Kabekkodu Sushmitha, Sreedharan Suja, Gupta Kirti, Murali Nirupama
Otorhinolaryngology, Kasturba Medical College Mangalore, Manipal Academy of Higher Eduction, Mangalore, Karnataka, India.
Otorhinolaryngology, Kasturba Medical College Mangalore, Manipal Academy of Highe Education, Mangalore, Karnataka, India
BMJ Case Rep. 2021 Mar 5;14(3):e238778. doi: 10.1136/bcr-2020-238778.
Mucopolysaccharidosis (MPS) type II is a rare multisystem disorder resulting from the accumulation of breakdown products of glycosaminoglycans in the body tissues. Many patients with this disease undergo ENT (ear, nose and throat) surgeries such as adenotonsillectomy and tympanocentesis at a very early age, much before the diagnosis of MPS. Nasal polyposis is a rare occurrence, with only one case of MPS II with polyposis reported in the literature. We present a patient who presented with recurrent nasal polyposis from the age of 2 years. Hale's colloidal iron was used to stain these 'nasal polyps', which revealed that they are, in fact, mucopolysaccharide-laden sinonasal mucosa prolapsing into the nasal cavities. We believe this is the first time that this stain has been used to stain nasal polyps in MPS. In addition to the histopathological peculiarities of these nasal masses, we also discuss the natural history of nasal polyposis in MPS II.
II型黏多糖贮积症（MPS）是一种罕见的多系统疾病，由糖胺聚糖分解产物在人体组织中蓄积所致。许多患有这种疾病的患者在很小的时候，即在MPS确诊之前很久，就接受了耳鼻喉（耳、鼻、喉）手术，如腺样体扁桃体切除术和鼓膜穿刺术。鼻息肉病很少见，文献中仅报道过1例II型MPS合并息肉病的病例。我们报告1例患者，从2岁起就反复出现鼻息肉。用黑尔氏胶态铁对这些“鼻息肉”进行染色，结果显示它们实际上是充满黏多糖的鼻窦黏膜脱垂入鼻腔。我们认为这是首次使用这种染色方法对MPS患者的鼻息肉进行染色。除了这些鼻肿物的组织病理学特征外，我们还讨论了II型MPS中鼻息肉病的自然病程。
