Volume 4, Issue 3 , Pages 129-133, September 2009
Cholesterol granuloma of the maxillary sinus in a young patient with associated neurosurgical pathology
Article Outline
Summary
The cholesterol granuloma is a frequent exhibit in otomastoid inflammatory pathology. The evidence of such in the nasal-sinus region is rarely described in the literature. The diagnosis is usually difficult because the clinical, endoscopic and radiological findings are non-specific. In our study we are describing a case of cholesterol granuloma of the maxillary sinus found in a 16-year-old who presented an endoscopic exhibit suggesting antrochoanal polyp. The CT scan also showed an arachnoid cyst that caused frequent epileptic episodes in the patient. The neoformation was therefore extracted via endonasal endoscopy including the base of implantation. A few days after the boy was discharged he was hospitalized again because of the appearance of a chronic subdural hematoma and was treated by neurosurgical drainage.
The clinical, radiological and histopathological characteristics of the cholesterol granuloma of the maxillary sinus are largely discussed researching the possible correlations with the associated neurosurgical pathology.
Keywords: Cholesterol granuloma, Arachnoid cyst, Subdural hematoma, Endoscopic sinus surgery, Antrochoanal polyp
1. Introduction
The cholesterol granuloma is an anatomical–pathological exhibit frequently found in chronic pathologies of the middle ear where it represents a granulomatous tissue reaction, which occurs during the inflammatory phase of the pathology because of the obstruction of the otomastoid aerated spaces. Finding of such in the paranasal sinuses is extremely rare: Graham and Michaels in 1978 first described cases of cholesterol granuloma of the maxillary sinus [1], but the future references in the literature are exceptional [2].
The etiopathogenetic factors that are called into question are fundamentally three: altered ventilation of the sinus, impaired drainage of the secretions and a hemorrhage inside the sinus cavity with consequential hemolysis and precipitation of cholesterol.
The diagnosis is difficult because the symptoms are usually non-specific (facial pain, yellow rhinorrhea, unilateral nasal obstruction, cefalea) and endoscopic signs (a polyoid mass emerging from the ostio-meatal complex) and radiological (a cyst-like or massive opacification of the maxillary sinus, exhibits of bony erosion) which cause problems in the differential diagnosis with other pathologies such as mucocele, non-secreting cysts, chronic sinusitis [3] and in rare cases a malignant pathology of the maxillary sinus [4]. Because it is a chronic monolateral disease of the maxillary sinus, a surgical therapy using the Caldwell–Luc technique has been used in the past for the radical removal of the process. As of now an endoscopic transnasal approach is largely used in benign pathologies, and in limited cases, even in malignant cases of the paranasal sinus, which have been giving excellent results even in the treatment of cholesterol granulomas [2], [5], [6], [7].
In this study we are presenting a rare case of cholesterol granuloma of the maxillary sinus with onset at a very young age. It is, in fact, a 16-year-old boy with coagulatory problems who associated to this disease had a conjoined arachnoid cyst and that had a complex surgical procedure: 10 days after the endoscopic treatment on the sinus the patient had to in fact undergo an emergency neurosurgical treatment to drain a subdural hematoma.
This study has examined the clinical, radiological and histopathological characteristics as well as the possible correlation between the two diseases.
2. Case report
In our case study the 16-year-old boy was in treatment in the infant neuropsychiatric department of our University hospital for the appearance of significant epileptic episodes characterized by loss of consciousness, trouble breathing, generalized sudden stiffness especially in the upper limbs, followed by tonic–clonic contractions that lasted a few minutes with drooling from the mouth. Upon awakening the patient was disoriented and confused. A CT scan showed minor dimensional asymmetry of the lateral ventricles, prevailing on the right side and an amplification of the right frontal-lateral periencephalic liquoral spaces of about 15
mm×10mm which conconmitated a slight thinning of the adjacent bony plane. Such exhibit, compatible with the diagnosis of the arachnoid cyst, was confirmed by the MRI scan (Fig. 1). Both exams showed a maxillary sinus opacification on the right side. The hematochemical tests were normal except for a low prothrombin activity (56% n.v. 70–120) and a slight reduction of red blood cells (4,390,000 n.v. 4,500,000–6,100,000), and a low cholesterol level (134 n.v. 140–200).
Fig. 1.
Coronal MRI shows the mass of the left maxillary sinus that emerges from the ostio-meatal complex reaching the nasal fossa. Associated with this is the dilation of the subarachnoid spaces in the right frontal region with scalloping of the internal bony trabeculae.
For the sinus pathology report the patient was sent to the Department of Otolaryngology for a consult: from the case history reoccurring episodes emerged (2–3 times a year) of complete obstruction of the left nasal fossa, beginning at the age of 6, treated with oral antihistamines and cortisone nasal spray. A first standard X-ray at the age of 10 showed signs of a left maxillary sinusitis.
