Case Reports Periorbital Nodular Fasciitis in Pregnancy: Case Report and Review of the Literature Brent Skippen, B.Med.Sci., M.B.B.S., M.P.H.*, Jeanne Tomlinson, F.R.C.P.A.†, and Krishna Tumuluri, M.B.B.S., F.R.A.N.Z.C.O.‡ Abstract: Nodular fasciitis is a comparatively common benign soft tissue tumor, which may rarely occur in the periorbital and orbital regions. It can be confused with a malignant sarcoma both in its clinical behavior and histologic appearance. Trauma is a suspected risk factor for nodular fasciitis and pregnancy may also be a risk factor. This may be due to the hormone-related changes occurring in pregnancy, which are suspected to be responsible for the growth of some orbital masses in pregnancy. However, there are other cases of orbital masses, which have grown in pregnancy and were not proven positive for estrogen or progesterone receptors, suggesting another mechanism for tumor growth. In any case, awareness of nodular fasciitis in the differential diagnosis of a rapidly growing, soft tissue mass in the periorbital and orbital regions is important to avoid misdiagnosis of a malignancy and unnecessary treatment.
N
odular fasciitis is a comparatively common benign soft tissue tumor, which usually occurs on the trunk, upper extremities, and head and neck, but can also involve the periorbital and orbital regions. It can be confused with a malignant sarcoma both in its clinical behavior and histologic appearance.1 Trauma is a suspected risk factor for nodular fasciitis and pregnancy may also be a risk factor. One other documented case of pregnancy-associated periorbital nodular fasciitis exists in the literature.1 Hormone-related changes that occur in pregnancy may result in the development of periorbital nodular fasciitis and other related myofibroblastic lesions.1,2
CASE REPORT A 31-year-old Asian woman presented 3 months postpartum with an uncomfortable, right medial canthal swelling extending into the glabellar region. It was first noticed when she was 7 months pregnant. There was no history of trauma. Examination demonstrated vision of 6/6, full range of extraocular movements, and a firm mass, slightly tender to palpation. There was normal overlying and surrounding sensation and no lymphadenopathy. Ultrasound of the right periorbital region demonstrated a well-defined subcutaneous hypoechoic lesion measuring 20 × 10 × 8 mm. CT orbits confirmed no intraorbital extension of the lesion (Figs. 1–3). Excisional biopsy of the mass with primary closure was performed. Histologic examination of a well-circumscribed, unencapsulated nodule within soft tissue revealed plump spindle cells with uniform ovoid nuclei, fine chromatin, small
Accepted for publication December 8, 2014. *Sydney Eye Hospital, Sydney, Australia; †Department of Tissue Pathology, ICPMR, Westmead Hospital, Sydney, Australia; and ‡Westmead and Liverpool Hospitals, Sydney, Australia. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Brent Skippen, B.Med. Sci., M.B.B.S., M.P.H., Sydney Eye Hospital, Macquarie Street, Sydney, New South Wales 2000, Australia. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000408
Ophthal Plast Reconstr Surg, Vol. 32, No. 1, 2016
nucleoli, and elongated tails of basophilic cytoplasm. Also present were admixed myxoid areas, extravasated red blood cells, and some inflammatory and giant cells. The spindle cells demonstrated weak cytoplasmic staining for Smooth Muscle Actin, with negative staining for Desmin and S100. The aggregate of clinical, light microscopic, and immunophenotypic features was consistent with a diagnosis of periorbital nodular fasciitis. As the tumor developed during pregnancy, immunohistochemical staining for the estrogen and progesterone receptors was also performed. They were interpreted as negative, with no nuclear staining identified. The patient has recovered well and has been asymptomatic for 9 months postsurgery. There has been no demonstrated radiologic evidence of recurrence (Figs. 4 and 5).
