Orbitopalpebral emphysema in a child after the removal of a giant meningioma: a case report and mini-review

Abstract

Research question

to describe and investigate the case of an 11-year-old boy with the concomitant pneumocephalus, subcutaneous- and orbitopalpebral emphysema after the removal of a giant meningioma. Furthermore, our aim is to discuss the findings and the pathophysiology in relation to cases found in literature.

Material and methods

We performed a search in PubMed, Cochrane, MEDLINE and Google Scholar by the usage of the words orbital or periorbital, combined with emphysema and neurosurgery. In addition, a manual search was performed from reference lists.

Results

In the absence of a trauma and fracture in the orbit, it is considered extremely rare with the simultaneous presentation of an orbital emphysema and pneumocephalus. The literature search revealed 1101 results, with four cases of the simultaneous presentation of orbital emphysema and pneumocephalus after a neurosurgical procedure. Our case of an orbitopalpebral emphysema and pneumocephalus following the removal of a giant meningioma is unique.

Discussion and conclusion

Orbital emphysema might give rise to orbital compartment syndrome, an ophthalmologic emergency, that untreated can result in blindness. Differentiating orbitopalpebral emphysema from postoperative swelling can be accomplished through palpation, which might reveal crepitations, and via an acute CT scan that highlights the presence of air.

Following a neurosurgical procedure, orbital emphysema is an extremely rare phenomenon. Given the rarity of this case, we present informed assumptions and propose a bidirectional migration of air: from intracranial space, to the orbit and subcutaneously to the palpebrae.

Highlights

• •Migration of intracranial air may have several trajectories.
• •Orbitopalpebral emphysema following the removal of a meningioma is never described.
• •Orbital emphysema might give rise to orbital compartment syndrome.
• •Pneumocephalus and orbital compartment syndrome can display similar symptoms.
• •If untreated, orbital compartment syndrome can result in blindness.

1. Introduction

Iatrogenic pneumocephalus is a frequent complication, often considered a benign condition detected on CT scans following neurosurgical procedures, seen in almost half of the cases following evacuation of chronic subdural hematomas with a burr hole (Ihab, 2012). Apart from surgical procedures, skull defects with following pneumocephalus may arise from trauma, eroding tumors or congenital conditions. A review from 2010 further classifies pneumocephalus into three additional etiological subtypes: those caused by post invasive procedures such as spinal anesthesia, barotraumas and infections with gas-producing bacteria. (Schirmer et al., 2010). While the in-situ production of gas is rare, the main pathophysiological mechanisms involve a ball-valve effect and an inverted soda bottle effect. The ball valve mechanism allows air enter the intracranial cavity, where it becomes trapped due to tissue movement triggered by the increased pressure. The inverted soda bottle effect occurs when cerebrospinal fluid (CSF) leaks, leading to air replaces the lost fluid until an equilibrium is established (Dabdoub et al., 2015). A gas follows the pathway of least resistance, and an intracranial migration may occur if the pressure of the gas exceeds the intracranial pressure.

Distinguishing pneumocephalus from the potentially life-threatening tension pneumocephalus, where the latter exerts pressure on the brain that untreated can lead to herniation, is of vital importance. Conducting a comprehensive examination and being alert to signs of increased intracranial pressure, such as headaches and altered consciousness and changes in pupillary responses is essential (Vanderlaan et al., 2021) and an acute CT scanning is recommended in cases of suspicion (Osborn et al., 1978).

