Proptosis in a young child
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《印度儿科学杂志》
Abstract
We encountered an extremely unusual presentation of proptosis in a 7-yr-old boy due to psammomatoid juvenile ossifying fibroma of frontoethmoidal region. The tumor also led to development of mucocele in the frontal sinus not yet fully developed as on opposite side.
Keywords: Proptosis; Mucocele; Psammomatoid juvenile ossifying fibroma
How to cite this article:
Singh I, Ghimire A, Bhadani P, Nepal A, Maharjan M, Gauum D, Sen R. Proptosis in a young child. Indian J Pediatr 2006;73:537-538
How to cite this URL:
Singh I, Ghimire A, Bhadani P, Nepal A, Maharjan M, Gauum D, Sen R. Proptosis in a young child. Indian J Pediatr [serial online] 2006 [cited 2006 Jul 14];73:537-538. Available from: http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2006;volume=73;issue=6;spage=537;epage=538;aulast=Singh
The psammomatoid ossifying fibroma, also known as juvenile ossifying fibroma, is a rare tumor commonly arising in the mandible and maxilla. The tumor is locally aggressive and has a high rate of recurrence. Histologically, it is generally well circumscribed, shows bone trabeculae lined by osteoblasts distributed in variable amount of vascularized fibrous stroma. The pathognomonic feature is the presence of spherical ossicles which are similar to psammoma bodies [1]. We report a case of psammomatoid ossifying fibroma arising in the fronto-ethmoidal region presenting with proptosis and mucocele of the frontal sinus.
Case Report
A 7-year-old male child was referred to otolaryngology services from the department of ophthalmology for non-painful but progressively bulging left eye for the last one year. There was history of occasional headache but there was no loss of vision or diplopia. Clinical examination revealed left eye proptosis with lateral and downward displacement of the eyeball with normal movement . The vision was 6/6. There was minimal broadening of the nasal bridge. CT scan was suggestive of proptosis and mucocele on left side with fibro osseous lesion in the left frontal sinus. The frontal sinus extended into the anterior cranial fossa in contrast to the barely recognizable frontal sinus on the right side [Figure - 1]. The patient was operated by Lynch-Haworth approach. Both ethmoid and frontal sinuses were explored. The bone at the roof of the orbit was resorbed and papery thin. The frontal mucocele contained straw colored fluid. Whitish cheese like material was removed from the floor and medial wall of the orbit which extended to the apex of the orbit, brought close to the optic nerve [Figure - 2]. Drain was placed in the fronto-nasal duct and fixed. Histopathological examination of the cheesy material was reported as psammomatoid ossifying fibroma [Figure - 3]. The proptosis decreased markedly and headache settled. No recurrence was seen during a follow up of six months.
Discussion
Ossifying fibroma, commonly seen in mandible, is a benign slow growing tumor which may behave in a non- predictable aggressive fashion.[2] Johnson et al hypothesized that the lesion arose from overproduction of the myxofibrous cellular stroma normally involved in the development of the septa in the paranasal sinuses as they enlarge and pneumatize. These stromal cells secrete hyaline material that ossifies and connective tissue mucin that initiates the cystic areas.[3]
Majority of the cases have been reported in children and young adults between 5 to 25years of age with slight male predominance (1.2 :1).The tumor has been found confined to the orbital bones and para nasal sinuses in 61.6% of the cases and in the maxilla in 19.7 %.[3],[4] The present case was also of a male child of 7 years with involvement of the frontal bone by the tumor as seen on CT scan. Frontal sinus arises from the anterior ethmoid air cells. It makes it's appearance radiologically at 6 years of age.[5] In our case, the right frontal sinus was seen as air bud only, but there was full blown mucocele on the left side. Frontal mucocele at this age is very rare. In a somewhat similar case, a 16-years-old female had diplopia and large frontal sinus mucocele.[6]
Treatment of choice is surgical. The tumor has tendency for aggressive growth and high recurrence rate (30%).[3] Local recurrence is more likely due to the incomplete removal of the tumor.[4] An open surgical technique is preferred many times for adequate visualization of the mass in an extensive tumor so that it is removed completely, without affecting the growth of the maxillofacial bones and the aesthetic looks in the young patients. In the index case too an open surgical procedure was done to remove the tumor completely.The case is on regular follow up and there is no recurrence till now. In the study of Hartstein et al, there was no recurrence of the tumor when the removal of the disease was complete.[7]
References
1. Samir El-mofty, St Louis. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathological entities. Oral Surg Oral Med Oral Pathol. Oral Radiol Endod 2002; 93 : 296-304.
2. Bhat K V, Naseeruddin K. Int J Paediatr Otorhinolaryngol 2002; 64: 239-242.
3. Johnson LC, Yousefi M.,Vinh TN, Heffner DK, HyamsVJ, Hartman KS. Juvenile active ossifying fibroma: its nature, dynamic and origin. Acta Otolaryngol Suppl 1991; 488:1-40.
4. Makek M. Clinical pathology of fibro-osteo-cemental lesions of the craniofacial skeleton and jaw bones. Basel Switzerland Karger; 1983; 128-227.
5. Kuhn JP, Slovis TL, Haller JO. Caffey's paediatric diagnostic imaging 2004; 2 : 431-447.
6. Vandekerckhove K, Van den Abeele K, Van den Broecke C, Verstraete K, Meire F. Psammomatoid ossifying fibroma of the ethmoid. Bull Soc Belge Ophtalmol 2003; (287): 9-14.
7. Hartstein ME, Grove AS Jr, Woog JJ, Shore JW, Joseph MP. The multidisciplinary management of psammomatoid ossifying fibroma of the orbit. Ophthalmology 1998; 105(4) : 591-595.(Singh Ishwar, Ghimire Ana)
We encountered an extremely unusual presentation of proptosis in a 7-yr-old boy due to psammomatoid juvenile ossifying fibroma of frontoethmoidal region. The tumor also led to development of mucocele in the frontal sinus not yet fully developed as on opposite side.