For about 3 years mucous rhinnorhea was also present, constant during the year, even though it was less accentuated during the summer months, associated with a reoccurring headache and light but constant left nasal obstruction and pressure sensation on the left side of the face, especially in the maxillary region. Data regarding one episode of epistaxis almost every month is important and not due to secondary traumas or colds.
The fibroendoscopy exam showed a grayish polypoid mass coming from the left ostio-meatal complex and invading the nasal cavity. Such evidence, associated with a case CT and MRI with massive opacification of the left maxillary sinus and a soft tissue density mass that went from the middle meatus to the nasal septum, without signs of bony erosion, was orientating for a diagnosis of a left antrochoanal polyp, given the young age of the patient.
The removal of the tumor via sino-nasal endoscopy under general anesthesia via a maxillary antrostomy was therefore solicited; the mass was removed in one block with the implantation site positioned in the corresponding posterior-lateral angle of the sinus. The histological exam of the tumor showed that in the soft edematous connective tissue, many clusters of cholesterol crystals formed characteristic clefts, surrounded by multinucleated giant cells. Also present were erythrocytes, foamy histiocytes, and lymphomononuclear inflammatory infiltrate (Fig. 2). This histological overview was suggestive of a cholesterol granuloma of the maxillary sinus.
Fig. 2.
Cholesterol granuloma characterized by numerous dense masses of cholesterol crystals surrounded by multinucleated giant cells. Also present, extravasated erythrocytes, foamy histiocytes and lymphomononuclear inflammatory infiltration in the context of edematous tissue (hematoxylin–eosin stain, ×40).
The patient was discharged the next day in an absolutely healthy state. About 10 days after the patient was discharged, he was hospitalized again in infant neuropsychiatric department because of a sudden headache in the right frontal–temporal–parietal region associated with mental confusion. From an emergency cranial CT scan emerged a vast layer of chronic extra-axial hematoma (seemingly subdural) in the right frontal-parietal region with hematic hyperdensity and a discreet right-left shift of the median structures; the hematoma was reported by the radiologist as chronic and the arachnoid cyst that was previously found in the right frontal region was not visible any longer (Fig. 3). The hematochemical tests confirmed the previous reduced prothrombin activity with similar values (54%). The boy was therefore taken to the neurosurgery department for a low right parietal craniotomy to evacuate the hematoma. The check-up CT scan, done 20 days after discharge, did not show signs of endocranial build-up or the presence of any tumor (Fig. 4). An endoscopic follow-up after 12 months excluded reoccurring disease in the maxillary sinus that was previously related to the cholesterol granuloma.
Fig. 3.
CT with contrast shows a vast layer of the chronic right frontal–parietal subdural hematoma with hematic hyperdensity and right-left shift of the median structures.
Fig. 4.
CT scan 20 days post-operatively: complete reabsorption of the hematoma and the disappearance of the arachnoid cyst whose previous presence is proven by the minus on the internal bony trabeculae.
3. Discussion
The cholesterol granuloma is a typical disease of the mastoid antrum and of the other aerated portions of the temporal bone. However, even though rarely, it is possibly found in other organs such as the sternum, the sella turcica and the paranasal sinuses [4]. The cases where it is localized in the maxillary sinus are described in middle-aged men (range 27–56 years; median age 38 years) [7], and in one exceptional case in a 14-year-old boy [8]. The expression of this disease at an adolescent age, found even in our study, is due to the unusually precocious etiopathogenetic mechanisms that are hypothesized.
Such mechanisms can be synthesized in the reduced ventilation and/or in the altered drainage of the sinus and in hemorrhagic phenomenons in the maxillary sinus, with hemolysis and consequential precipitation of cholesterol. This substance, coming from the destroyed cellular membranes of the erythrocytes, crystallizes because of the slow drainage of the sinus (it in fact requires much time to dissociate from the lipoprotein and precipitate), causing the inflammatory process of the adjacent tissues and the formation of the granulomas. This mechanism is clearly deductible from the histological picture in which many clefts appear, formed from the cholesterol crystals that dissolved during the processing, around which a granulomatous reaction forms with foreign body giant cells, macrophages containing hemosiderin, dilated lymphatic vessels and erythrocytes, signs of more or less recent bleeding. Such histological characteristics and random mechanisms have been widely studied by Shvili in a clinical-pathological study that specifically regarded the cholesterol granulomas present in antrochoanal polyps [9].
If a situation with altered sinus ventilation occurs very prematurely, the intervention of other pathogenetic factors, in particular an intrasinus hemorrhage, could cause the eruption of the cholesterol granuloma at an adolescent age. In our case in fact a history of sinopathy was already present at the age of 6 and objectified with a radiological exam at the age of 10. A hemorrhagic deposition into the sinus is therefore very probable, considering the coagulatory alteration found in our patient more than once.
