Keywords
proptosis - orbit - Graves'disease - ethmoid
Introduction
Proptosis is defined as the axial displacement of one or both eyeballs relative to
the skull.[1] An otorhinolaryngologistshould do a thorough evaluation of proptosis. Proptosis
can be an early and, at times, the only manifestation of an ENT pathology. As there
is close anatomical proximity of the orbit to the nose, sinuses, and the nasopharynx,
the majority of ENT pathologies present with ocular manifestations.[2] The various routes attributed to the spread of this pathology are the foramen, fissures,
vascular, erosion, and compression of the anatomical barriers.[3] Radiological investigations play a major role in the diagnosis.[4] Early diagnosis and treatment can revert proptosis in the majority of cases.[5]
Objectives
To determine the demographic characteristics, etiological factors, pathogenesis, and
the various management strategies for proptosis in otorhinolaryngology
Materials and Methods
The present prospective study was conducted at the department of ENT of atertiary
care center in Telangana, India, for the duration of 2 years (June 2017–June 2019).
Sixty cases of proptosis secondary to ENT disorders have been reported. Patients of
all age groups presenting with proptosis due to ENT disorders who have given informed
consent were included in present study. Patients with proptosis due to primary ocular
pathology as well as those who have not given informed consent were excluded from
present study. All patients were subjected to detailed history taking, clinical examination,
diagnostic procedures and were subjected to treatment after signing the informed consent.
Data regarding age, gender, etiology, ocular manifestations and treatment were recorded,
and a statistical analysis was performed.
Ethical Approval
All procedures performed in studies involving human participants were in accordance
with the ethical standards of the institution and with the 1964 Helsinki declaration
and its later amendments or comparable ethical standards.
Results
([Figs. 1–3] and [Tables 1–4]).
Fig. 1 Graph depicting age distribution of study population.
Fig. 2 Gender distribution of study population.
Fig. 3 Paranasal sinuses computed tomography coronal section depicting proptosis of the
right eye resulting due to right ethmoid mucocele causing erosion of the right lamina
papyracea.
Table 1
Etiology distribution of study population
|
Etiology
|
N; %
|
|
Nose and sinuses
|
|
Nasal vestibulitis
|
1; 2%
|
|
Acute bacterial rhinosinusitis
|
3; 5%
|
|
Allergic fungal rhinosinusitis
|
6; 10%
|
|
Acute invasive fungal sinusitis
|
1; 2%
|
|
Fungal granuloma
|
3; 5%
|
|
Wegener granulomatosis
|
1; 2%
|
|
Ethmoid mucocele
|
2; 3%
|
|
Sinonasalschwannoma
|
2; 3%
|
|
Inverted papilloma
|
1; 2%
|
|
Frontal sinus osteoma
|
1; 2%
|
|
Fibrous dysplasia of maxilla
|
3; 5%
|
|
Fibrous dysplasia of ethmoid
|
1; 2%
|
|
Sinonasalsquamous cell carcinoma
|
12; 20%
|
|
Sinonasaladenocarcinoma
|
2; 3%
|
|
Sinonasaladenoid cystic carcinoma
|
2; 3%
|
|
Sinonasalembryonal rhabdomyosarcoma
|
1; 2%
|
|
Olfactory neuroblastoma
|
1; 2%
|
|
Nasopharynx
|
|
Juvenile nasopharyngeal angiofibroma
