Eosinophlic oesophagitis

Introduction:

Eosionophlic oesophagitis ¹ is a relatively new disease entity. It is characterised by chronic / intermittent dysphagia, reflux like symptoms and intermittent oesophageal food impaction.

This condition was first reported by Landres in 1978 ². In 1993 Attwood and DeMeester reported 12 cases of dysphagia with no evidence of anatomic obstruction. They also reported dense eosinophilic infiltrates in the oesophagus. Attwood hence applied the criteria of presence of more than 20 eosinophils / high power field as histological criteria for diagnosing this condition ³.

Incidence:

True incidence of this disorder is still uncertain ^4. Review of literature puts this figure as high as

1% ⁵. It is more common in men than in women. Male : female ratio is 3:1. Age of presentation may vary between 2^nd – 4^th decades.

Conditions associated with oesophageal eosinophilia:⁶

1. Eosinophlic oesophagitis
2. GERD
3. Collagen vascular disorders
4. Parasitic infections
5. Eosinophlic gastroenteritis

Pathophysiology of eosinophlic oesophagitis:

1. This is a primary disorder involving oesophagus
2. Biopsy of oesophageal mucosa should contain atleast 50 eosinophils / high power field.
3. There should not be associated eosinophlic infiltration of stomach / intestine.
4. Eosinophlic microabscess can be see in the oesophagus extending up to its lumen
5. The caliber of oesophageal lumen is drastically reduced.
6. Asthma / atopia +
7. Peripheral eosinophilia common

Endoscopic features:

Linear furrowing of oesophageal mucosa.

Presence of white plaques / exudates

Presence of concentric rings / strictures of oesophageal mucosa.

Appearance of crepe paper mucosa is diagnostic.

Multiple biopsies should be studied before a categorical diagnosis of this condition could be made. Multiple biopsy specimen increases the accuracy of diagnosis.

Diagnosis of eosinophlic oesophagitis should not be made until GERD has been categorically ruled out by performing ambulatory pH testing or performing repeat biopsy after a 8 week trial course of proton pump inhibitor.

Clinical features:

1. Abdominal pain
2. Chest burns
3. Dysphagia
4. These patients are very slow eaters

Classification of eosinophlic oesophagitis:

Vasilopoulous ⁷ proposed the first classitication of eosinophilic oesophagitis.

Type I : Early small caliber oesophagus

Type II: Advanced small caliber oesophagus

Type III: Ringed oesophagus

Managment:

1. Avoidance of food allergen
2. Topical steroids
3. Oral steroids
4. Leukotriene inhibitors

Oesophageal dilatation is reserve for patients with extreme dysphagia

References:

1. Furuta GT, Liacouras CA, Collins MH, et al. Eosinophilic esophagitis in children and adults: a systematic review and consensus recommendations for diagnosis and treatment. Gastroenterology.

2007;133:1342-1363.

2. Landres RT, Kuster GGR, Strum WB. Eosinophilic esophagitis in a patient with vigorous achalasia. Gastroenterology 1978;74:1298-1301.

3. Attwood SEA, Smyrk TC, DeMeester TR, et al. Esophageal eosinophilia with dysphagia: a distinct clinicopathological syndrome. Dig Dis Sciences 1993;38:109-116.

4. Ronkainen J, Talley NJ, Aro P, et al. Prevalence of oesophagealeosinophils and eosinophilic oesophagitis in adults: the population-based Kalixanda study. Gut. 2007;56:615-620.

5. Arora AS, Yamazaki K. Eosinophilic esophagitis: asthma of the esophagus? Clin Gastroenterol Hepatol 2004;2:523-530.

6. Rodrigo S, Abboud G, Oh D, et al. High intraepithelial Eosinophil counts in esophageal squamous epithelium are not specific for eosinophilic esophagitis in adults Am J Gastroenterol 2008;103:435- 442.

7. Vasilipoulos S, Shaker R. Defiant dysphagia: small-caliber esophagus and refractory benign esophageal strictures. Current Gastroenterology Reports 2001;3:225-230.

Esthesioneuroblastoma

Introduction:

This tumor arises from the olfactory nerve. These are rare malignant tumors arising from the olfactory epithelium situated at the roof of the nasal cavity, cribriform plate, upper portion of supeior turbinate and corresponding upper portion of nasal septum. These tumors typically involve the upper portion of nasal cavity and the cribriform plate area. These tumors classically arise from the basal cells of olfactory neuroepithelium. No clearl cut genetic / other causes have been attributed for this tumor. This tumor represents about 3% of all malignant tumors involving the nasal cavity.

