Management of frontoethmoidal osteoma causing pneumocephalus and cerebrospinal fluid leakage with minimally invasive techniques: illustrative cases

Nicholas G Candy Department of Surgery-Otolaryngology, Head and Neck Surgery, The University of Adelaide, Adelaide, South Australia, Australia

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Kyle C Wu Department of Neurosurgery, The Ohio State University College of Medicine, Columbus, Ohio; and

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Guilherme Finger Department of Neurosurgery, The Ohio State University College of Medicine, Columbus, Ohio; and

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Kyle VanKoevering Department of Otolaryngology and Skull Base Surgery, The Ohio State University Wexner Medical Center, Ohio

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Daniel M Prevedello Department of Neurosurgery, The Ohio State University College of Medicine, Columbus, Ohio; and

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BACKGROUND

Osteoid osteoma is a common benign bone tumor frequently seen in the frontoethmoid region. However, involvement of the skull base is rare, with few cases previously reported.

OBSERVATIONS

The authors report two cases of spontaneous, symptomatic frontoethmoidal osteoma: one presented with neurological deficit secondary to tension pneumocephalus and the other with cerebrospinal fluid leakage. The first case was managed with a transfrontal sinus craniotomy and pneumocephalus decompression with osteoma resection and skull base reconstruction. The second case was managed with a uninaral endoscopic endonasal approach to the anterior skull base with osteoma resection and reconstruction.

LESSONS

Given the paucity of cases with associated tension pneumocephalus described in the literature, it was relevant to describe the authors’ experience with surgical decision-making and the expected outcomes among patients with this pathology when using minimally invasive techniques.

ABBREVIATIONS

CPAP = continuous positive airway pressure; CSF = cerebrospinal fluid; CT = computed tomography; MRI = magnetic resonance imaging

BACKGROUND

Osteoid osteoma is a common benign bone tumor frequently seen in the frontoethmoid region. However, involvement of the skull base is rare, with few cases previously reported.

OBSERVATIONS

The authors report two cases of spontaneous, symptomatic frontoethmoidal osteoma: one presented with neurological deficit secondary to tension pneumocephalus and the other with cerebrospinal fluid leakage. The first case was managed with a transfrontal sinus craniotomy and pneumocephalus decompression with osteoma resection and skull base reconstruction. The second case was managed with a uninaral endoscopic endonasal approach to the anterior skull base with osteoma resection and reconstruction.

LESSONS

Given the paucity of cases with associated tension pneumocephalus described in the literature, it was relevant to describe the authors’ experience with surgical decision-making and the expected outcomes among patients with this pathology when using minimally invasive techniques.

ABBREVIATIONS

CPAP = continuous positive airway pressure; CSF = cerebrospinal fluid; CT = computed tomography; MRI = magnetic resonance imaging

Osteoid osteoma is one of the most common benign tumors arising in the paranasal sinus, most commonly in the frontal sinus.1 Osteomas are slow growing, typically asymptomatic, and detected incidentally on approximately 3% of all computed tomography (CT) scans of the paranasal sinuses.2 Rarely, frontoethmoidal osteomas can present with neurological sequalae secondary to erosion through the skull base resulting in cerebrospinal fluid (CSF) leakage, meningitis, mucocele formation, and/or pneumocephalus.3,4 There are eight cases of tension pneumocephalus associated with an anterior skull base floor osteoma in the literature with variable management strategies.

Herein, we report two cases of symptomatic frontoethmoidal osteoma, one presenting with neurological deficit secondary to tension pneumocephalus and another with CSF leakage. Moreover, after having performed a literature review, we present and discuss the findings among the eight cases of tension pneumocephalus in the literature and describe our approach to management with minimally invasive techniques.

We report a series of two consecutive cases from a single institution. A retrospective review was performed to examine patients who had undergone surgical management of an osteoma located in the floor of the anterior fossa. Data were collected from The Ohio State University Skull Base Database. The case reports, including the surgical technique, are described. Case review and report followed the instructions of the Committee on Publication Ethics and Case Report guidelines.5

Illustrative Cases

Case 1

A 63-year-old male presented with a 2-week history of progressively intermittent weakness, foot drop, and falls after commencing use of a continuous positive airway pressure (CPAP) device at night. On examination, his left lower extremity was 4/5 in the quadriceps and 3/5 on dorsiflexion. He presented to the emergency room with concerns for a cerebrovascular insult. CT imaging can be seen in Fig. 1.