Keywords: Proptosis; Mucocele; Psammomatoid juvenile ossifying fibroma
How to cite this article:
Singh I, Ghimire A, Bhadani P, Nepal A, Maharjan M, Gauum D, Sen R. Proptosis in a young child. Indian J Pediatr 2006;73:537-538
How to cite this URL:
Singh I, Ghimire A, Bhadani P, Nepal A, Maharjan M, Gauum D, Sen R. Proptosis in a young child. Indian J Pediatr [serial online] 2006 [cited 2006 Jul 14];73:537-538. Available from: http://www.ijppediatricsindia.org/article.asp?issn=0019-5456;year=2006;volume=73;issue=6;spage=537;epage=538;aulast=Singh
The psammomatoid ossifying fibroma, also known as juvenile ossifying fibroma, is a rare tumor commonly arising in the mandible and maxilla. The tumor is locally aggressive and has a high rate of recurrence. Histologically, it is generally well circumscribed, shows bone trabeculae lined by osteoblasts distributed in variable amount of vascularized fibrous stroma. The pathognomonic feature is the presence of spherical ossicles which are similar to psammoma bodies [1]. We report a case of psammomatoid ossifying fibroma arising in the fronto-ethmoidal region presenting with proptosis and mucocele of the frontal sinus.
Case Report
A 7-year-old male child was referred to otolaryngology services from the department of ophthalmology for non-painful but progressively bulging left eye for the last one year. There was history of occasional headache but there was no loss of vision or diplopia. Clinical examination revealed left eye proptosis with lateral and downward displacement of the eyeball with normal movement . The vision was 6/6. There was minimal broadening of the nasal bridge. CT scan was suggestive of proptosis and mucocele on left side with fibro osseous lesion in the left frontal sinus. The frontal sinus extended into the anterior cranial fossa in contrast to the barely recognizable frontal sinus on the right side [Figure - 1]. The patient was operated by Lynch-Haworth approach. Both ethmoid and frontal sinuses were explored. The bone at the roof of the orbit was resorbed and papery thin. The frontal mucocele contained straw colored fluid. Whitish cheese like material was removed from the floor and medial wall of the orbit which extended to the apex of the orbit, brought close to the optic nerve [Figure - 2]. Drain was placed in the fronto-nasal duct and fixed. Histopathological examination of the cheesy material was reported as psammomatoid ossifying fibroma [Figure - 3]. The proptosis decreased markedly and headache settled. No recurrence was seen during a follow up of six months.
Discussion
Ossifying fibroma, commonly seen in mandible, is a benign slow growing tumor which may behave in a non- predictable aggressive fashion.[2] Johnson et al hypothesized that the lesion arose from overproduction of the myxofibrous cellular stroma normally involved in the development of the septa in the paranasal sinuses as they enlarge and pneumatize. These stromal cells secrete hyaline material that ossifies and connective tissue mucin that initiates the cystic areas.[3]
Majority of the cases have been reported in children and young adults between 5 to 25years of age with slight male predominance (1.2 :1).The tumor has been found confined to the orbital bones and para nasal sinuses in 61.6% of the cases and in the maxilla in 19.7 %.[3],[4] The present case was also of a male child of 7 years with involvement of the frontal bone by the tumor as seen on CT scan. Frontal sinus arises from the anterior ethmoid air cells. It makes it's appearance radiologically at 6 years of age.[5] In our case, the right frontal sinus was seen as air bud only, but there was full blown mucocele on the left side. Frontal mucocele at this age is very rare. In a somewhat similar case, a 16-years-old female had diplopia and large frontal sinus mucocele.[6]
Treatment of choice is surgical. The tumor has tendency for aggressive growth and high recurrence rate (30%).[3] Local recurrence is more likely due to the incomplete removal of the tumor.[4] An open surgical technique is preferred many times for adequate visualization of the mass in an extensive tumor so that it is removed completely, without affecting the growth of the maxillofacial bones and the aesthetic looks in the young patients. In the index case too an open surgical procedure was done to remove the tumor completely.The case is on regular follow up and there is no recurrence till now. In the study of Hartstein et al, there was no recurrence of the tumor when the removal of the disease was complete.[7]
References
1. Samir El-mofty, St Louis. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathological entities. Oral Surg Oral Med Oral Pathol. Oral Radiol Endod 2002; 93 : 296-304.
2. Bhat K V, Naseeruddin K. Int J Paediatr Otorhinolaryngol 2002; 64: 239-242.
3. Johnson LC, Yousefi M.,Vinh TN, Heffner DK, HyamsVJ, Hartman KS. Juvenile active ossifying fibroma: its nature, dynamic and origin. Acta Otolaryngol Suppl 1991; 488:1-40.
4. Makek M. Clinical pathology of fibro-osteo-cemental lesions of the craniofacial skeleton and jaw bones. Basel Switzerland Karger; 1983; 128-227.
5. Kuhn JP, Slovis TL, Haller JO. Caffey's paediatric diagnostic imaging 2004; 2 : 431-447.
6. Vandekerckhove K, Van den Abeele K, Van den Broecke C, Verstraete K, Meire F. Psammomatoid ossifying fibroma of the ethmoid. Bull Soc Belge Ophtalmol 2003; (287): 9-14.
7. Hartstein ME, Grove AS Jr, Woog JJ, Shore JW, Joseph MP. The multidisciplinary management of psammomatoid ossifying fibroma of the orbit. Ophthalmology 1998; 105(4) : 591-595.(Singh Ishwar, Ghimire Ana)