The symptoms of the CG are notably non-specific since the nasal obstruction, rhinnorhea and facial pain that characterize it are similar to many inflammatory sinopathies.
Some authors recognize the clear golden yellow rhinnorhea (found even in the sinus lavage fluid) as the only specific sign of the disease [2]. In our study however the rhinnorhea did not have the characteristics since it was clear serosa or mucus. At the same level with the other case that arose in an adolescent age [8], the prevailing symptoms were nasal obstruction and episodes of epistaxis. The boy moreover referred to have a worsening disturbance to the left side of his face but without an algesic component that was instead described in many other studies [1], [3], [4], [7], [10]. In our study this phenomenon was most likely due to the growing of the lesion towards the nasal fossa, and therefore with scarce pressure on the sinus wall.
The polypoid appearance, the grayish color and the extension of the common meatus through the natural ostium of the maxillary sinus are peculiarities of our case since they are only reported in very few cases [7], [11], [12], [13]; the most common is in fact the intramaxillarly cystic appearance that is prevailingly a bluish, yellowish or brown color [2]. The objective findings pre and during surgery was most likely suggesting an antrochoanal polyp with an implantation site narrowly localized in the anterior-lateral angle of the sinus.
Even the imaging methods are of scarce help in the differential diagnosis with cystic lesions, by retention or from a dental origin, mucoceles, neoplasm and chronic inflammatory processes of the sinus mucosa. Infact CG presents in most cases a CT image of cyst-like or a massive opacification of the sinus, that in some cases (as in ours) even involves the ostio-meatal complex. An MRI scan would therefore be useful to complete a correct diagnosis that is capable of identifying the characteristic findings of increased signal intensity in both T1 and T2 weighted images [7] especially in the presence of signs of bony erosion or of the invasion of the neighboring structures, elements that are highly suggestive of a malignant disease [4]. However a histological diagnosis is fundamental in this pathology. In our case, according to the endoscopic and radiological picture, the differential diagnosis could be among chronic inflammatory polyp of the sinus mucosa, antro-choanal polyp and benignant neoplasm (inverted papilloma). A hypothesis of a malignant tumor has been excluded because of the regular radiological aspect of the mass without bone erosion while a simple cystic lesion or a mucocele should be contained into the maxillary sinus. Only a histological exam revealed the rare diagnosis of cholesterol granuloma.
In the past the treatment used to eradicate this neoformation called upon the Caldwell–Luc technique and in rare cases a lateral rhinotomy [2]. In the last period the surgical technique utilizes exclusively a nasal-sinus endoscopy [2], [5], [6], [7], which we as well have used by means of a rigid optic fiber of 30° to check the interested maxillary sinus. This technique has given us the optimum visualization of the disease and in particular of its implantation site, for which it was possible to completely eradication through the widening of the natural ostium of the sinus. In all the studies such approach has had an optimal result, with the disappearance of symptoms and relapses at the follow-up (in our case after 12 months).
Another peculiarity of our case is the appearance of a subdural hematoma, defined as chronic, a short time after the paranasal surgery, on the same side in which during the preoperative tests an arachnoid cyst was present, an event not due to surgical trauma since no contusional maneuvers was performed intraoperatively. The chronic subdural hematoma is a typical pathology in the elderly, tied to the alteration of the meninges vessels in these patients. In accordance with the neurosurgeon, we can hypothesize that in our case the hematoma derived from the rupture of a vessel in the arachnoid cyst which, because of the effect of the altered coagulation parameters, caused a continuous hematic flow and consequential collection. The arachnoid cyst is in effect a predisposing factor to endocranial hemorrhages but these are usually acute and a consequence of traumas, therefore never mentioned by the patient. The disappearance of the arachnoid cyst in the post-operative imaging exams is probably secondary to the rupture and to its subsequent crushing on the bony wall by the encephalic mass. The cholesterol granuloma and the chronic hematoma that we verified in our patient have, therefore, probably had a casual common factor, which was caused by the coagulation defect.
4. Conclusion
The cholesterol granuloma of the maxillary sinus is a difficult disease to diagnose without the auxiliary help of a histological exam because of the non-specific symptoms and the endoscopic and radiological signs similar to other diseases (neoplastic or inflammatory). However the coexistence of the sinusitis symptoms (nasal obstruction, rhinorrhea and facial pain) and reoccurring epistaxis episodes in a subject with partial or total opacification of the maxillary sinus in a CT scan must induce the suspicion of a cholesterol granuloma. In this case it would be useful, especially in a young patient, to evaluate the coagulation parameters, if it is suspected that the granuloma could either prelude or coexist with other more serious diseases caused by the coagulatory disease.
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PII: S1871-4048(08)00070-1
doi:10.1016/j.pedex.2008.10.001
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 3 , Pages 129-133, September 2009