In addition to the potential pathophysiology of a ball-valve mechanism, orbital emphysema (OE) shares other parallels with pneumocephalus, since both conditions untreated can lead to devastating consequences. In cases of OE, when the pressure increases within the orbital compartment, it can lead to orbital compartment syndrome (OCS) (Vanderlaan et al., 2021). When intraorbital pressure exceeds the pressure of its vascular structures, it can compress the vessels of the optic nerve, giving rise to ischemia (O'Brien et al., 2000; McCallum et al., 2019). Additionally, elevated pressure can exert direct compress of the optic nerve (McCallum et al., 2019; Wood et al., 1996) or on structures of the eye (Zoumalan et al., 2008; Quigley et al., 1980). The examination of the oculomotor nerve is crucial for the ophthalmologist suspecting OCS, as a diminished pupillary response to afferent signals may occur. This is similar to the examination performed by the neurosurgeons to identify symptoms of elevated intracranial pressure. Treating pneumocephalus and OCS focuses on reducing the pressure and interventions addressing the underlying cause. The first condition may require a cranial re-opening (Ihab, 2012), while OCS may require surgical decompression to preserve vision (McCallum et al., 2019). Although OE and pneumocephalus share some clinical features, their co-occurrence is considered extremely rare (O'Brien et al., 2000).

1.1. Historical background

As early as 1865 Dr. A-L. Thomas first described pneumocephalus during an autopsy, followed by Dr. Chiari some years later (Thomas, 1865; Chiari, 1884). Dr. W.H. Luckett later published an article with the first imaging. He described the case of a 47-year-old man who had been struck by a trolley car and subsequently fallen to the pavement, resulting in intraventricular air identified via an x-ray (Luckett, 1913). In 1914 the article titled Luftansammlung im rechten Seitenventrikel des Gehirns (Pneumozephalus) was published, giving the condition its contemporary name (Wolff, 1914).

Around the same period, a Danish ophthalmologist published a review on emphysema in the orbital region. In this review, the author categorized the condition into a purely orbital or palpebral emphysema, or into a mixed form referred to as orbitopalpebral emphysema (Heerfordt, 1904). Just over 100 years later, 26 different etiologies for OE were compiled where the authors concluded that disruption of the medial orbital wall from blunt trauma is the most common cause of OE (Roelofs et al., 2019).

2. Exemplary case report

2.1. Clinical presentation

An 11-year-old boy, previously in good health and medication-free, but with a six-month history of intermittent blurred vision and diplopia was referred by a private ophthalmologist. Although his great-grandfather had an unspecified brain tumor, he had no other family history of illness. The boy had been fully vaccinated, and previously had chickenpox and atopic dermatitis. His main symptom involved difficulty reading TV subtitles and he often closed one eye to enhance vision. Notably, he reported no headaches, nausea or vomiting.

An ophthalmological examination revealed a bilateral visual acuity of 1.0, a pale sclera and conjunctiva and a clear cornea. Nevertheless, a bilateral papilledema with no present drusen was noted. Further ophthalmological evaluation uncovered a homonymous left-sided hemianopsia.

An urgent CT cerebral scan revealed an 8 × 8 × 8 cm right-sided intraventricular tumor causing mass effect with midline deviation, a compression of the mesencephalon and the right lateral ventricle. The basal cisterns were open. Laboratory tests showed negative results for alpha-fetoprotein and hCG. Coagulation factors, hormone axes, and hematological parameters were in the normal range.

The boy was admitted to the pediatric ward, an MRI of cerebrum and spine were added to the diagnostic investigation the following day, revealing no additional tumor (Fig. 1).

Fig. 1.

Initial MRI brain displaying a right sided meningioma with a midline shift to the left. a Coronal FLAIR sequence and b Axial T2-weighted image.

The boy underwent surgery under general anesthesia in a 6-h long procedure. The surgery involved a supine position with a Mayfield Doro-skull clamp and cerebral entry was achieved by the usage of vector vision. A curved incision and a larger parietal craniotomy were performed, displaying a thinned and compressed cortex. The tumor was resected through an intergyral corticotomy, and was systematically separated from the parenchyma and plexus choroideus before en-bloc removal. After hemostasis was confirmed, the cavity was waterfilled and followed by a watertight closure of the dura mater, and the cranial plate was fixated with two resorbable craniofix. The skin was closed in two layers and a drain was placed in the resection cavity. An intraoperative MRI confirmed successful macroscopic tumor removal, and additional finding of pneumocephalus (Fig. 2). No air was detected in the orbital region. Final histology demonstrated a meningioma WHO grade II.

