20/20 to NLP due to Blowing the Nose and Orbital Emphysema: A Case Report

Abstract

Introduction

Orbital emphysema is characterized by presence of air in the soft tissues of the orbits. In severe cases, this can lead to orbital compartment syndrome, a medical emergency that can result in severe permanent vision loss without urgent intervention. Generally, the severity of presenting vision loss and longer time to intervention have been associated with worse visual outcome.

Case Presentation

We present the case of a 26-year-old healthy male who developed an acute orbital compartment syndrome with orbital emphysema following nose blowing in the setting of a bacterial orbital cellulitis. Once documented to have no light perception (NLP) vision in the affected eye, he was urgently taken to the operating room for surgical drainage (approximately 2.5 h after onset of the NLP). Over the subsequent days, he gradually regained 20/20 visual acuity.

Conclusion

This case highlights that despite complete loss of vision, this patient had an excellent visual outcome.

Introduction

Orbital emphysema is a condition characterized by the presence of air within the soft tissues of the orbit [1]. Most cases occur in the context of trauma or infections and are self-limiting with spontaneous resorption of air overtime [1]. Severe and rapidly developing cases can result in an increase of intraorbital pressure leading to orbital compartment syndrome (OCS), a medical emergency requiring immediate intervention [2]. Vision loss occurs in OCS as elevated intraorbital pressure compromises ocular perfusion, leading to ischemia of the optic nerve and retinal structures [2].

OCS is most commonly caused by acute orbital hemorrhage from trauma, surgery or retro-orbital injections causing rapid increase in intraorbital pressure but infectious processes have also been reported [3]. Orbital cellulitis can predispose to orbital emphysema through inflammatory breakdown of the orbital walls, most often the lamina papyracea, resulting in a communication between the orbit and periocular sinuses [1]. Sudden increases in sinus pressure, such as nose blowing, can force air into the orbit through these defects [1]. Early diagnosis of OCS secondary to orbital emphysema is critical. Emergency management includes interventions to relieve intraorbital pressure [2]. This can range from lateral canthotomy and cantholysis, needle decompression, or orbital decompression in severe cases [1].

Here, we present a rare case of a 26-year-old healthy male who developed a severe OCS resulting in no light perception (NLP) vision due to orbital emphysema and cellulitis. He regained 20/20 vision after urgent surgical drainage. The patient provided consent for this case report. The authors adhered to the Declaration of Helsinki and HIPAA compliance. The CARE Checklist has been completed by the authors for this case report, attached as online online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000549287).

Case Description

A 26-year-old healthy male presented to the emergency department following a 1-day history of right periorbital swelling, severe headache, and blurry vision of the right eye. This was preceded by 4 days of sore throat and rhinorrhea. On assessment by the emergency physician at approximately 19:30 he had mildly blurry vision (no formal visual acuity documented but known to be 20/20 at baseline), mild lid swelling but able to open eye easily, no relative afferent pupil defect (RAPD), right hypotropia, with mild limitations in right eye ductions initially thought to be consistent with a 3rd cranial nerve palsy (Fig. 1a).

Fig. 1.

Initial presentation of orbital emphysema and cellulitis resulting in NLP vision of the right eye. a External photograph on initial presentation to ED prior to complete vision loss. b External photograph after sudden onset worsening of periorbital edema and emphysema with complete vision loss. c Coronal CT orbits with thin cuts showing superior gas collection in both right orbit and sinuses causing mass effect on the globe. Arrows indicate a boney defect in the orbital roof in direct communication with the frontal sinus. d Axial CT head with contrast showing large air-fluid level in the right superomedial orbit. e Humphrey visual field at 1-month post-drainage showing a small residual inferior arcuate defect.

A CT head with contrast was done at 20:30 which showed a large air-fluid level on the superomedial aspect of the right orbit causing mass effect on the extraocular muscles, proptosis, and tenting of the right globe (Fig. 1c, d). This was in direct communication with a large air-fluid level in the frontal ethmoidal sinus (Fig. 1d). On reassessment by the emergency physician at 21:00, the periorbital edema had increased substantially where the eyelids could barely be opened forcefully, found to have a RAPD, and an intraocular pressure of 36 (Fig. 1b). At that time ophthalmology was consulted for urgent assessment.