DISCUSSION Nodular fasciitis is a condition that can present both as a subcutaneous mass in the periorbital region or deeper in the orbit. It was first described in the ocular region in 1966 by Font and Zimmerman3 but is more often found in the trunk and upper extremities. The exact pathophysiology of nodular fasciitis remains unknown. It was initially thought to be a reactive lesion, but the identification of the MYH9-USP6 gene fusion as a commonly occurring event in nodular fasciitis has confirmed its clonal neoplastic nature.4 The most common demographic are adults aged 20 to 40 years.5 Men and women are equally affected. In the literature, 10% to 15% of cases have been found to be associated with prior trauma, and the mechanism is believed to be a reactive process.3 One other case of pregnancy-associated periorbital nodular fasciitis has been published recently.1 The authors’ patient represents the second reported case of periorbital nodular fasciitis occurring during pregnancy. Pregnancy may be a causative factor for development of this condition. Nodular fasciitis is a benign proliferation of fibroblasts and myofibroblasts in subcutaneous tissues.6 However, it simulates a malignant neoplasm clinically and histologically and for this reason it should be included in the differential diagnosis of a rapidly growing, soft tissue mass in the periorbital and orbital regions.7 The clinical differential diagnoses include dermoid cyst, epidermal inclusion cyst, fibroma, solitary fibrous tumor, dermatofibroma, fibrosarcoma, neuroma, neurofibroma, lipoma, and granuloma.1 The histologic differential diagnosis includes malignant sarcoma.1,7 Intravascular fasciitis (IVF) is a variant of nodular fasciitis. The authors of a documented case of IVF which developed during pregnancy suggested that estrogen, a known stimulant of fibroblasts and smooth muscle cell types, may play a role in the pathogenesis of IVF.2 The role of estrogen was also proposed in a case of nodular fasciitis arising in pregnancy.1 However, as immunohistochemical staining for the estrogen and progesterone receptor in this tumor and the authors’ case was negative, this notion remains speculative. Other orbital masses reported to have grown in pregnancy include spindle cell tumors such as schwannoma, meningioma, and solitary fibrous tumor.8–12 Only one other documented case exists of such growth associated with positive staining for estrogen or progesterone receptors.8 The authors’ patient developed periorbital nodular fasciitis during pregnancy. She had no history of trauma. Knowledge of nodular fasciitis in the differential diagnosis of a rapidly growing, soft tissue mass in the periorbital and orbital regions is important to avoid misdiagnosis of a malignancy and unnecessary treatment.13 Pregnancy, along with trauma, may be a risk factor for development of periorbital nodular fasciitis. However,
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Case Reports
FIG. 1. Right medial canthal lesion extending into glabellar region.
FIG. 4. Image showing a lesion circumscribed, but not encapsulated, partly surrounded by fibrofatty soft tissue and muscle (hematoxylin and eosin ×20).
FIG. 2. CT orbits: Lobulated complex cystic structure in preseptal space near medial canthus of right eye.
FIG. 5. Focal areas of bluish myxoid change and fibrosis are evident (hematoxylin and eosin ×40).
further cases, examined with appropriate immunohistochemical markers and the use of genetic testing, are needed to confirm this potential association.
REFERENCES
FIG. 3. Excised mass measuring 20 × 10 × 8 mm.
e2
1. Phillips BN, Eiseman AS. Periorbital nodular fasciitis arising during pregnancy. Indian J Ophthalmol 2014;62:520–1. 2. Anand A, Tsapakis EM, Narvani AA, et al. “Pseudosarcoma” in a pregnant woman. World J Surg Oncol 2007;5:7. 3. Font RL, Zimmerman LE. Nodular fasciitis of the eye and adnexa. A report of ten cases. Arch Ophthalmol 1966;75:475–81. 4. Fletcher, C.D.M., Bridge, J.A., Hogendoorn, P., Mertens, F (eds). WHO Classification of Tumours of Soft Tissue and Bone, 4th ed. Lyon: IARC Press, 2013. 5. Mukherjee B, Mittal S. Peri-orbital nodular fasciitis in a child. Orbit 2012;31:124–5. 6. de Paula SA, Cruz AA, de Alencar VM, et al. Nodular fasciitis presenting as a large mass in the upper eyelid. Ophthal Plast Reconstr Surg 2006;22:494–5. 7. Zuber TJ, Finley JL. Nodular fasciitis. South Med J 1994;87: 842–4.