Fig. 2.

Intraoperative MRI brain. 1,5 T GE Artist scanner displaying status after resection of tumor and intracranial air. a Coronal T1-weigted image and b axial T2-weighted image.

2.2. Diagnosis and management

After surgery, the boy was alert with no new neurological deficits. Just over 12 hours after surgery, the boy's parents noted a swelling around his right eye, and the on-call neurosurgeon was requested.

Crepitation was noted during palpation of the affected areas, and an emergency CT brain scan revealed pneumocephalus, a right-sided subcutaneous emphysema extending from the cranial fixation plate into the right orbit, and an orbitopalpebral emphysema (Fig. 3). No bone dehiscence or sinus abnormalities were found.

Fig. 3.

Postoperative CT brain. In addition to the exposure of a right-sided subcutaneous emphysema. a Coronal image showing pneumocephalus, air under the parietal flap and an orbital emphysema b Pneumocephalus and gas migrating through the craniotomy c Axial image exposing an orbitopalpebral emphysema.

An ophthalmologist performed a puncture of the right conjunctiva, which promptly released the entrapped subconjunctival air (Fig. 4), facilitating a proper examination of the eyes. Beside a swollen eyelid and the expanded conjunctiva, the examination revealed a pale sclera, near-normal visual acuity, normal intraocular pressure and a normal pupillary reaction.

Fig. 4.

A photography of the patient's right eye, taken before the puncture of the conjunctiva by the ophthalmologist. Air is entrapped underneath conjunctiva and in eyelid.

2.3. Prognosis and outcome

The following days after the puncture, the emphysema gradually resolved. During the entire 5-day hospital stay, the boy's condition was stable, maintaining GCS 15 without headache, nausea or vomiting.

One-month after surgery during a follow-up appointment with the neurosurgeon and ophthalmologist, the boy expressed an overall sense of being well. There were no new neurological sequels. Despite the left-sided homonym hemianopsia persisted, there were noticeable improvements. The papillary edema had decreased, and the patient's color vision had returned to normal. Additionally, an MRI of the brain demonstrated satisfactory intracranial conditions, except for a minor chronic, subdural hematoma (Fig. 5).

Fig. 5.

A 1-month follow-up MRI brain. Coronal T1-weighted showing a complete tumor excision, complete regress of midline shift and pneumocephalus and a minor, right-sided chronic subdural hematoma.

3. Discussion

Understanding the simultaneous presence of air within the orbit and intracranial space, we consider three scenarios: migration of air from the intracranial space into the orbit, the reversed passage, or in situ production of gas from bacteria. In our case, the findings on the perioperative MRI with pneumocephalus but no OE, together with the absence of trauma and infection, a relocation of the intracranial air into the orbit from one or more pathways is more likely. However, the question of how the air migrated remains.

To address this question, we performed a search in the literature by a combination of the keywords “orbital or periorbital”, “emphysema” and “neurosurgery” using the Boleen operators “AND” and “OR”, Fig. 6. Although, the majority of cases with concurrent OE and pneumocephalus have derived from trauma with defects in the skull-dura barrier, yet, there are other pathways described. In a case report from 2009 a patient presented with air in the right superior orbital vein and cavernous sinus following a peripheral venous access suggesting iatrogenic air embolism (Tran et al., 2010). A similar migration is mentioned in a case report where a patient was injured in the medial part of the orbit without visible fractures. The authors suggested the laceration of the eyelid and a vein facilitated air migration through the venous drainage of the eye, into the intracranial cavernous sinus (Huggins et al., 2016). Williams and Frankel (1999) describe a case of a 47-year-old man who had an accident with compressed gas where the free end of the air hose hit his left eye, resulting in damage to the conjunctiva and exposure of Tenon's fascia. Although the sclera was described intact, a CT scan revealed air along the intraocular and intracranial portions of the optic nerve, and in the suprasellar cistern. The authors postulate that air likely had dissected beneath Tenon's fascia, encircled the optic nerve, into the nerve's subarachnoid space, through the optic canal into the ventricle of the brain (Williams and Frankel, 1999). They found this plausible since the cerebrospinal fluid surrounding the optic nerve connects with intracranial subarachnoid space, a theory supported by authors of a similar case (Yuksel et al., 2007). Whether the air penetrated the dura, which adheres to the posterior sclera is not clear.

