Journal of Postgraduate Medicine
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Year : 1989  |  Volume : 35  |  Issue : 2  |  Page : 93-7  

Profile of congenital cholesteatomas of the petrous apex.

CE DeSouza, CO Menezes, RA DeSouza, SB Ogale, MM Morris, AP Desai 
 

Correspondence Address:
C E DeSouza


Abstract

Congenital cholesteatomas of the petrous apex are now frequently being approached by otologists. Involvement of the cerebello pontine angle by this lesion produces a myriad of signs and symptoms. Otological as well as neurosurgical literature is reviewed to achieve an overall understanding of the nature and behaviour of this uncommon but interesting lesion.



How to cite this article:
DeSouza C E, Menezes C O, DeSouza R A, Ogale S B, Morris M M, Desai A P. Profile of congenital cholesteatomas of the petrous apex. J Postgrad Med 1989;35:93-7


How to cite this URL:
DeSouza C E, Menezes C O, DeSouza R A, Ogale S B, Morris M M, Desai A P. Profile of congenital cholesteatomas of the petrous apex. J Postgrad Med [serial online] 1989 [cited 2021 Jan 20 ];35:93-7
Available from: https://www.jpgmonline.com/text.asp?1989/35/2/93/5702


Full Text



 INTRODUCTION



Cholesteatomas are well known to cause otorrhea and deafness. However, congenital cholesteatomas of the petrous apex very rarely cause otorrhea. They often have to be distinguished from other lesions that might occur in the cerebello-pontine angle. They can present in an unusual fashion and can be missed. However, an alert otolaryngologist with an understanding of their behaviour will not be misled.

Embryology

Congenital cholesteatomas may arise from the embryonic inclusions of the epidermal portion of the epiblasts during the closure of the neural tube.[30],[43] Trauma or differentiation from multipotential cell rests[47] may also be responsible. Nager[40] refers to cholesteatomas simply as aberrant epithelial remnants and therefore blastomatous malformations. Gacek[23] and Delozier et al[16] argued that cholesteatomas of the petrous apex originate from epithelial cells from Seesels pouch which get encased within the fibrocartilage of the foramen lacerum during cephalic flexure and may give rise to cholesteatomas in later life. Aimi[2] believes that petrous cholesteatomas are neuroectodermal in origin. They have also been postulated to arise from pial tissues in the plexus tufts.[4] Cholesteatomas develop in the posterior basal cistern. Their capsule consists of a thickening of arachnoid matter and develop within the arachnoid mater.[46]

A true congenital cholesteatoma is that which has its nidus of squamous epithelium present at birth and may be found anywhere in the temporal bone depending on where the cell rests may be situated. Such cholesteatomas are sterile but can expand and erode bone, implying that bacterial infection per se is not an essential aspect of cholesteatoma advancement 38 [Fig. 1].

Presentation

It is well known that congenital cholesteatomas of the petrous apex may occur without otorrhea and in an otherwise normal temporal bone.[20],[21]

Facial nerve deficits are the first lesions to occur followed by a unilateral hearing loss.[37]

Peron and Schukneckt[44] and Kerr and Smyth[34] on the other hand found that the commonest presenting symptom was unilateral hearing loss, followed by palsies of the 7th nerve.

Facial paralysis occurs more often in congenital cholesteatomas than in an acoustic neuroma because the cholesteatoma tends to encircle the 7th nerve and interfere with its blood supply.[3]

In some reports, headaches were the commonest symptom[27] while others stress the long duration of complaints and note that the lesion was often found to predominate in age groups between 20-40 years.[11],[56]

There have been reports where trigeminal neuralgia was the common presenting symptom,[5],[31],[33] while others note that mental changes occurred more often.[49]

Incidence Congenital cholesteatomas are relatively uncommon lesions of the cerebello-pontine angle.[18] Granick et al[26] found that cerebello-pontine angle cholesteatomas formed only 2% of his cases. They have been found to account for 0.2% to 1.0% of neoplasms of the central nervous systems.[8] The commonest tumours found at the cerebello-pontine ankle are acoustic neuromas[24],[48]

Interestingly, Behnke and Schindler[6] found cholesteatomas to be commoner than acoustic neuromas in their series. Scott[51] found the cerebello-pontine angle to be the commonest site at which congenital cholesteatomas were found. Nager40 found the majority (30-40%) of congenital cholesteatomas were situated in the cerebellopontine angle. The remaining were distributed to the optic, chiasma, diploe of the skull and the rhomboid fossa.

Peculiar Features

Recent biomathematical analysis suggest that cholesteatomatous lesions grow linearly at rates approximating those reported for skin rather than exponentially as most tumours do.[35] It is found that the protein content in the cerebro-spinal fluid is usually not elevated.[24],[55]

There are many reports in literature of the lesion undergoing a malignant transformation.[22],[36],[41],[52],[53],[67]

On CT scanning hyperdense cholesteatoma have also been reported.[17],[29],[39] There is an isolated report of cholesteatoma with an associated intracranial haemorrhage.[1] If its contents leak into the subarachnoid space, it can also cause aseptic or chemical meningitis.[7],[10],[50],[54]

Neuro-radio-diagnostic investigations

Before the advent of CT scanning, pneumo-encephalography was responsible for outlining the entire tumour and its relationship to adjacent structures.[14] A typical filigree appearance was seen.

CT scanning is an important investigation for various lesions involving the cerebello-pontine angle.[19],[28] CT scanning will show its various extensions and its effects on neural, vascular and cranial structures. It may erode into the ear[45] or may fill the entire ipsilateral posterior and middle fossae.[25]

The attenuation numbers of cholesteatomas (approximately -2 to + 10 Hounsfield units) and cerebrospinal fluid density are similar, so it is the mass effect that is most important when diagnosing a cholesteatoma[13] [Fig. 2]

The patients must be thoroughly investigated to locate the extent and site of the lesion to decide the surgical approach, otherwise an unsuitable approach might prove disasterous.[9]

For despite the absence of localizing signs, such as 7th and 8th cranial nerve involvement, the lesion may be extensive.[12]

Treatment

The treatment of cholesteatomas of the petrous apex or anywhere else for that matter is always surgery.

Paparella and Rybak[43] suggest fistulization of the cholesteatoma into the ear. A case has been reported where the cholesteatoma was draining into the middle ear. Despite this, hearing was only marginally affected. The cholesteatoma continued to grow but because of the fistula keratin debris did not accumulate.[32] However, this may lead to an ascending infection into the cranial cavity and may cause meningitis, encephalitis or abscess formation.

Radical removal of the disease is the solution.[15] The residual epithelium can continue growing if it is not totally excised and the cholesteatoma can reform.

 CONCLUSION



Congenital cholesteatomas behave exactly like the acquired cholesteatomas that occur in the mastoid with the exception that they are sterile. If a patient presents with lesion that is suggestive of a pathology in the cerebello-pontine angle lesion, congenital cholesteatomas should be thought of as a possible pathology.

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