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 1, 2016
8. Chang BY, Moriarty P, Cunniffe G, et al. Accelerated growth of a primary orbital schwannoma during pregnancy. Eye (Lond) 2003;17:839–41. 9. Lee BJ, Schoenfield L, Perry JD. Orbital intramuscular hemangioma enlarging during pregnancy. Ophthal Plast Reconstr Surg 2009;25:491–3. 10. Birkholz ES, Lee AG, Nerad JA, et al. A pregnant pause. Surv Ophthalmol 2010;55:162–8. 11. Sugo N, Yokota K, Nemoto M, et al. Accelerated growth of an orbital schwannoma during pregnancy. J Neuroophthalmol 2007;27:45–7. 12. Das JK, Sharma AS, Deka AC, et al. Solitary fibrous tumor of the orbit presenting in pregnancy. Indian J Ophthalmol 2009;57:238–40. 13. Shields JA, Shields CL, Christian C, Eagle RC. Orbital nodular fasciitis simulating a dermoid cyst in an 8-month old child. Ophthal Plast Reconstr Surg. 2001;17:144–8.
Tension Pneumocephalus Following Orbital Exenteration Wen-Shi Shieh, M.D.*; Christopher Farrell, M.D.†; Joseph Curry, M.D.‡; Ann P. Murchison, M.D., M.P.H.§; and Jurij R. Bilyk, M.D.§ Abstract: Pneumocephalus is a known complication of skull base surgery, but is rarely seen by orbital surgeons. We report a case of postoperative mental status changes after exenteration due to tension pneumocephalus. After surgical and medical management, the patient’s pneumocephalus resolved and she recovered fully. Risk factors for tension pneumocephalus, mechanism, clinical presentation, and management techniques are discussed.
T
ension pneumocephalus, defined as the accumulation of air under pressure within any intracranial compartment, can lead to neurologic deterioration from mass effect and parenchymal herniation, and is considered a neurosurgical emergency.1 It is most commonly associated with skull base-related trauma2 or prior neurosurgical procedures such as craniotomy3,4, and with transnasal endoscopic surgery for paranasal sinus disease or pituitary lesions.5 Symptomatic pneumocephalus following orbital surgery is a rare event. The authors describe a case of tension pneumocephalus following uncomplicated orbital exenteration. This report is HIPPA-compliant.
CASE REPORT An 87-year-old woman with a previously excised periocular basal cell carcinoma presented with a frozen globe and was found to have tumor recurrence invading the right orbit and surrounding bone on biopsy. An exenteration of the right orbit including removal of the lateral orbital wall to the level of greater wing of the sphenoid, rotation of a temporalis muscle flap, and a myocutaneous midfacial flap repair was performed with no obvious intraoperative complications. On the second postoperative day, altered mental status and increased confusion Accepted for publication December 11, 2014. *Wills Eye Hospital; †Department of Neurosurgery, Thomas Jefferson University Hospital; ‡Department of Otolaryngology Head Neck Surgery, Thomas Jefferson University Hospital; and §Skull Base Division, NeuroOphthalmology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, U.S.A. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Ann P. Murchison, M.D., M.P.H., Department of Research, Wills Eye Hospital, 840 Walnut Street, Suite 802, Philadelphia, PA 19107; Email: [email protected] DOI: 10.1097/IOP.0000000000000407
Case Reports
were noted. A head CT scan revealed an extensive pneumocephalus with associated intracranial mass effect on the frontal lobes bilaterally along with the “Mount Fuji sign” (Fig. 1).6 Neurosurgical consultation was initiated and conservative management with supplemental oxygenation was recommended to expedite reabsorption of the intracranial air. Despite these measures, no clinical or radiographic improvement was noted on the following day. The patient subsequently underwent orbital exploration and 3 small areas of bony dehiscence with associated dural defects were identified in the orbital roof. In addition, a bony dehiscence was noted in the lacrimal sac fossa communicating with the nasopharynx. The pneumocephalus was relieved by gentle irrigation of the subdural space with saline to allow for reexpansion of the brain. The dural defects were then primarily repaired with DuraMatrix (Stryker Corporation, Kalamazzo, MI, U.S.A), a collagen-engineered membrane dural substitute, and a fat graft harvested from the abdomen was utilized to cover the bony orbital defects. Finally, the previous temporalis muscle flap was revised to provide reinforcement of the newly placed fat graft. The patient remained intubated to facilitate 100% inspired oxygen during recovery in the intensive care unit. Subsequent serial CT scans demonstrated decreased air volume within the intracranial space and frontal lobe expansion (Fig. 2), which was mirrored clinically by the patient’s rapid mental status improvement. The patient has done well, with no evidence of tumor recurrence or focal neurologic deficits 1 year after surgery.