|
7; 11%
|
|
Nasopharyngeal carcinoma
|
4; 6%
|
|
Thyroid
|
|
Graves' disease
|
6; 10%
|
Table 2
Etiopathogenesis of study population
|
Etiology
|
Pathogenesis
|
|
Nose and sinuses
|
|
Nasal vestibulitis
|
Vascular (1; 2%)
|
|
Acute bacterial rhinosinusitis
|
Vascular (2; 3%)
Compression (1; 2%)
|
|
Allergic fungal rhinosinusitis
|
Compression (6; 10%)
|
|
Acute invasive fungal sinusitis
|
Erosion (1; 2%)
|
|
Fungal granuloma
|
Compression (1; 2%)
Erosion (2; 3%)
|
|
Wegener granulomatosis
|
Erosion (1; 2%)
|
|
Ethmoid mucocele
|
Compression (1; 2%)
Erosion (1; 2%)
|
|
Sinonasalschwannoma
|
Compression (1; 2%)
Erosion (1; 2%)
|
|
Inverted papilloma
|
Erosion (1; 2%)
|
|
Frontal sinus osteoma
|
Compression (1; 2%)
|
|
Fibrous dysplasia of maxilla
|
Compression (3; 5%)
|
|
Fibrous dysplasia of ethmoid
|
Compression (1; 2%)
|
|
Sinonasalsquamous cell carcinoma
|
Erosion (12; 20%)
|
|
Sinonasaladenocarcinoma
|
Erosion (2; 3%)
|
|
Sinonasaladenoid cystic carcinoma
|
Erosion (2; 3%)
|
|
Sinonasalembryonal rhabdomyosarcoma
|
Erosion (1; 2%)
|
|
Olfactory neuroblastoma
|
Erosion (1; 2%)
|
|
Nasopharynx
|
|
Juvenile nasopharyngeal angiofibroma
|
Erosion (1; 2%)
Compression (3; 5%)
Spread via fissures (3; 5%)
|
|
Nasopharyngeal carcinoma
|
Erosion (2; 3%)
Spread via fissures (2; 3%)
|
|
Thyroid
|
|
Graves' disease
|
Increased intraconal fat (6; 10%)
|
|
TOTAL:
Erosion: 28; 47%
Compression: 18; 30%
Vascular causing cavernous sinus involvement: 3; 5%
Spread via fissures: 5; 8%
Increased intraconal Fat: 6; 10%
|
Table 3
Non-surgical treatment distribution of study population
|
Etiology
|
Non-surgical management
|
|
Nose & sinuses
|
|
Nasal vestibulitis
|
IV antibiotics (1; 2%)
IV steroids (1; 2%)
|
|
Acute bacterial rhinosinusitis
|
IV antibiotics (3; 5%)
IV steroids (2; 3%)
|
|
Allergic fungal rhinosinusitis
|
Intranasal steroids (6, 10%)
|
|
Acute invasive fungal sinusitis
|
IV amphotericin B (1; 2%)
Oral itraconazole (1, 2%)
|
|
Fungal granuloma
|
IV amphotericin B (3; 5%)
Oral itraconazole (3; 5%)
|
|
Wegener granulomatosis
|
Oral steroids (1, 2%)
Oral cyclosporin (1; 2%)
|
|
Ethmoid mucocele
|
–
|
|
Sinonasal schwannoma
|
–
|
|
Inverted papilloma
|
–
|
|
Frontal sinus osteoma
|
–
|
|
Fibrous dysplasia of maxilla
|
–
|
|
Fibrous dysplasia of ethmoid
|
–
|
|
Sinonasal squamous cell carcinoma
|
Radiotherapy
(10; 17%)
Postoperative radiotherapy
(2; 3%)
|
|
Sinonasal adenocarcinoma
|
–
|
|
Sinonasal adenoid cystic carcinoma
|
Postoperative radiotherapy
(2; 3%)
|
|
Sinonasal embryonal rhabdomyosarcoma
|
Chemotherapy
(1; 2%)
|
|
Olfactory neuroblastoma
|
Postoperative radiotherapy
(1; 2%)
|
|
NASOPHARYNX
|
|
Juvenile nasopharyngeal angiofibroma
|
_
|
|
Nasopharyngeal carcinoma
|
Radiotherapy (4; 6%)
|
|
Thyroid
|
|
Graves' disease
|
Antithyroid drugs (6; 10%)
|
|
TOTAL:
Antibiotics: IV (4; 6%)
Steroids: Oral (1; 2%), IV (3; 5%), intranasal (6; 10%) = 10, 17%
Immunosuppressive: Oral cyclosporin (1; 2%)
Antifungals: IV amphotericin B (4; 6%), Oral itraconazole (4; 6%)
Antithyroid drugs: 6; 10%
Radiotherapy: 19; 32% of which 5; 8% were postoperative radiotherapy
Chemotherapy: 1; 2%
|
Abbreviation: IV, intravenous.