This tumor was first reported by Berger etal in 1924. Since then only less than 1000 cases have been reported in literature.

Incidence:

Affects both male and female patients with equal frequency.

Age incidence shows a bimodal peak (peaking at the second and the sixth decades of life).

Etiopathogenesis (theories):

1. Belongs to peripheral neuroectodermal family. (Not supported by immunohistochemistry)
2. Presence of Trisomy 8 has been documented in these tumor cells

None of these theories convincingly explain etiopathogenesis of this tumor.

Clinical features:

This tumor classically involves the nasal cavity with extension into the ethmoid sinus, anterior skull base and orbit. Since these tumors are very aggressive ones they tend to metastasize extensively. Spread to cervical nodes are common. Retropharyngeal nodes are the first echelon node. Rarely these tumors may be active in an endocrine sense. These tumors have known to cause ectopic secretion of ACTH causing Cushing's syndrome.

Common presenting symptoms were usually associated with the nasal cavity:

1. Nasal block
2. Epistaxis
3. Anosmia

Staging system:

Staging a tumor helps in deciding the optimal management modality and also in determining the prognosis of the disease.

Kadish staging system:

This system stages Esthesioneuroblastoma into three stages:

Stage A: Tumor limited to nasal cavity

Stage B: Tumor extending to paranasal sinuses

Stage C: Tumor extending beyond paranasal sinuses

Major inadequacy of this staging system is too broad a staging at the level of Stage C.

Modified TNM staging system:

Tumor

T1 – tumor involving nasal cavity and / or paranasal sinuses (excluding sphenoid) sparing most of the superior ethmoidal cells

T2- tumor involving nasal cavity and / or paranasal sinuses including sphenoid with extension to the cribriform plate

T3 – tumor extending to orbit with involvement of extradural anterior cranial fossa

T4 – tumor involving brain

Node

N0 – No nodal metastasis

N1 – any form of nodal metastasis

Metastasis

M0 – No metastasis

M1 – Metastasis present

Hyam came out with a histopathological grading system which turned out to be more accurate than the preceding two staging systems.

Hyam's Histopathological grading system

Grade	LA preservation	Mitotic index	Nuclear polymorphism	Fibrillary matrix	Rosettes	Necrosis
I	+	Zero	None	Prominent	HW	None
II	+	Low	Low	Present	HW	None
III	+/-	Moderate	Moderate	Low	FW	Rare
IV	+/-	High	High	Absent	None	Frequent

Fw – Flexner-wintersteiner

HW – Homer – Wright

Management:

This tumor is managed with wide surgical resection with adequate tumor margins. Radiotherapy is indieated as primary treatment modality in unresectable tumors and as a follow up to surgical debulking.

Maxillectomy a review

Maxillectomy a Review

Pathophysiology of allergic fungal sinusitis

Introduction:

Allergic fungal sinusitis is known to cause extensive sinonasal polyposis. This article discusses the pathophysiologic mechanisms involved. Studies have revealed that this is a form of non invasive fungal sinusitis causing sinonasal polyposis. Hypersenstivity to fungus has been implicated as the probable cause. Hypersensitivity to Aspergillus / dematiaceous fungi (this group of fungi secrete melanin in their cell walls) have been commonly implicated to the cause.

Role of hypersensitivity:

Eventhough hypersensitivity to fungus has been implicated as the cause for allergic fungal sinusitis the precise nature of this hypersensitivity is still not clear. Studies reveal that patients with allergic fungal sinusitis show elevated levels of IgE and IgG to fungal proteins. Hence a combination of Coomb's Type I and Type III hypersensitivity to these fungal allergens could play a role.

Type I hypersensitivity reaction is rather immediate and is caused by IgE and IgG4 immunoglobins.

Type III hypersensitivity reaction is known to be causeed by immune complex formation and is caused by IgG and complements.

Current hypothesis suggests that allergic fungal sinusitis develops in susceptible persons due to a combination of local anatomic as well as environmental factors. Fungi enter the nose and trigger immune response causing inflammatory changes. This inflammation induces polyp formation and accumulation of allergic mucin. Fungi which are trapped within the mucosa of nasal cavity causes continuing stimulation of immune reaction causing a vicious cycle leading on to extensive nasal polyp formation.