Abortive Pneumatization of Sphenoid Sinus
Case 1: What is the diagnosis? The degree of pneumatization of themost sphenoid likely sinus is sometimes quite variable. Pneumatization usually proceeds from anterior to posterior. The pneumatization process may arrest at any point in the process with resultant changes that can be quite confusing. The sinus that is involved is usually smaller in size with abnormally appearing bone along the posterior margin of the smaller sinus. The abnormal bone often has a soap-bubbly appearance, sclerotic margins (blue arrow) with the presence of fat (red arrows) within the area of aborted pneumatization.
DDx: Abortive Pneumatization 1. Multiple Myeloma 2. Metastasis 3. Chordoma 4. Chondrosarcoma 5. Histiocytosis
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DDx
DDx: Choanal Atresia
Case 2: What is the most likely diagnosis?
1. Midface Hypoplasia 2. Choanal Stenosis 3. Choanal Stenosis from Fibrous Dysplasia
Choanal Atresia Atresia of the posterior nasal choanal may be unilateral (red arrows) or bilateral, membranous or bony, or bony on one side and membranous on the contralateral side. Patients with bilateral atresia usually present at birth with cyanosis and/or respiratory distress with feeding. Unilateral atresias or choanal stenosis may not be as obvious clinically or even be unsuspected. The atresia is often due to hypertrophy or overgrowth of the palatine bone that forms part of the posterior nasal choana.
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DDx
Case 3: What is the most likely diagnosis?
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DDx: Cleft Lip, Maxillary Alveolus, and Face
1. 2. 3. 4. 5.
Case 3: What is the most likely diagnosis?
Ununited Midface Fracture Orodigitofacial Dysostosis Popliteal Pterygium (Web) Syndrome Amniotic Band Syndrome Mandibulofacial Dysostosis
Cleft Lip, Maxillary Alveolus, and Face Cleft lip (cheiloschisis) and cleft palate (palatoschisis), which can also occur together as cleft lip and palate, are variations of congenital clefting deformity caused by abnormal facial development during gestation. Approximately 1 in 700 children born have a cleft lip and/or a cleft palate. Clefts can also affect other parts of the face, such as the eyes, ears, nose, cheeks, and forehead (red arrows). In 1976, Paul Tessier described fifteen lines of midface clefting (next page).
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DDx
Case 3: What is the most likely diagnosis?
Tessier Facial Cleft Classification
Case 4: What is the most likely diagnosis?
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DDx: Nasoalveolar (Nasolabial) Cyst
1. 2. 3. 4. 5.
Case 4: What is the most likely diagnosis?
Nasopalatine Duct Cyst Glandular Odontogenic Cyst Dermoid or Epidermoid Cyst Median Alveolar Cyst Median Palatal Cyst
Nasoalveolar (Nasolabial) Cyst Nasolabial cysts have a strong female predilection (75% occur in women) and appears to occur more frequently in blacks than in whites. It is found near the base of the nostril, just above the floor of the nasal cavity, or in the superior aspect of the upper lip (red arrows). They are bilateral in approximately 10% of all cases. The cyst usually obliterates the nasolabial fold and may elevate the ala of the nose on the affected side. Almost always there a fibrous or epithelial attachment to the nasal mucosa . The cyst may be secondarily inflamed and somewhat tender to palpation. Occasional cysts rupture or drain into the oral cavity or nose.
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Case 5: What is the most likely diagnosis?
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Nasopalatine Duct Cyst
Case 5: What is the most likely diagnosis?
This cyst, also known as an elevator shaft cyst, occurs in the midline of the anterior hard palate just superior to the incisive foramen. It is the most common type (about 70%) of oral non-odontogenic cysts. Radiographically, it appears as a heart-shaped radiolucency. It is usually asymptomatic, but may sometimes result in fullness of the mucosa in the anterior portion of the palate. Nasopalatine duct are developmental in nature. Nasopalatine duct cysts occur in all groups, including fetuses. Most patients who are symptomatic are from 30-60 years of age.
DDx: Nasopalatine Duct Cyst 1. 2. 3.
Odontogenic Cyst Odontogenic Neoplasm Nonodontogenic Neoplasm
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DDx
Case 6: What is the most likely diagnosis?
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Pyriform Aperture Stenosis
Case 6: What is the most likely diagnosis?
