Valsalva-Induced Orbital and Subcutaneous Periorbital Emphysema Secondary to Lamina Papyracea Dehiscence: A Case Report

Valsalva-induced orbital and periorbital emphysema is well described after trauma or surgery involving the medial orbital wall; however, congenital or idiopathic dehiscence of the lamina papyracea may predispose to this clinical entity as well [1]. There is a risk of elevated intraocular pressure and optic neuropathy secondary to orbital compartment syndrome, necessitating prompt recognition and early management [2, 3]. Herein, we describe a case of orbital and subcutaneous periorbital emphysema secondary to Valsalva maneuver in the setting of an idiopathic lamina papyracea dehiscence. The collection and evaluation of protected patient health information were compliant with the Health Insurance Portability and Accountability Act and adhered to the tenets of the Declaration of Helsinki. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000544098).

A 28-year-old man presented to the emergency room after experiencing a sudden onset of significant left-sided periorbital soft tissue swelling following Valsalva maneuver to clear his ears of water after swimming. He described blowing his nose forcefully while pinching the nares closed. He performed the maneuver again in the emergency room which exacerbated his presentation further. He endorsed a self-resolving mild sinus infection in the previous year but otherwise denied any past medical, surgical, or traumatic history. The periorbital soft tissues were significantly swollen and mildly tender, but he denied loss of vision or diplopia (Fig. 1).

Visual acuity was 20/20 in the right eye and 20/25 in the left eye. Intraocular pressures were 8 mm Hg in the right eye and 18 mm Hg in the left eye with a handheld tonometer (Tono-Pen AVIA, Reichert Inc., Depew, NY, USA). Caution was taken to avoid placing pressure on the globe while measuring intraocular pressure, and multiple measurements were taken to ensure reliability. Pupils were equal, round, and reactive to light without a relative afferent pupillary defect. There was no color vision deficiency with the Hardy-Rand-Rittler plates. He had a 3-mm proptosis of the left eye with Hertel exophthalmometry (base 115 mm, 17 mm right eye, 20 mm left eye). His extraocular movements were full in the right eye, but he had pain on extraocular movements of the left eye with a 20% limitation in supraduction.

Slit lamp examination of the left eye showed subcutaneous emphysema, crepitus, violaceous discoloration of the upper and lower lids, and chemosis of the palpebral conjunctiva of the upper and lower lids. Dilated fundus examination was within normal limits. Computed tomography (CT) of the orbits showed extensive subcutaneous and extraconal orbital emphysema along the left periorbital region extending to the temporal region. Several foci of gas were noted tracking to the left anterior lamina papyracea representing a probable sinus wall dehiscence at this location (Fig. 2). The patient was examined by otolaryngology, and a nasal endoscopy was performed which was normal.

The patient was advised to avoid nose blowing (i.e., sinus precautions) and was followed closely. Over the following 5 days, the patient gradually returned to baseline without sequelae. His visual acuity was 20/20 in both eyes, intraocular pressure was 10 mm Hg in both eyes, extraocular movements were full, and there was no proptosis or subcutaneous tissue swelling (Fig. 1e).

Lamina papyracea is of Latin nomenclature, meaning paper-thin, and is the component of the ethmoid bone that comprises the majority of the medial wall of the orbit [4]. Dehiscence of the lamina papyracea without a history of prior surgery or trauma is rare. It is believed that a hyperaerated ethmoid sinus may contribute to the development of dehiscence [1, 5]. First discovered in skull dissections and later described on radiologic imaging, lamina papyracea dehiscence tends to remain asymptomatic, albeit posing a risk of inadvertent orbital injury during endoscopic sinus surgeries [1]. CT findings typically comprise protrusion of the orbital fat through a gap in the anterior ethmoid sinus [1, 5].

Several other cases of orbital and subcutaneous periorbital emphysema after sneezing, forceful nose-blowing, and even constipation-associated Valsalva maneuver have been reported [2, 6‒11]. Some cases had overtly identifiable fractures of the lamina papyracea or medial orbital wall [6, 7, 9, 11], whereas others were not detectable with radiographic imaging [8, 10]. Most cases were self-resolving without visual sequelae, although 1 case did require decompression of the air with needle aspiration without resultant permanent vision loss [2, 3]. Indeed, the paper-thin nature of the lamina papyracea makes it prone to anatomic defects whether congenital, idiopathic, or traumatic from Valsalva maneuver thereby facilitating a channel into the orbit and periorbita. Some other causes of orbital emphysema include orbital trauma, postsurgical sequelae, infection, positive-pressure ventilation, and compressed air injury [12].

Strengths of our case presentation include that it is a novel presentation of a young, otherwise healthy male with significant Valsalva-induced orbital extraconal emphysema and subcutaneous periorbital and temporal emphysema. The emphysema was reproducible on examination with only a suspected subtle dehiscence of the lamina papyracea on CT imaging. Spontaneous orbital and subcutaneous periorbital emphysema associated with Valsalva maneuver is an exceedingly rare phenomenon associated with loss of integrity in the lamina papyracea. Fortunately, most cases resolve without consequence. However, healthcare providers must be cautious of the risk of orbital compartment syndrome necessitating prompt decompression as well as the risk of inadvertently violating the orbit during endoscopic sinus surgery in patients with dehiscence of the lamina papyracea. Patients should be counseled on sinus precautions to avoid exacerbating the emphysema and should be advised to seek emergency care in the event of vision loss, pain, proptosis, or limitation of extraocular motility which may suggest orbital compartment syndrome. Limitations include the lack of long-term follow-up to assess for reconstitution of the lamina papyracea or ongoing symptoms of pneumo-orbit with Valsalva maneuver.

This case report did not require Research Ethics Board approval according to the Tri-Council Policy Statement: Ethical Conduct for Research Involving Humans. Informed consent was obtained in writing from the patient for publication of the details of their case and any accompanying images.

The authors have no conflicts of interest to disclose.

This study was not supported by any sponsor or funder.

S.M.T.: conceptualization, data curation, and writing – original draft and preparation. M.K.: resources and writing – original draft and preparation. F.K.: resources and writing – review and editing. M.B.M.: conceptualization and writing – original draft and preparation.

All data generated or analyzed during this study are included in this article and its online supplementary material files. Further inquiries can be directed to the corresponding author.