DISCUSSION Air within the intracranial space has many well-described clinical signs, with headache being the most common presenting complaint. Other signs and symptoms include cerebrospinal fluid (CSF) rhinorrhea, meningismus, seizures, altered mental state, and hemiparesis.7 Peri- and intraoperative risk factors that have been cited for development of pneumocephalus include head position during surgery, surgical duration, nitrous oxide anesthesia, hydrocephalus, hyperventilation, spinal or epidural anesthesia, barotrauma, continuous CSF drainage via lumbar drain, infection, and neoplasms. Additional factors include incomplete reconstruction following skull base procedures and positive pressure events such as coughing, sneezing, nose blowing, or Valsalva maneuvers.8,9 A small amount of pneumocephalus (“nontension pneumocephalus”) is a common finding following intracranial surgery and typically resolves with observation alone. Tension pneumocephalus is a significantly more serious finding and is a neurosurgical emergency.6 In tension pneumocephalus, intracranial air causes a mass effect, most commonly on the frontal lobes. Radiographically, this may manifest as the “Mount Fuji sign.”6 Compression and distortion of the frontal lobes by increased pressure results in widening and separation of the frontal lobes in addition to compression; on axial CT images, these features mimic the silhouette of Mount Fuji (Fig. 1).6,10 Interestingly, the volume of air seen on CT is not an accurate predictor of tension pneumocephalus.10 There are 2 postulated theories that explain the development of tension pneumocephalus. The first is the “inverted bottle” mechanism, where intracranial negative pressure created by drainage of CSF is relieved by influx of air. Alternatively, the “ball-valve” theory suggests that positive pressure induced during episodes of sneezing, coughing, or vomiting force air through cranial defects.11 In our case, the bony defects in the lacrimal sac fossa and the orbital roof may have facilitated the influx of air beneath the soft tissue flaps; a slight positive pressure through this tract most likely occurred postoperatively with
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e3
N
odular fasciitis is a comparatively common benign soft tissue tumor, which usually occurs on the trunk, upper extremities, and head and neck, but can also involve the periorbital and orbital regions. It can be confused with a malignant sarcoma both in its clinical behavior and histologic appearance.1 Trauma is a suspected risk factor for nodular fasciitis and pregnancy may also be a risk factor. One other documented case of pregnancy-associated periorbital nodular fasciitis exists in the literature.1 Hormone-related changes that occur in pregnancy may result in the development of periorbital nodular fasciitis and other related myofibroblastic lesions.1,2
CASE REPORT A 31-year-old Asian woman presented 3 months postpartum with an uncomfortable, right medial canthal swelling extending into the glabellar region. It was first noticed when she was 7 months pregnant. There was no history of trauma. Examination demonstrated vision of 6/6, full range of extraocular movements, and a firm mass, slightly tender to palpation. There was normal overlying and surrounding sensation and no lymphadenopathy. Ultrasound of the right periorbital region demonstrated a well-defined subcutaneous hypoechoic lesion measuring 20 × 10 × 8 mm. CT orbits confirmed no intraorbital extension of the lesion (Figs. 1–3). Excisional biopsy of the mass with primary closure was performed. Histologic examination of a well-circumscribed, unencapsulated nodule within soft tissue revealed plump spindle cells with uniform ovoid nuclei, fine chromatin, small
Accepted for publication December 8, 2014. *Sydney Eye Hospital, Sydney, Australia; †Department of Tissue Pathology, ICPMR, Westmead Hospital, Sydney, Australia; and ‡Westmead and Liverpool Hospitals, Sydney, Australia. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Brent Skippen, B.Med. Sci., M.B.B.S., M.P.H., Sydney Eye Hospital, Macquarie Street, Sydney, New South Wales 2000, Australia. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000408
Ophthal Plast Reconstr Surg, Vol. 32, No. 1, 2016
nucleoli, and elongated tails of basophilic cytoplasm. Also present were admixed myxoid areas, extravasated red blood cells, and some inflammatory and giant cells. The spindle cells demonstrated weak cytoplasmic staining for Smooth Muscle Actin, with negative staining for Desmin and S100. The aggregate of clinical, light microscopic, and immunophenotypic features was consistent with a diagnosis of periorbital nodular fasciitis. As the tumor developed during pregnancy, immunohistochemical staining for the estrogen and progesterone receptors was also performed. They were interpreted as negative, with no nuclear staining identified. The patient has recovered well and has been asymptomatic for 9 months postsurgery. There has been no demonstrated radiologic evidence of recurrence (Figs. 4 and 5).