FIG. 1
FIG. 1

Case 1. CT images of the head in the axial (A), coronal (B), and sagittal (C) planes. There is a large area of low density consistent with pneumocephalus that appears intraxially, along the medial aspect of the frontal lobe. The pneumocephalus originates from the frontal and anterior ethmoidal air cells. The osteoma can be visualized as a hyperdensity filling the entire frontal sinus up to the superior aspect. Yellow arrow indicates the suspected area of dural breach. Correlation with an intraoperative photograph (D) demonstrating the osteoma penetrating the dura with the component of the osteoma that had invaded into the inferior frontal bone and anterior table of the frontal sinus. Green arrow indicates the osteoma.

Because of the subacute neurological deficit and large volume of pneumocephalus concerning for tension, the decision was made to proceed to surgery to decompress the brain, repair the skull base defect, and resect the suspected osteoma. To achieve this, the patient underwent a bicoronal incision with a transfrontal sinus craniotomy.

The patient was positioned supine with the head fixed in a Mayfield head frame. Frameless stereotactic navigation was registered, and a bicoronal incision was marked. Initial incision to the level of the pericranium was carried laterally. Sharp dissection was used to raise a subgaleal flap until 2 cm above the orbital rim. A subfascial dissection of temporalis muscle was performed bilaterally, and a large, vascularized pericranial flap was elevated. The flap was then carried below the superior orbital rim in the subperiosteal plane. Neuronavigation was used to map out the frontal sinus. A high-speed drill was used to create osteotomies above the orbital rim and nasion, which was carried over the frontal bone inside of the frontal sinus. The frontal sinus bone flap was then outfractured in a controlled fashion.

The protruding fragment of osteoma that had punctured the dura was evident in the area expected by the preoperative imaging (Fig. 1). It was drilled away with the posterior table. Upon removal of the osteoma, air decompressed through the dural defect with resulting dural relaxation. Osteoma involving bone of the inferior aspect of the anterior table was further drilled until it was macroscopically clear.

The frontal sinus was cranialized and then burred with a diamond burr to ensure the clearance of mucosa. A free graft of temporalis fascia and muscle was used to plug the nasofrontal ducts bilaterally. The dural defect where the osteoma had penetrated the dura was primarily repaired, and a Valsalva maneuver was used to confirm watertight closure. The vascularized pericranial flap was tacked down onto the undersurface of the frontal lobe dura, which was gently compressed with a Gelfoam sponge. The anterior table was plated with titanium cranial fixation plates, with space above the nasion left for the pedicle of the pericranial flap.

Postoperative imaging demonstrated complete removal of the osteoma and reduction of the pneumocephalus (Fig. 2). The patient’s course was uncomplicated, and he regained strength immediately after surgery and was discharged home on postoperative day 2.

FIG. 2
FIG. 2

Case 1. Postoperative axial (A), sagittal (B), and coronal (C and D) CT images with bone windowing. The images (A and B) demonstrate a reduction in the volume of pneumocephalus and the size of the craniotomy within the confines of the anterior table of the frontal sinus, as well as complete clearance of the osteoma and cranialization of the frontal sinus (C and D).

Case 2

A 27-year-old male with a history of intermittent sinus drainage for 3 years presented with a 4-month history of daily nasal congestion associated with occasional sharp headaches and frequent episodes of clear watery discharge from his nose that would soak the pillow at night. He reported that the drainage often occurred with movement in his head position. He described the drainage as only right sided and having a salty taste. He denied previous trauma to the head or face. He had no other neurological complaints. His neurological examination was normal.

An endoscopic inspection of the bilateral nasal cavities and nasopharynx demonstrated a normal nasal cavity with healthy mucosa, no purulence or polyps, and no obstructing lesions and normal nasopharynx. Further investigation with head CT and magnetic resonance imaging (MRI) was performed (Figs. 3 and 4).

FIG. 3
FIG. 3

Case 2. Preoperative axial (A), coronal (B and C), and sagittal (D) CT images demonstrate a right-sided anterior ethmoidal hyperdense lesion consistent with an osteoid osteoma with associated pneumocephalus.

FIG. 4
FIG. 4

Case 2. Preoperative axial postgadolinium T1-weighted (A), axial T2-weighted (B), coronal postgadolinium T1-weighted (C), and coronal T2-weighted (D) MRI sequences demonstrating low T2 signal (B and D) and low T1 signal without any enhancement (A and C).