The first ophthalmology assessment was conducted at 21:45 and showed best-corrected visual acuity of NLP OD and 20/25 OS, intraocular pressure was 51 OD and 15 OS, +3 RAPD OD, with −4 limitation of all extraocular movements OD. On history, the patient endorsed repeated nose blowing during his emergency department stay and that he experienced acute worsening of periorbital pain around the time his right eyelid became swollen shut at 21:00.

Given his presumably recent onset of NLP vision, the decision was made to consent the patient for emergent surgical intervention. To our surprise, the operating room (OR) was ready immediately and the patient was transferred which precluded any additional bedside intervention. Methylprednisolone 1 gram intravenous (IV) was given enroute to the OR. The surgical case began at 23:30 with an anterior superior orbitotomy via a medial full thickness lid incision to drain the abscess and air pocket. During drainage, air and a dark purulent material was encountered which was subsequently irrigated with cefazolin and saline. A Penrose drain with multiple perforations with placed along the orbital roof and exited the upper eyelid above the entry incision.

Immediately post-surgery, he could open his eyelids but did not endorse any recovery of vision. While admitted, he was comanaged by infectious disease and otolaryngology. His cellulitis was treated with mometasone and xylometazoline nasal sprays BID, piperacillin/tazobactam 3.375 g IV q6h, and vancomycin 1 g IV q12h. The antibiotic choice was guided by the infectious disease service who initially had concern for an atypical gas-forming organism. Cultures of orbital abscess grew Streptococcus anginosus. Over the subsequent days he gradually regained both afferent and efferent visual function (Table 1). His vision on the operative side was 20/20 by day 4 (Table 1). The Penrose drain was removed after 5 days. Once stable, he was transitioned to ceftriaxone 2 g IV daily and metronidazole 500 mg PO q12h for 6 weeks total. At 1-month post-surgery, a 30-2 Humphrey visual field test showed a small peripheral inferior arcuate scotoma in the right eye (Fig. 1e).

Table 1.

Progression of visual recovery after urgent drainage for OCS caused by orbital emphysema and orbital cellulitis

Time, POD	Best-corrected visual acuity OD	Grade of relative afferent pupillary defect	Intraocular pressure	Extraocular movement limitation OD
Pre-drainage	No light perception	+3	51	−4 supraduction
				−4 adduction
				−4 abduction
				−4 infraduction
POD-1	20/200	+3	17	−3 supraduction
				−1 adduction
				−1 abduction
				−1 infraduction
POD-2	20/60–1	+3	21	−3 supraduction
				−1 adduction
				−1 abduction
				−1 infraduction
POD-3	20/60–1	+3	20	−3 supraduction
				−1 adduction
				−1 abduction
				−1 infraduction
POD-4	20/20	0	23	−3 supraduction
				−1 adduction
				−1 abduction
				−0.5 infraduction
POD-29	20/20	0	16	−0 supraduction
				−0 adduction
				−0 abduction
				−0 infraduction

Conclusion

This case highlights visual recovery in a patient with a severe case of OCS due to orbital emphysema presenting with NLP vision. Most cases of orbital emphysema are asymptomatic and self-limiting and thus do not require any intervention [1]. A recent literature review of 134 patients with orbital emphysema found that 88% resolved with observation alone, 9% were treated with needle decompression and/or canthotomy with cantholysis, and 3% had surgical orbital decompression [1]. Possible reporting bias of cases published in the literature likely underestimate the proportion of cases that are managed conservatively. This review also suggests that the level of intervention can be generally determined by the severity of vision loss where needle decompression can be considered for cases with moderate vision loss (i.e., 20/40 to 20/800) and surgical drainage be used for severe vision loss (i.e., 20/800 to NLP) [1].

In our case, urgent surgical drainage was initiated not only due to the severity of vision loss but also it would allow drainage of the orbital abscess simultaneously. In addition, the OR was requesting him right away which would reduce the time to definitive management. It is important to consider the etiology of orbital emphysema as intervention may be required to treat the underlying cause. In this case, intraoperative culture grew Streptococcus anginosus which has been previously described to result in severe orbital abscesses [4]. In this latter case series, all adult patients required at least 2 surgeries to resolve the infection and 1 patient had to be exenterated [4]. OCS in our case was likely partially due to the orbital abscess with acute decompensation secondary to the emphysema. Even in the absence of orbital emphysema, orbital drainage in our case helped resolve the infection which based on previous literature may not have responded to medical management alone [4]. Other examples of etiologies that may also require surgical intervention include emphysema from an orbital foreign body, occult malignancy, large orbital fracture, or lacrimal sac pneumocele [1]. Typically the communication between the orbit and sinuses occurs due to acute or remote trauma. However, there have been cases of OCS requiring surgical intervention in the absence of trauma and with repeated Valsalva [5].

