A 14-year old boy presents with history of frequent nasal bleeding. His Hb was found to be 6.4 g/dL and peripheral smear showed normocytic hypochromic anemia. The most probable diagnosis is:
Ans. a. Juvenile nasopharyngeal angiofibroma (Ref: Dhingra 5 e p261-263)A 14-year-old boy presents with history offrequent nasal bleeding. His Hb was found to be 6.4 g/dL and peripheral smear showed normocytic hypochromic anemia. The most probable diagnosis is juvenile nasopharyngeal angiofibroma.The main vessel involved in bleeding from Juvenile nasopharyngeal angiofibroma is Internal maxillary artery."Juvenile Nasopharyngeal Angiofibroma: The main feeding vessel in most of the cases is internal maxillary artery, branch of external carotid artety; "Juvenile Nasopharyngeal AngiofibromaMC benign tumor of nasopharynxQ (but overall angiofibroma is rare).MC site: Posterior part of nasal cavity close to the margin of sphenopalatine foramen.Seen almost exclusively in males of 10--20 yearsQTestosterone dependent tumor, seen in pre-pubertal to adolescent malesQLocally invasive vasoformative tumor consisting of endothelium lined vessels with no muscle coat.Major blood supply: From internal maxillary arteryClinical Features:MC symptom: Spontaneous profuse and recurrent epistaxisQ.Progressive nasal obstruction, denasal speech, hyposmia or anosmia, broadening of nasal bridgeQOtalgia, conductive hearing loss, serous otitis media due to eustachian tube obstructionQ.Tumor in the orbit causes proptosis and frog-face deformityQ; diplopia and diminished vision.Tumor in infratemporal fossa can cause trismus and bulge of parotid.II, III, IV, V, VI cranial nerve can be involved.Signs: Splaying of nasal bones, pink or purplish mass obstructing one or both ehoanae in nasopharynx, swelling of cheek and fullness of face.In an adolescent male, profuse recurrent episodes of nosebleed suggests juvenile nasopharyngeal angiofibroma until proven otherwiseQDiagnosis:IOC: CT scan of head with contrast enhancementCT shows extent, bony destruction or displacements and anterior bowing of the posterior wall of maxilla due to tumor enlarging in pterygopalatine fossaAntral sign or Holman miller signQ:Pathognomic of angiofibroma.Anterior bowing of the posterior wall of maxilla due to tumor enlarging in pterygopalatine fossaMRI: To view the soft tissue extension and is complementary to CT scan.Carotid angiography: Shows extent of the tumor, its vascularity and feeding vesselBiopsy is contraindicated in juvenile nasopharyngeal angiofibromaQTreatment:Surgical excision is treatment of choiceQ.Preoperative embolization and estrogen therapy or cryotherapy or radiotherapy reduce blood loss in surgery. Preoperative embolization of the tumour reduces its blood supply and causes Jess bleeding, if tumour removal is performed within 24-48 hour of embolization before collaterals have time to develop.Surgical approach for Nasopharyngeal AngiofibromaSurgical approach of choice: Midfacial degloving approach to nasopharynx.Wilson approachQ: Transpalatal approach is for tumor confined to nasopharynx.Lateral rhinotomy: For larger tumors involving nasal cavity, paranasal sinuses.Other Approaches:Sardana's approachQ: Transpalatine + SublabialExtended Denker's approachQ.Recurrence:Recurrence rate after surgery: 30-50%Recurrences usually become evident within 2--3 years of initial resection.
Juvenile nasopharyngeal angiofibroma
Hemangioma
Antrochonal polyp
Carc inoma of nasopharynx
Tumors of Nose and PNS