Stenosis of the anterior nasal apertures is a rare anomaly that produces respiratory difficulty immediately after birth. Clinical differential diagnosis is bilateral choanal atresia. Congenital pyriform aperture stenosis can be associated with holoprosencephaly, endocrine abnormalities, and maxillary teeth anomalies. Patients with a central mega-incisor have an increased incidence of pituitary and endocrine abnormalities. Imaging of the entire head and face is recommended in these patients. It is a potentially life-threatening condition unless identified and treated. It can cause acute respiratory distress in feeding neonates as they are obligate nasal breathers.
DDx: Pyriform Aperture Stenosis 1. Midface Hypoplasia 2. Nasal Hypoplasia (Chondrodysplasia punctata, Warfarin Teratogenicity)
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DDx
DDx: Cystic Fibrosis
1. 2. 3. 4.
Case 7: What is the most likely diagnosis?
Aspergillosis Asthma (Nasal Polyposis) Allergic Fungal Sinusitis Immotile Cilia Syndromes
Cystic Fibrosis Cystic fibrosis (also known as CF or mucoviscidosis) is a common recessive genetic disease which affects the entire body, causing progressive disability and often early death. The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas. Difficulty breathing is the most serious symptom and results from frequent lung infections. A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and infertility result from the effects of CF on other parts of the body. It is one of the most common chronic lung diseases in children and young adults.
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DDx
Case 8: What is the most likely diagnosis?
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DDx: Sinonasal Wegener Granulomatosis
1. 2. 3. 4. 5.
Case 8: What is the most likely diagnosis?
Cocaine Nose Sinonasal Sarcoidosis Invasive Fungal Sinusitis Non-Hodgkin T-cell Lymphoma Squamous Cell Carcinoma
Sinonasal Wegener Granulomatosis Non-neoplastic, aseptic, necrotizing vasculitis that preferentially involves upper & lower respiratory tracts and kidneys. Soft tissue mass in the nose with septal & non-septal bone destruction. A sinus CT with contrast is useful for initial evaluation. If orbital, deep facial, skull base or meningeal involvement are suspected from the CT or the clinical symptoms, an enhanced MRI is useful. Age at presentation: 40-60 years. Most common location is in the nose, followed by the sinuses. Often affects nasal septum first. Orbital invasion most common extra-sinonasal site.
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DDx
Case 9: What is the most likely diagnosis?
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Case 9: What is the most likely diagnosis?
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DDx: Antrochoanal Polyp
1. 2. 3. 4. 5.
Case What Sinonasal 9a-b: Polyp (of usual type) is the most likely diagnosis? Juvenile Nasopharyngeal Angiofibroma (young males) Angiomatous Polyp (older individuals) Inverting Papilloma Botryoid Embryonal Rhabdomyosarcoma
Antrochoanal Polyp An inflammatory polyp arising from the antrum of the maxillary sinus. They are often associated with sinusitis but are not thought to be of infectious origin. They are slightly more common in males, and occur in the first through fourth decades of life. They make up approximately one-third of sinonasal polyps in children. Symptoms include rhinorrhea and nasal obstruction. A unique feature of these polyps is that the lesions extend Case 9a Caseinto 9bthe nasal cavity, and from the maxillary sinus via a pedicle through the natural or secondary maxillary ostia, into the posterior choana. It is an inflammatory polyp with a long stalk. They frequently exhibit scattered, bizarre stromal cells.
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Case 10: What is the most likely diagnosis?
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Case 10: What is the most likely diagnosis?
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DDx: Allergic Fungal Sinusitis - Expansile Sinonasal Polyposis 1. 2. 3.
Case 10: What is the most likely diagnosis?
Sinonasal Neoplasm Fungal Sinusitis Esthesioneuroblastoma
Expansile Sinonasal Polyposis- Allergic Fungal Sinusitis Allergic fungal sinusitis (AFS) may initiate a severe form of chronic polypoid sinusitis. It probably results from Type I hypersensitivity reaction to antigens within the sinuses. Multiple sinuses may be involved with expansion of the involved sinus by a combination of inflammatory polyps and hyperdense, fungal laden secretions. AFS may be associated with sinus wall erosion and polypoid mucosal thickening. The disease may invade the orbits or intracranial cavity. Sinus contents are usually of mixed iso- to hyperdensity on CT. Secretions are iso- to hyperintense on T1W images and typically iso- to hypodense on T2W images. In some cases the sinus contents may be so back on MR that they can be misinterpreted as air. There is enhancement of the peripheral mucosa. Polyps are present in 85% of patients with allergic fungal sinusitis (AFS). AFS affects immunocompetent children and adults. There is a higher prevalence in warm, humid environments and it accounts for 7% of chronic sinusitis requiring surgery.
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