Fig. 6.

The search was inspired by the PICO-model. We included human patients (P - patient) undergoing any neurosurgical procedure (I - intervention) with pneumocephalus and orbital/orbitopalpebral emphysema (O - outcome) as a side effect. There were no restrictions in the time from the neurosurgical procedure until the outcome, neither in the demographics of the patients or the study design. One reviewer (EK) assessed the potential eligibility of the studies. Due to inherent restrictions in Google Scholar, only the first 1000 results were able to be reviewed.

In our case we find a route from the intracranial space, through the dura via the optic nerve less likely. Arguments against this pathway includes the absence of air around the optic nerve on the postoperative CT scan, and the adherence of the dura to the posterior sclera (Fig. 3, Table 1).

Table 1.

Hypotheses of the migration of air, from the intracranial space into the orbit.

Hypothesis	Favors the hypothesis	Opposes the hypothesis
1. A subdural migration of air through n. opticus.	An anatomically short distance.	Absence of air around n. opticus.
	Significant volume of intracranial gas.	Adherence of the dura to sclera.
	Low weight molecules have previously been described to have a higher permeability through dura.	With an anatomical continuum/high permeability, the incidences of brain stem anesthesia following administration of local anesthesia in the orbital compartments before surgical procedures to the eye, would be higher.
	The intracranial dura was opened during surgery.	The presence of subcutaneous air.
	Air dissection through n. opticus previously described.	Migration through the dura of n. opticus would require a high pressure, giving rise to a significant reduced vision.
2. An epidural migration of air through the optic foramen, and/or the superior and inferior orbital fissure.	Same arguments as in 1.	No epidural gas was seen on the CTca.
	Direct access of the air to the orbit, above the dura.	Absence of air around n. opticus, or the structures in superior/inferior fissure.
		The presence of subcutaneous air.
3. Trough the orbital roof.	An anatomically short distance.	No complete bone dehiscence on CT.
	A thin orbital roof of the right orbit on the CTc.	No reports of prior or acute trauma.
	Major volume of gas intracranially.	Low probability of iatrogenic perforation of the orbital roof.
		Does not explain the presence of subcutaneous air.
4. Through the craniotomy site, subcutaneously to the orbit.	Subcutaneous air dissection commonly known.	An anatomically long distance.
	Subcutaneous air noted in CTc, at craniotomy site and in right cheek.	Would require a high pressure to penetrate the conjunctiva and orbital septum.
	Previously described following neurosurgery.

^aCTc: Computer tomography of the cerebrum.

The literature review revealed only four published cases of OE following neurosurgical procedures (Yuksel et al., 2007; Fukuda et al., 2020; Gauden et al., 2012; Solivera et al., 2007) of which two had the mixed state of orbital and palpebral emphysema, similar to our case (Fukuda et al., 2020; Lupu et al., 2020). The articles are summarized in Table 2. To our knowledge it has not previously been described after the removal of a meningioma.

Table 2.

Summary of the reported cases in the literature.