The patient underwent a right-sided uninaral endoscopic endonasal approach to the anterior skull base with resection of the osteoid osteoma. We performed resection of the tumor with reconstruction using a method similar to one that has been previously published by our unit.6 Reconstruction was achieved with Duragen (Integra Lifesciences) augmented with a mucosal free graft from the middle turbinate, which was supported by a gelatin sponge.

The patient was discharged from the hospital on postoperative day 1 with no signs of CSF leakage and was neurologically intact. At his 6-week postoperative follow-up, the CSF leakage had not recurred, and his sense of smell had recovered.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Osteoid osteoma is the most common benign neoplasm of the skull with a growth rate of 1.84 mm2 per year.7 Although usually asymptomatic, osteomas can cause symptoms by slow growth causing either cosmetic deformity or displacement of adjacent structures. Histologically, they represent neoplasms of mature, well-differentiated, hyperplastic osseous tissue.8 They are most often found in the frontal sinus, followed by the ethmoid sinuses.

Rarely, these osteomas can create a one-way valve mechanism with the frontobasal dura and result in CSF leakage and pneumocephalus. A recent review has examined osteomas associated with mucocele formation.4

The literature reports eight cases3,9–15 of pneumocephalus and focal neurological deficit secondary to an osteoma in adults (Table 1). The management of these cases varied among reports and has involved single endoscopic or open resections where the osteoma was either resected totally or debulked. In addition, there were two cases of staged stereotactic needle decompression followed by resection. The main reason cited by authors for staging their procedures was because they did not think they would be able to decompress the pneumocephalus adequately from an endoscopic endonasal approach alone. Of note, previous cases were either caused by sneezing or the mechanism was unclear. Our case is the first time this condition has been described in relation to CPAP use.

TABLE 1

Reported cases of tension pneumocephalus associated with focal neurological deficit due to a paranasal sinus osteoid osteoma

Authors & YearAge (yrs), SexOsteoma LocationPreceding CSF LeakManagement
Bramley & Ghosh, 2001963, MRt ethmoidNoBifrontal craniotomy combined w/ lat rhinotomy & near-total resection of osteoma
Johnson & Tan, 20021362, MRt frontalNoRt frontal craniotomy & debulking of osteoma
Kamide et al., 20091457, MRt ethmoidNoRt frontal craniotomy & debulking of osteoma
Guedes et al., 20111033, MLt frontoethmoidNoEndoscopic endonasal transethmoid resection & reconstruction
Harasaki et al., 2013361, FLt frontoethmoidNoStereotactic needle decompression of pneumocephalus, immediately followed by modified endoscopic Lothrop & gross-total resection
Umredkar et al., 20171522, MRt frontalNoUndefined
Hackenbroch et al., 20171124, MRt frontalNoStereotactic needle decompression of pneumocephalus & later during admission surgical resection undefined
Iplikcioglu & Karabag, 20191224, MRt frontoethmoidNoBifrontal craniotomy & resection

Observations

In our case, we were confident that by exposing the osteoma where it had penetrated the dura, osteoma removal would allow decompression of the pneumocephalus. Our surgical goal was to decompress the pneumocephalus, with the secondary goals of complete resection of the osteoid osteoma and prevention of future CSF leakage. Gross-total resection of paranasal sinus osteomas should be the goal, as residual resection can lead to long-term recurrence.8

Lessons

Our unit is a high-volume endoscopic and open skull base unit, and after discussion among neurosurgical and otolaryngology colleagues, it was believed that the surgical priority was to decompress the pneumocephalus. Given the location of the osteoma, this could have been achieved through an endoscopic endonasal approach via a Draf III sinusotomy combined with a vascularized mucosal flap reconstruction. However, because this approach would take longer before pneumocephalus decompression could be achieved, we believed that an open approach was most appropriate.

The surgical goals were different in the second case described, because the patient had presented with chronic CSF leakage and no major signs of elevated intracranial pressure from the pneumocephalus. This reduced the urgency of surgical intervention. In addition, because the osteoma was located more posteriorly within the anterior ethmoidal air cells, an entirely endoscopic approach was less demanding. The described minimally invasive uninaral approach contributed to the patient’s recovery of olfaction, an outcome that may have been more challenging to achieve from a transcranial approach.

