Mastoid abscess as a Presenting Complaint of Kostmann syndrome in childhood. | ||||
| Sohag Medical Journal | ||||
| Article 23, Volume 24, Issue 2, April 2020, Page 158-162 PDF (774.69 K) | ||||
| Document Type: Original Article | ||||
| DOI: 10.21608/smj.2020.20958.1081 | ||||
 View on SCiNiTO | ||||
| Authors | ||||
ramadan sayed1; ahmed ismail1; khaled gamal dahy1; arafat mohamed 1; Farghali Mekki  2
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| 1otorhinolaryngology faculty of medicine sohag university egypt | ||||
| 2otorhinolaryngology faculty of medicine sohag University Egypt | ||||
| Abstract | ||||
| Kostmann syndrome is a rare congenital disorder characterized by severe neutropenia due to impairment of myeloid differentiation in the bone marrow, with the neutrophil count being characteristically less than 500_103 cells/l Male child 4.5-year old complains from a bilateral aural discharge of 2years duration. examination there is multiple skin scars, gapped postauricular wound with necrosis in parts and inflamed lower part of mastoid and upper part of neck, and bilateral pinhole central perforation with pulsating discharge. Normal cranial nerve examinations. Hematological investigations were done that reveal severe neutropenia (kostmann syndrome). canal wall down (CWD) mastoidectomy with wide meatoconchoplasty for exteriorization of the cavity was done Removal of infected and necrosed tissues in the postauricular region, undermining of skin and primary repair was done in two layers . after that, ear discharge and postauricular swelling improved and patient with good general condition with an excellent outcome.so mastoid abscess can be a presenting symptom of Kostmann syndrome. | ||||
| Keywords | ||||
| mastoid; abscess; kostmann; syndrome | ||||
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