum calcium, phosphate and alkaline phosphatase). Patient was taken up for surgery by transfacial and transcranial approach for resection. Under general anaesthesia, surgery was started by ENT surgeon, via left lateral rhinotomy approach and the mass was removed in piecemeal. From the areas of ethmoid and sphenoid, mass was removed under endoscopic vision. It was firm in consistency and was involving the left nasal cavity, ethmoidal and sphenoidal sinuses. The encroachment of mass in the area of superior orbital fissure and optic canal could not be dealt by lateral rhinotomy approach, hence neurosurgeon took transcranial approach (left frontal extradural approach) orbital roof was removed as a free flap, medial-posterior decompression of the left orbit and

deroofing of the optic canal upto the superior orbital fissure was done. Bony and soft fascia reconstruction of orbit roof and ethmoid gallery was done. The result of the surgery was evaluated peroperafively in view of proptosis which was satisfactory. The postoperative period was uneventful and patient recovered well, was asymptomatic with significant reduction in proptosis by third day. The histopathological examination of the specimen revealed large areas of old and flesh haemorrhage, proliferating fibroblasts and endothelial cells and areas of irregular trabeculae of immature bone separated by cellular fibrous stroma. Most of these t r a b e c u l a e were d e v o id o f peripheral osteoblastic rimming. There were no mitotic figures or pleomorphism in the stromal tissue. A few bits of mature calcified bone were also present. Hence, histopathology was consistent with fibrous dysplasia with haemorrhagic cystic change. The patient is doing well after 3 months of follow up and is being regularly followed up by nasal diagnostic endoscopy for any recurrence. DISCUSSION Fibrous dysplasia is a benign bone lesion consisting of bone forming fibrous tissue in one of the bones of the face and sinuses. It comprises 2.5% of all bone neoplasia and 7% of all benign bone tumors. In the polyostotic form, deformity of the long bones may occur, alongwith the skull lesions. It is usually seen in first and second decades of life. Maxilla is the most frequently involved bone. Ramsey et al (1968) found an equal sex ratio, and mandible to be the most frequently involved bone. The disease does not progress rapidly. When the dysplasia is monostotic, eye symptoms are prominent - proptosis, deformity of the orbit, and even optic pressure atrophy may occur. Moore's review of the ophthalmic literature describes the rapid onset of proptosis over weeks to month. There is downward displacement of the globe, diplopia, nasal obst ruct i on and epiphora. With sphenoid sinus involvement, optic atrophy occurs. The roentgenogram shows a homogenous lesion having a ground glass appearance, with a gradual blending into normal bone, this appearance comes from the mixture of tiny spicules of bone within the dysplasia. Radiography of the sinuses may show that the extent of the disease can be much greater than is apparent on the surface and a series of sinuses may be obliterated by the abnormal vascular spongy bone. Fries (1957) classified X-ray findings as (a) Pagetoid with expansion of the bone (b)

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Sclerotic and (c) Cystic. Radiologically, the lesion i~ translucent if fibrous tissue predominates and appears like g r o u n d glass if there is more c a l c i f i c a t i o n . Microscopic examination reveals a moderate to highly cellular fibrous stroma composed of uniform, benign appearing spindle-cells. Irregular trabeculae of bone without lamella and osteoblastic rims are mixed in the fibrous stroma. The condition is considered to be a bony hamartoma. Although fibrous dysplasia is termed a benign lesion, it tends to blend imperceptibly into adjacent normal b o n e and no d e f i n i t i v e bor de r s can be distinguished at surgery. Malignant transformation of fibrous dysplasia into osteosarcoma is rare. The diagnosis is made on the history of a slowly developing painless swelling. Any lesion that suddenly increases in size or becomes painful must be suspected of having such a change. Surgery is the treatment of choice and is i n d i c a t e d for cosmetic or functional problems. Tr eatm e nt is needed only when there are s y m p t o m a t i c g r o w t h s , when the process threatens important structures, such as the eyes or optic nerve, or when there is c o n s e q u e n t i a l cosmetic def orm i t y or serious pain that is cl ear l y related to the process. Cosmetic trimming of the excess bone is necessary in some cases. Decompression of the optic nerve has been performed for the treatment of sphenoid sinus lesions, but the results are unpredictable. With the advent of sinonasal endoscopy and microneurosurgical techniques in the mid 1980s, and s u b s e q u e n t a d v a n c e s in t e c h n o l o g y and surgical techniques, management of some of these lesions is now feasible. As these lesions tend to stabilise in adult life, surgery should preferably be delayed until puberty, unless the growth is aggressive. Radiotherapy should not be used because osteosarcoma may be induced in lesions so treated.

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Vishwakarma S. K., Verma N., Kumar R. (1998) : Fibrous dysplasia o f facial bones. Hospital Today; III(9) : 581-583.

8. L i c t e n s t e i n L., Jaffe H. L. (1942) : Fibrous dysplasia o f bone condition affecting one, several many bones, graver cases o f which present as abnormal pigmentation o f skin, premature s k e l e t a l d e v e l o p m e n t , h y p e r t h y r o i d i s m o r still o t h e r extraskeletal abnormalities. Arch Path ; 33 : 777. 9.

McCune D. J. and Bruch H.(1937) : A m J. of diseases in children; 54 : 806-848.

10. Albright F., Butler A. M., Hampton A. d., Smith P. (1937) : syndrome characterized by osteitis fibrosa disseminate areas o f p i g m e n t a t i o n and e n d o c r i n e d y s f u n c t i o n w i t h ~ p r e c o c i o u s puberty in females. N. Eng. J. Med. ; 216 : 726-746. 11. Coley B. L. (1960) : Neoplasms o f bone and related conditions. second Ed. P. 16 N e w York, Paul Hoeber incorporated. 12. Bernard C. Pecaso (1986) : 'Fibro-osseous lesions o f the head and neck O C N A ; 19(3) : 489-496. 13. Ramsey M. E., Strong E. W., Frazeil E. L., (1968) : Am. J. Surg.; 116:542-547. 14. Ramsey M. E., Strong E. W., Frazeil E. L., (1970) : Progressive fibrous dysplasia o f the maxilla. J. Am. Dental Assoc. ; 81 : 1388. 15. Slow in., Freidman E. W. (1971) : Osteogenic sarcoma arising in a preexisting fibrous dysplasia. J. Oral Surg; 24 : 126. 16. Dutta S. K., Das U., Hazra T. K. (1998) : Fibrous dysplasia o f fight upper j a w - u n c o m m o n presentation. Indian Journal o f Otolaryngology and head and neck surgery. ; 50(4) : 390-391. 17. Chhangani D. L., Saxena R. K., (1972) : Clinical variations o f Fibroous dysplasia. Ind. J. Otol; X X I V (3) : 108-1. 18. Simon E., Matee M., Shubi f., Mselle T. (1999) : Cranio-facial fibrous dysplasia in a 38 year old African woman : a case history. Oral Dis; 5(3) : 247-249.

Address for Correspondence : Dr. Saurabh Varshney Assistant Professor (ENT) Himalayan Institute of Medical Sciences Jollygrant, Dehradun (U.P.) 248 140

Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 53 No. 2, April - June 2001