Reducing patient morbidity is an important principle in skull base surgery, and these cases demonstrate a way that this can be achieved. These principles and surgical techniques are generalizable to other cases, despite the rare pathology. However, it is important to understand the strengths and skill set of the surgical team involved, because attempting advanced techniques without necessary training can cause morbidity. This case also serves as a cautionary tale regarding the use of CPAP in a patient with CSF leakage.

Author Contributions

Conception and design: all authors. Acquisition of data: Prevedello, Candy, Wu, Finger. Analysis and interpretation of data: all authors. Drafting the article: Candy, Wu, Finger. Critically revising the article: Prevedello, Candy, Wu, VanKoevering. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Prevedello. Study supervision: Prevedello, Wu, VanKoevering.

References

  • 1

    Lim HR, Lee DH, Lim SC. Surgical treatment of frontal sinus osteoma. Eur Arch Otorhinolaryngol. 2020;277(9):24692473.

  • 2

    Lee DH, Jung SH, Yoon TM, Lee JK, Joo YE, Lim SC. Characteristics of paranasal sinus osteoma and treatment outcomes. Acta Otolaryngol. 2015;135(6):602607.

  • 3

    Harasaki Y, Pettijohn KJ, Waziri A, Ramakrishnan VR. Frontoethmoid osteoma with pneumocephalus: options for surgical management. J Craniofac Surg. 2013;24(3):953956.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Licci M, Zweifel C, Hench J, Guzman R, Soleman J. Frontoethmoidal osteoma with secondary intradural mucocele extension causing frontal lobe syndrome and pneumocephalus: case report and review of literature. World Neurosurg. 2018;115:301308.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Riley DS, Barber MS, Kienle GS, et al.. CARE guidelines for case reports: explanation and elaboration document. J Clin Epidemiol. 2017;89:218235.

  • 6

    Albonette-Felicio T, Martinez-Perez R, Vankoevering K, et al.. Soft gasket seal reconstruction after endoscopic endonasal transtuberculum resection of craniopharyngiomas. World Neurosurg. 2022;162:e35e40.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Halawi AM, Maley JE, Robinson RA, Swenson C, Graham SM. Craniofacial osteoma: clinical presentation and patterns of growth. Am J Rhinol Allergy. 2013;27(2):128133.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Sofokleous V, Maragoudakis P, Kyrodimos E, Giotakis E. Management of paranasal sinus osteomas: A comprehensive narrative review of the literature and an up-to-date grading system. Am J Otolaryngol. 2021;42(5):102644.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Bramley DC, Ghosh S. Tension pneumocephalus attributable to an ethmoid osteoma presenting as a stroke in evolution: an unusual presentation. Emerg Med J. 2001;18(4):317318.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Guedes BdeV, da Rocha AJ, da Silva CJ, dos Santos AR, Lazarini PR. A rare association of tension pneumocephalus and a large frontoethmoidal osteoma: imaging features and surgical treatment. J Craniofac Surg. 2011;22(1):212213.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Hackenbroch C, Kleinagel U, Hossfeld B. Tension pneumocephalus due to an osteoma of the frontal sinus. Dtsch Arztebl Int. 2017;114(31–32):534.

  • 12

    Iplikcioglu AC, Karabag H. Frontoethmoid osteoma causing tension pneumocephalus. J Neurosci Rural Pract. 2019;10(3):548550.

  • 13

    Johnson D, Tan L. Intraparenchymal tension pneumatocele complicating frontal sinus osteoma: case report. Neurosurgery. 2002;50(4):878879, discussion 880.

  • 14

    Kamide T, Nakada M, Hayashi Y, Hayashi Y, Uchiyama N, Hamada J. Intraparenchymal pneumocephalus caused by ethmoid sinus osteoma. J Clin Neurosci. 2009;16(11):14871489.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Umredkar AB, Disawal A, Anand A, Gaur P. Frontal sinus osteoma with pneumocephalus: a rare cause of progressive hemiparesis. Indian J Radiol Imaging. 2017;27(1):4648.

    • PubMed
    • Search Google Scholar
    • Export Citation
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  • FIG. 1

    Case 1. CT images of the head in the axial (A), coronal (B), and sagittal (C) planes. There is a large area of low density consistent with pneumocephalus that appears intraxially, along the medial aspect of the frontal lobe. The pneumocephalus originates from the frontal and anterior ethmoidal air cells. The osteoma can be visualized as a hyperdensity filling the entire frontal sinus up to the superior aspect. Yellow arrow indicates the suspected area of dural breach. Correlation with an intraoperative photograph (D) demonstrating the osteoma penetrating the dura with the component of the osteoma that had invaded into the inferior frontal bone and anterior table of the frontal sinus. Green arrow indicates the osteoma.