Table 4
Surgical treatment of study population
|
Etiology
|
Surgery
|
|
Nose & sinuses
|
|
Nasal vestibulitis
|
Incision &drainage (1; 2%)
|
|
Acute bacterial rhinosinusitis
|
Functional endoscopic sinus surgery (3; 5%)
|
|
Allergic fungal rhinosinusitis
|
Functional endoscopic sinus surgery (6; 10%)
|
|
Acute invasive fungal sinusitis
|
Endoscopic sinus surgery and debridement (1; 2%)
|
|
Fungal granuloma
|
Endoscopic sinus surgery (3; 5%)
|
|
Wegener granulomatosis
|
Endoscopic sinus surgery (1; 2%)
|
|
Ethmoid mucocele
|
Functional endoscopic sinus surgery (2; 3%)
|
|
Sinonasalschwannoma
|
Endoscopic resection (2;3%)
|
|
Inverted papilloma
|
Endoscopic resection (1; 2%)
|
|
Frontal sinus osteoma
|
Bicoronalincision and excision (1; 2%)
|
|
Fibrous dysplasia of maxilla
|
Lateral rhinotomy &paring (3; 5%)
|
|
Fibrous dysplasia of ethmoid
|
Lynch-Howarth external approach and removal (1,; 2%)
|
|
Sinonasalsquamous cell carcinoma
|
Total maxillectomy (2; 3%)
|
|
Sinonasaladenocarcinoma
|
Total maxillectomy (2; 3%)
|
|
Sinonasaladenoid cystic carcinoma
|
Total maxillectomy (2; 3%)
|
|
Sinonasalembryonal rhabdomyosarcoma
|
–
|
|
Olfactory neuroblastoma
|
Endoscopic resection (1; 2%)
|
|
Nasopharynx
|
|
Juvenile nasopharyngeal angiofibroma
|
Endoscopic surgery with coblation (2; 3%)
Lateral rhinotomy (5, 8%)
|
|
Nasopharyngeal carcinoma
|
–
|
|
Thyroid
|
|
Graves' disease
|
Orbital decompression (2; 3%)
Subtotal thyroidectomy (6; 10%)
|
|
TOTAL:
Incision and drainage: 1; 2%
Functional endoscopic sinus surgery: 11; 18%
Endoscopic sinus surgery: 5; 8%
Endoscopic resection: 4; 6%
Bicoronal incision & excision: 1; 2%
Lynch-Howarth external approach & removal: 1; 2%
Lateral rhinotomy: 8; 13%
Total maxillectomy: 6; 10%
Endoscopic coblation surgery: 2; 3%
Orbital decompression: 2; 3%
Subtotal thyroidectomy: 6; 10%
|
Discussion
Sixty cases of ENT disorders leading to proptosis as one of the manifestations are
reported in the present study.
Demographics
In the present study, the highest incidence of proptosis was noted in elderly patients(>
50 years old group), accounting for 22 cases (37%);this was followed by tmiddle-aged
patients(31–50 years old group), accounting for 17 cases (28%), followed by the young
patientsgroup (16–30 years old), accounting for 13 cases (22%). The lowest incidence
was noted in in the group of patients aged 0–15 years old, accounting for 8 cases
(13%). These data aresimilar tothe ones presented in the study by Venugopal et al,[6] in which the incidence of proptosis secondary to ENT disorders increased with advancing
age.
The highest incidence of proptosis secondary to ENT disorders was noted in male patients,
accounting for 40 cases (67%). In female patients, it accounted for 20 cases (33%).
This is similar tothe data presented in the study by Sinha et al.[3] The male to female ratio was 2:1.