No.	Author	Year	Age/Sex	Preoperative diagnosis/type of surgery	Presentation	Post operative imaging data	Management	Outcome	The author's theories
1	Fukuda et al.	2020	58/Wa	Unruptured, left-sided ICAb/AChAc aneurysm. Left-sided craniotomy and clipping. Minor intraoperative opening of the supraorbital wall.	PreOPd: eyelid edema and diplopia. PostOPe: pain in the left eye proptosis, diminished visual activity, swelling around the orbit.	CT and MRI of the brain: pneumocephalus, bone dehiscence in the left orbital roof, orbitopalpebral emphysema, intraorbital fluid retention. stretching of the left optic nerve and proptosis. Affected left MTVf.	Conservative (diuretics, steroids i.v.g and vitamin supplements).	Diminished visual acuity at 2-year follow-up.	Air migration and accumulation of fluid through the dehiscence in the orbital roof. Potentially affected frontal superficial venous system with an affected perfusion on the venous side.
2	Gauden et al.	2012	58Mh	Unruptured, AComi aneurysm. Left-sided pterional craniotomy and clipping.	PreOP: Vertical diplopia and bilateral proptosis. PostOP: agitation and severe straining. Left-sided periorbital swelling and ecchymoses, no crepitus. No perception of light, minor pupil reaction to light, ophthalmoplegia.	CT and MRI of the brain: pneumocephalus, a bone dehiscence in the left orbital roof, orbital emphysema and venous congestion in the left orbit with an increased proptosis and bilateral stretching of the optic nerves.	Initially conservative. Decompression 10h postOP.	Left optic nerve and retinal atrophy. Visual acuity improved to 6/18, diminished ophthalmoplegia.	Reduced volume from the beginning in the orbit due to preOP protrusion. Compression the globe by the left sided skin flap. PostOP agitation causing venous congestion. Implying the air might have traveled through the bone defect.
3	Lupu et al.	2020	72W	Right-sided VP-shuntj 11 years prior. Intrathoracic gastric herniation. Laparoscopic Nissen fundoplication.	PreOP: no neurological symptoms. PostOP: crepitus along the cervical part of the shunt trajectory. Orbitopalpebral emphysema in the right eye.	Chest- and neck-X-ray: subcutaneous gas around the shunt catheter. Presence of pneumocephalus only suggested.	Conservative.	No sequalae.	Air dissection of from the abdomen, along the shunt into the intracranial space.
4	Solivera	2007	4W	ETVk for hcpl and 4 days later, resection of a pilocytic astrocytoma.	PreOP: headache, nausea, and vomiting, somnolence, mild ataxia, difficulty walking and bilateral papilledema.	CT and MRI of the brain: pneumocephalus, subcutaneous- and OEm.	Conservative.	No sequalae.	Valsalva maneuver increasing the intracranial pressure, giving rise to subcutaneous air dissection from the burr hole to the orbit.

^aW - Woman.

^bICA - Internal carotid cartery.

^cAChA - Anterior choroidal artery.

^dPreOP - Pre operative.

^ePostOP - Post operative.

^fMTV - Middle temporal vein.

^gi. v. - Intravenous.

^hM - Male.

ⁱACom - Anterior communicating artery.

^jVP-shunt - Ventriculoperitoneal shunt.

^kETV - Endoscopic third ventriculostomy.

^lhcp - Hydrocephalus.

^mOE - Orbital emphysema.

Fukuda et al. and Gauden et al. respectively describe a case with the development of OE following left-sided frontotemporal craniotomies for unruptured aneurysms, where the patients develop ipsilateral eyelid edema and visual loss. Postoperative MR and CT-scans reveals pneumocephalus, bone dehiscence in the left orbital roofs, an orbito-palpebral emphysema in one case, and isolated OE in the other, intraorbital fluid accumulations, proptosis and stretching of the optic nerves. Both patients suffered from visual loss and were diagnosed with OCS (Fukuda et al., 2020; Gauden et al., 2012). Initially both patients were treated conservatively, however, after 10 hours one of the patients underwent orbital decompression, probably preserving the vision (Fukuda et al., 2020; Gauden et al., 2012). In our case, the boy had a very thin right orbital roof, though no visible dehiscence, making this route considered less likely. He showed no signs of OCS, with bilaterally normal light reflexes, and no exophthalmos.