  • FIG. 2

    Case 1. Postoperative axial (A), sagittal (B), and coronal (C and D) CT images with bone windowing. The images (A and B) demonstrate a reduction in the volume of pneumocephalus and the size of the craniotomy within the confines of the anterior table of the frontal sinus, as well as complete clearance of the osteoma and cranialization of the frontal sinus (C and D).

  • FIG. 3

    Case 2. Preoperative axial (A), coronal (B and C), and sagittal (D) CT images demonstrate a right-sided anterior ethmoidal hyperdense lesion consistent with an osteoid osteoma with associated pneumocephalus.

  • FIG. 4

    Case 2. Preoperative axial postgadolinium T1-weighted (A), axial T2-weighted (B), coronal postgadolinium T1-weighted (C), and coronal T2-weighted (D) MRI sequences demonstrating low T2 signal (B and D) and low T1 signal without any enhancement (A and C).

  • 1

    Lim HR, Lee DH, Lim SC. Surgical treatment of frontal sinus osteoma. Eur Arch Otorhinolaryngol. 2020;277(9):24692473.

  • 2

    Lee DH, Jung SH, Yoon TM, Lee JK, Joo YE, Lim SC. Characteristics of paranasal sinus osteoma and treatment outcomes. Acta Otolaryngol. 2015;135(6):602607.

  • 3

    Harasaki Y, Pettijohn KJ, Waziri A, Ramakrishnan VR. Frontoethmoid osteoma with pneumocephalus: options for surgical management. J Craniofac Surg. 2013;24(3):953956.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Licci M, Zweifel C, Hench J, Guzman R, Soleman J. Frontoethmoidal osteoma with secondary intradural mucocele extension causing frontal lobe syndrome and pneumocephalus: case report and review of literature. World Neurosurg. 2018;115:301308.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Riley DS, Barber MS, Kienle GS, et al.. CARE guidelines for case reports: explanation and elaboration document. J Clin Epidemiol. 2017;89:218235.

  • 6

    Albonette-Felicio T, Martinez-Perez R, Vankoevering K, et al.. Soft gasket seal reconstruction after endoscopic endonasal transtuberculum resection of craniopharyngiomas. World Neurosurg. 2022;162:e35e40.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Halawi AM, Maley JE, Robinson RA, Swenson C, Graham SM. Craniofacial osteoma: clinical presentation and patterns of growth. Am J Rhinol Allergy. 2013;27(2):128133.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Sofokleous V, Maragoudakis P, Kyrodimos E, Giotakis E. Management of paranasal sinus osteomas: A comprehensive narrative review of the literature and an up-to-date grading system. Am J Otolaryngol. 2021;42(5):102644.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Bramley DC, Ghosh S. Tension pneumocephalus attributable to an ethmoid osteoma presenting as a stroke in evolution: an unusual presentation. Emerg Med J. 2001;18(4):317318.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Guedes BdeV, da Rocha AJ, da Silva CJ, dos Santos AR, Lazarini PR. A rare association of tension pneumocephalus and a large frontoethmoidal osteoma: imaging features and surgical treatment. J Craniofac Surg. 2011;22(1):212213.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Hackenbroch C, Kleinagel U, Hossfeld B. Tension pneumocephalus due to an osteoma of the frontal sinus. Dtsch Arztebl Int. 2017;114(31–32):534.

  • 12

    Iplikcioglu AC, Karabag H. Frontoethmoid osteoma causing tension pneumocephalus. J Neurosci Rural Pract. 2019;10(3):548550.

  • 13

    Johnson D, Tan L. Intraparenchymal tension pneumatocele complicating frontal sinus osteoma: case report. Neurosurgery. 2002;50(4):878879, discussion 880.

  • 14

    Kamide T, Nakada M, Hayashi Y, Hayashi Y, Uchiyama N, Hamada J. Intraparenchymal pneumocephalus caused by ethmoid sinus osteoma. J Clin Neurosci. 2009;16(11):14871489.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Umredkar AB, Disawal A, Anand A, Gaur P. Frontal sinus osteoma with pneumocephalus: a rare cause of progressive hemiparesis. Indian J Radiol Imaging. 2017;27(1):4648.

    • PubMed
    • Search Google Scholar
    • Export Citation

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