Etiology
In the present study, the anatomical categorization was nose, sinuses, nasopharynx,
and thyroid, the pathology of which lead to one of the clinical manifestations of
proptosis. In the nose and sinuses, the infective causes for proptosis were nasal
vestibulitis (1; 2%), acute bacterial rhinosinusitis (3;5%), allergic fungal rhinosinusitis
(6; 10%), and acute invasive fungal sinusitis (1; 2%). The granulomatous causes were
fungal granuloma (3; 5%), and Wegener granulomatosis (1; 2%). The most common infectious
etiology was Allergic fungal rhinosinusitis, and the most common granulomatous etiology
was fungal granuloma. Sinonasal infections accounted for 15 cases (25%). In the nose
and sinuses, the benign causes for proptosis were frontal sinus osteoma (1; 2%); fibrous
dysplasia of the ethmoid sinus (1; 2%); fibrous dysplasia of the maxilla (3; 5%);
sinonasal inverted papilloma (1; 2%); and sinonasal schwannoma (2; 3%). In the nasopharynx,
the benign cause for proptosis was juvenile nasopharyngeal angiofibroma (7; 11%).
In the present study, there were 4 fibrous dysplasia cases presenting as proptosis.
Moore et al[7] have reported 16 cases of fibrous dysplasia presenting as proptosis. The most common
of benign tumor was juvenile nasopharyngeal angiofibroma. This is in accordance with
the study by Sinha et al.[3] Sinonasal and nasopharyngeal benign tumors accounted for 15 cases (25%). In the
nose and sinuses, the malignant causes for proptosis were sinonasal squamous cell
carcinoma (12; 20%); sinonasal adenocarcinoma (2; 3%); sinonasal adenoid cystic carcinoma
(2; 3%); sinonasal embryonal rhabdomyosarcoma (1; 2%); and olfactory neuroblastoma
(1,;2%). In the nasopharynx, the malignant cause for proptosis was nasopharyngeal
carcinoma (4; 6%). The most common of malignant tumor was sinonasal squamous cell
carcinoma. This is in accordance with the studies by Conley et al,[5] Sabharwal et al,[8] Sayed et al,[9] and Johnson et al.[10] Sinonasal and nasopharyngeal malignant tumors accounted for 22 cases (37%). The
miscellaneous causes for proptosis were ethmoid mucocele (2; 3%) and Graves' disease
(6; 10%). It was evident that malignant tumors, especially sinonasal squamous cell
carcinoma, presented most commonly with proptosis manifestation, followed by juvenile
nasopharyngeal angiofibroma and allergic fungal rhinosinusitis.
Pathogenesis
In the present study, the various pathogeneses for proptosis were erosion of anatomical
barriers (28; 47%), compression of anatomical barriers (18; 30%); vascular spread
leading to cavernous sinus involvement (3; 5%); spread via fissures(5; 8%); and increased
intraconal fat (6; 10%). The computed tomography (CT), magnetic resonance imaging
(MRI), and surgical exploration have played a role in identifying the various routes
of involvement of the orbit that led to proptosis as one of the manifestations. The
most common route was erosion of the anatomical barriers followed by compression of
the anatomical barriers. the majority of malignant tumors were responsible for erosion
of the anatomical barriers, whereas the majority of benign tumors and infective causes
were responsible for compression of the anatomical barriers. One case of nasal vestibulitis
and 2 cases of acute bacterial rhinosinusitis presented as cavernous sinus involvement,
suggesting the valvelessnature of communicating veins. This finding is similar tothat
reported in the study by Canon et al,[11] according to which sinusitis and untreated nasal vestibulitis can complicate to
cavernous sinus thrombosis.
Management
Five cases (8%) were treated with non-surgical modalities alone, of which 1 case was
of sinonasal embryonal rhabdomyosarcoma, and 4 cases were of nasopharyngeal carcinoma.
Nineteen cases (32%) were treated with surgical modality alone, 7 of which were cases
of juvenile nasopharyngeal angiofibroma, 2 cases were of sinonasaladenocarcinoma,
3 cases were of fibrous dysplasia of maxilla, 1 case was of fibrous dysplasia of ethmoid,
1 case was of frontal sinus osteoma, 1 case was of inverted papilloma, 2 cases were
of sinonasal schwannoma, and 2 cases were of ethmoid mucocele. Thirty-six cases (60%)
required a combined modality of both non-surgical and surgical treatments.