A primary etiology of vision loss in OCS is ischemia of ocular tissue. This occurs due to compression of blood vessels in the orbit if intraorbital pressure exceeds their perfusion pressure [3]. The vessels most at risk are the central retinal artery (CRA) and the posterior ciliary arteries (PCAs) which supply the retina and optic nerve, respectively [3]. The CRA may have some protection from direct compression due to its anatomic position and higher systolic pressure whereas lower pressure capillaries and the PCA are more vulnerable to ischemia [3]. Hunts et al. [6] described four major clinical stages of orbital emphysema. At lower pressures, compression of the small vessels supplying the optic nerve, such as PCA, are affected first which results in mild-moderate decreased vision. The final stage occurs when lOP exceeds 60 mm Hg and it is thought the CRA occlusion occurs at this stage leading to severe vision loss [6]. In our case, there was presumably occlusion of both the choroidal and retinal circulation given the NLP vision. Brown et al. [7] in their seminal paper demonstrated that occlusion of the CRA alone rarely results in NLP vision and retrospective review of those cases revealed many did in fact have compromised choroidal circulation.

Time to intervention is critical in OCS to allow reperfusion of ocular tissues. Previous review articles described decompression within 2 h to be ideal and associated with the best final visual outcome in a majority of cases [2, 3]. In animal studies, irreparable loss of retinal function after clamping the CRA was found after 105 min [8]. Our patient developed OCS while already admitted to the emergency department which certainly expedited his treatment, but there were still delays from the consultation of ophthalmology, confirmation of diagnosis, and booking of the OR. This resulted in intervention being done at least 2.5 h after onset, which is beyond the 90–120 min time frame typically thought for recovery in CRA ischemia. McCallum et al. [2] describe patients achieving good vision after being treated beyond 2 h but more than 50% had a presenting acuity of 20/40 or better which suggests that treatment beyond 2 h is more likely to lead to recovery of vision in less severe cases. There may be patient factors which impact visual outcome in OCS. For example, in our case the patient’s young age and lack of vasculopathic risk factors may have contributed to the excellent outcome despite the time delay and may limit generalizability to all patients. While visual acuity did return to 20/20 in our patient, on visual field testing we did identify a small arcuate scotoma which would be in keeping with damage from a compressive optic neuropathy. While likely functionally insignificant, particularly under binocular viewing conditions, it is important to recognize that visual acuity is not the only relevant outcome in OCS.

Our case highlights the temporal time course of visual recovery following orbital drainage where it took several days for visual acuity to improve. Hunt et al. include a series of cases where 1 patient had visual recovery from light perception to 20/200 postoperative day (POD)-1 after needle compression, and further improvement to 20/60 on POD-2 and 20/20 after 1 week [6]. Another case from their paper showed improvement of vision from hand motions to 20/400 immediately after needle decompression and further improvement to 20/25 on POD-4 [6]. Similar findings were described in a case of OCS from a combined compressed air and chemical cellulitis. The patient achieved visual recovery from hand movements to 20/20 after delayed decompression at 5 days. Similar to our case, the vision took days to recover with 20/30 on day 1, 20/80 day 3, 20/30-2 day 8, and 20/20 at 4-week follow-up [9]. It is, therefore, still worth considering orbital decompression in cases with delayed presentation.

In conclusion, this case demonstrates the rapid development of an OCS causing NLP vision, and the complete recovery of visual acuity to 20/20 over several days following OCS from orbital emphysema with surgical decompression. This result was achieved despite intervention approximately 2.5 h post onset and presumed ischemia of the CRA. Given the ocular morbidity associated with severe OCS this case adds to the evidence supporting complete orbital decompression in cases with profound vision loss.

Acknowledgments

The authors thank the patient for their willingness to share their case.

Statement of Ethics

Ethical approval is not required for this study in accordance with local or national guidelines. Written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

The authors have no funding sources to declare.

Author Contributions

All the authors (C.L., D.R.J.) contributed intellectually to the content and writing of this case report.

Funding Statement

The authors have no funding sources to declare.

Data Availability Statement

The data that support the findings of this study are not publicly available due to privacy reasons but are available from the corresponding author upon reasonable request.