DISCUSSION Nodular fasciitis is a condition that can present both as a subcutaneous mass in the periorbital region or deeper in the orbit. It was first described in the ocular region in 1966 by Font and Zimmerman3 but is more often found in the trunk and upper extremities. The exact pathophysiology of nodular fasciitis remains unknown. It was initially thought to be a reactive lesion, but the identification of the MYH9-USP6 gene fusion as a commonly occurring event in nodular fasciitis has confirmed its clonal neoplastic nature.4 The most common demographic are adults aged 20 to 40 years.5 Men and women are equally affected. In the literature, 10% to 15% of cases have been found to be associated with prior trauma, and the mechanism is believed to be a reactive process.3 One other case of pregnancy-associated periorbital nodular fasciitis has been published recently.1 The authors’ patient represents the second reported case of periorbital nodular fasciitis occurring during pregnancy. Pregnancy may be a causative factor for development of this condition. Nodular fasciitis is a benign proliferation of fibroblasts and myofibroblasts in subcutaneous tissues.6 However, it simulates a malignant neoplasm clinically and histologically and for this reason it should be included in the differential diagnosis of a rapidly growing, soft tissue mass in the periorbital and orbital regions.7 The clinical differential diagnoses include dermoid cyst, epidermal inclusion cyst, fibroma, solitary fibrous tumor, dermatofibroma, fibrosarcoma, neuroma, neurofibroma, lipoma, and granuloma.1 The histologic differential diagnosis includes malignant sarcoma.1,7 Intravascular fasciitis (IVF) is a variant of nodular fasciitis. The authors of a documented case of IVF which developed during pregnancy suggested that estrogen, a known stimulant of fibroblasts and smooth muscle cell types, may play a role in the pathogenesis of IVF.2 The role of estrogen was also proposed in a case of nodular fasciitis arising in pregnancy.1 However, as immunohistochemical staining for the estrogen and progesterone receptor in this tumor and the authors’ case was negative, this notion remains speculative. Other orbital masses reported to have grown in pregnancy include spindle cell tumors such as schwannoma, meningioma, and solitary fibrous tumor.8–12 Only one other documented case exists of such growth associated with positive staining for estrogen or progesterone receptors.8 The authors’ patient developed periorbital nodular fasciitis during pregnancy. She had no history of trauma. Knowledge of nodular fasciitis in the differential diagnosis of a rapidly growing, soft tissue mass in the periorbital and orbital regions is important to avoid misdiagnosis of a malignancy and unnecessary treatment.13 Pregnancy, along with trauma, may be a risk factor for development of periorbital nodular fasciitis. However,
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Ophthal Plast Reconstr Surg, Vol. 32, No. 1, 2016
Case Reports
FIG. 1. Right medial canthal lesion extending into glabellar region.
FIG. 4. Image showing a lesion circumscribed, but not encapsulated, partly surrounded by fibrofatty soft tissue and muscle (hematoxylin and eosin ×20).
FIG. 2. CT orbits: Lobulated complex cystic structure in preseptal space near medial canthus of right eye.
FIG. 5. Focal areas of bluish myxoid change and fibrosis are evident (hematoxylin and eosin ×40).
further cases, examined with appropriate immunohistochemical markers and the use of genetic testing, are needed to confirm this potential association.
REFERENCES
FIG. 3. Excised mass measuring 20 × 10 × 8 mm.
e2
1. Phillips BN, Eiseman AS. Periorbital nodular fasciitis arising during pregnancy. Indian J Ophthalmol 2014;62:520–1. 2. Anand A, Tsapakis EM, Narvani AA, et al. “Pseudosarcoma” in a pregnant woman. World J Surg Oncol 2007;5:7. 3. Font RL, Zimmerman LE. Nodular fasciitis of the eye and adnexa. A report of ten cases. Arch Ophthalmol 1966;75:475–81. 4. Fletcher, C.D.M., Bridge, J.A., Hogendoorn, P., Mertens, F (eds). WHO Classification of Tumours of Soft Tissue and Bone, 4th ed. Lyon: IARC Press, 2013. 5. Mukherjee B, Mittal S. Peri-orbital nodular fasciitis in a child. Orbit 2012;31:124–5. 6. de Paula SA, Cruz AA, de Alencar VM, et al. Nodular fasciitis presenting as a large mass in the upper eyelid. Ophthal Plast Reconstr Surg 2006;22:494–5. 7. Zuber TJ, Finley JL. Nodular fasciitis. South Med J 1994;87: 842–4.