Following a Nissen fundoplication comprising a pneumoperitoneum, Lupu et al. described a patient who had received a VP shunt 11 years before, presented with swelling around the right orbit. A neck X-ray revealed gas bubbles along the shunt, but no CT scan was performed. The authors argue the most likely trajectory of gas migration was dissection of air through regions characterized by lax connective tissue, ultimately reaching the right eye (Lupu et al., 2020). A similar pathomechanism was described by Soliviera et al., (2007). In this case, a 4-year-old girl underwent ventriculostomy for hydrocephalus due to a tumor obstructing the 4th ventricle. Three days later, the pilocytic astrocytoma was resected in a sitting position. Following a Valsalva maneuver, she developed swelling around the right orbit, where a CT scan revealed air at the burr hole from the ventriculostomy, subcutaneously around and in the orbit. No bone defects were present. This occurrence was attributed to air dissecting from the intracranial space, subcutaneously into the intraorbital region, facilitated by the child's lax skin and Valsalva maneuver (Solivera et al., 2007). Subcutaneous emphysema is a well-recognized phenomenon in medicine, with reports of a frequence exceeding 50% following some laprascopic procedures (McAllister et al., 1991) and is often considered harmless (Beck et al., 2002). On our patient's CT scan of the brain, air is clearly visible through the flap, on the right cheek, and in the right orbit (Fig. 3). This migration of air may be explained by the law of least resistance, allowing it to move from the non-compliant neurocranium, underneath an elastic skin consistent with the previously described.

Has the air thereafter traveled from the cheek, through the dense orbital septa and further into the orbit, requiring a relatively high pressure? Or has it migrated from the intracranial space, epidurally through the anatomically short distance via the orbital fissures, despite the absence of epidural air on the CT scan? We can only provide the readers with qualified assumptions. Upon evaluating the hypotheses and their respective arguments (Table 1), our main theory is a two directional migration: the subcutaneous emphysema has aroused from a subcutaneous dissection from the craniotomy, beneath the skin of the flap, to the right cheek, with a halt in upper conjunctiva. The OE could instead be explained by a migration of air from the intracranial space, epidurally through the orbital fissure(s), or through the very thin, right sided orbital roof (Fig. 3).

In addition to their own case with both OE and OCS, Fukuda et al. summaries 24 cases with OCS following aneurysm surgery, in which all patient presented with postoperative loss of vision ipsilateral to the site of surgery. Of the six patients who underwent decompressive surgery, three experienced improved vision, one died and data were unavailable for two cases (Fukuda et al., 2020). Our patient showed no clinical or radiological signs of tension pneumocephalus, and the following ophthalmological examination revealed no suspicion of OCS.

Retrospectively, we consider the treatment with a minor puncture of the expanded conjunctiva to have been appropriate. In addition to a reduced time of recovery for the palpebral part of the emphysema, the puncture also facilitated the investigation of the pupillary response and proptosis, ensuring no signs of a severe progression were missed.

4. Conclusion

Pneumocephalus is commonly a benign finding after neurosurgical procedures, but the simultaneous presence with an orbitopalpebral emphysema is extremely rare.

Orbital emphysema can contribute to an orbital compartment syndrome, an ophthalmologic emergency that untreated may lead to blindness.

Differentiating orbitopalpebral emphysema from postoperative swelling can be accomplished through palpation, which might reveal crepitations, and via an acute CT scan that highlights the presence of air.

Author contribution information

E.K. and J.-S.-R. conceived and designed the report and J.-S.-R. supervised E.K. continuously throughout the process. A.T.C. encouraged E.K. to investigate specific ophthalmologic aspects of the case. E.K. performed a literature search and data collection. All authors contributed with analysis of the case and with critical feedback of the manuscript.

Ethics approval and consent to participate

Written consent is provided by parents, and the patient has given oral consent.

Consent for publication

Written consent is provided by parents, and the patient has given oral consent.

Declaration of competing interest

The authors have no financial or non-financial interests to disclose.

Handling Editor: Dr W Peul