Non-surgical Measures
Nineteen cases (32%) were treated with radiotherapy, of which 10 cases were of sinonasal
squamous cell carcinoma, 4 cases were of nasopharyngeal carcinoma, and 5 cases were
given postoperative radiotherapy (1 case of olfactory neuroblastoma, 2 cases of sinonasal
squamous cell carcinoma, 2 cases of sinonasal adenoid cystic carcinoma). Ten cases
(17%) were treated with steroids, of which 1 case was of nasal vestibulitis, 2 cases
of acute bacterial rhinosinusitis were treated with intravenous steroids, 6 cases
of allergic fungal rhinosinusitis were treated with intranasal steroids, and 1 case
of Wegener granulomatosis was treated with oral steroids. Four cases (6%) were treated
with antifungals, of which 1 case was of acute invasive fungal sinusitis, and 3 cases
of fungal granuloma were treated with both IV amphotericin B and oral itraconazole.
Four cases(6%) were treated with intravenous antibiotics, of which 1 case was of nasal
vestibulitis and 3 cases were of acute bacterial rhinosinusitis. Cavernous sinus involvement
cases (3) were treated with intravenous antibiotics and intravenous steroids. This
is similar tothat reported in the study by Abhay et al.[12] One case of Wegener granulomatosis was also given oral cyclosporin. One case of
sinonasal embryonal rhabdomyosarcoma was treated with chemotherapy. All 6 cases of
Graves' disease (10%) were treated with antithyroid drugs.
Surgical Measures
Eleven cases (18%) were treated with functional endoscopic sinus surgery, of which
3 cases were of acute bacterial rhinosinusitis, 6 cases were of allergic fungal rhinosinusitis,
and 2 cases were of ethmoid mucocele. Six cases (10%) were treated with total maxillectomy,
of which 2 cases were of sinonasal squamous cell carcinoma, 2 cases were of sinonasal
adenoid cystic carcinoma, and 2 cases were of sinonasal adenocarcinoma. Three cases
required orbital exenteration (2 cases of sinonasal squamous cell carcinoma and 1
case of sinonasal adenoid cystic carcinoma). Eight cases (13%) were treated with a
lateral rhinotomy approach, of which 5 cases were of juvenile nasopharyngeal carcinoma,
and 3 cases were of fibrous dysplasia of maxilla, in which paring was done. Five cases
(8%) were treated with endoscopic sinus surgery, of which 1 case was of acute invasive
fungal sinusitis, in which debridement was done, 3 cases were of fungal granuloma,
and 1 case was of Wegener granulomatosis. In 4 cases (6%), endoscopic resection was
done;out of these, 1 case was of inverted papilloma, 1 case was of olfactory neuroblastoma,
and 2 cases were of sinonasal schwannoma. All 6 cases of Graves' disease were treated
with subtotal thyroidectomy, of which 2 cases also required orbital decompression
as an emergency treatment to prevent loss of vision due to increasing intraorbital
pressure. Nasal vestibulitis (1 case) required incision and drainage. Frontal sinus
osteoma (1 case) managed by bicoronalincision and excision. Fibrous dysplasia of ethmoidsinus
(1 case) was managed by Lynch-Howarth external approach and removal. Two cases of
nasopharyngeal angiofibroma were treated with endoscopic coblation method.
Conclusion
The majority of nose, sinuses, nasopharyngeal and thyroid pathologies can present
proptosis as one of the clinical manifestations. The resultant compression of the
orbit can raise the intraorbitalpressure, thus leading to increased mortality and
morbidity (blindness). Hence, it is essential for an otorhinolaryngologistand ophthalmologist
to thoroughly evaluate the proptosis and work as a team to diagnose and manage this
disorder at the earliest to prevent blindness as well as to address the cosmetic defect.