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 1, 2016
8. Chang BY, Moriarty P, Cunniffe G, et al. Accelerated growth of a primary orbital schwannoma during pregnancy. Eye (Lond) 2003;17:839–41. 9. Lee BJ, Schoenfield L, Perry JD. Orbital intramuscular hemangioma enlarging during pregnancy. Ophthal Plast Reconstr Surg 2009;25:491–3. 10. Birkholz ES, Lee AG, Nerad JA, et al. A pregnant pause. Surv Ophthalmol 2010;55:162–8. 11. Sugo N, Yokota K, Nemoto M, et al. Accelerated growth of an orbital schwannoma during pregnancy. J Neuroophthalmol 2007;27:45–7. 12. Das JK, Sharma AS, Deka AC, et al. Solitary fibrous tumor of the orbit presenting in pregnancy. Indian J Ophthalmol 2009;57:238–40. 13. Shields JA, Shields CL, Christian C, Eagle RC. Orbital nodular fasciitis simulating a dermoid cyst in an 8-month old child. Ophthal Plast Reconstr Surg. 2001;17:144–8.
Tension Pneumocephalus Following Orbital Exenteration Wen-Shi Shieh, M.D.*; Christopher Farrell, M.D.†; Joseph Curry, M.D.‡; Ann P. Murchison, M.D., M.P.H.§; and Jurij R. Bilyk, M.D.§ Abstract: Pneumocephalus is a known complication of skull base surgery, but is rarely seen by orbital surgeons. We report a case of postoperative mental status changes after exenteration due to tension pneumocephalus. After surgical and medical management, the patient’s pneumocephalus resolved and she recovered fully. Risk factors for tension pneumocephalus, mechanism, clinical presentation, and management techniques are discussed.
T
ension pneumocephalus, defined as the accumulation of air under pressure within any intracranial compartment, can lead to neurologic deterioration from mass effect and parenchymal herniation, and is considered a neurosurgical emergency.1 It is most commonly associated with skull base-related trauma2 or prior neurosurgical procedures such as craniotomy3,4, and with transnasal endoscopic surgery for paranasal sinus disease or pituitary lesions.5 Symptomatic pneumocephalus following orbital surgery is a rare event. The authors describe a case of tension pneumocephalus following uncomplicated orbital exenteration. This report is HIPPA-compliant.
CASE REPORT An 87-year-old woman with a previously excised periocular basal cell carcinoma presented with a frozen globe and was found to have tumor recurrence invading the right orbit and surrounding bone on biopsy. An exenteration of the right orbit including removal of the lateral orbital wall to the level of greater wing of the sphenoid, rotation of a temporalis muscle flap, and a myocutaneous midfacial flap repair was performed with no obvious intraoperative complications. On the second postoperative day, altered mental status and increased confusion Accepted for publication December 11, 2014. *Wills Eye Hospital; †Department of Neurosurgery, Thomas Jefferson University Hospital; ‡Department of Otolaryngology Head Neck Surgery, Thomas Jefferson University Hospital; and §Skull Base Division, NeuroOphthalmology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, U.S.A. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Ann P. Murchison, M.D., M.P.H., Department of Research, Wills Eye Hospital, 840 Walnut Street, Suite 802, Philadelphia, PA 19107; Email: [email protected] DOI: 10.1097/IOP.0000000000000407
Case Reports
were noted. A head CT scan revealed an extensive pneumocephalus with associated intracranial mass effect on the frontal lobes bilaterally along with the “Mount Fuji sign” (Fig. 1).6 Neurosurgical consultation was initiated and conservative management with supplemental oxygenation was recommended to expedite reabsorption of the intracranial air. Despite these measures, no clinical or radiographic improvement was noted on the following day. The patient subsequently underwent orbital exploration and 3 small areas of bony dehiscence with associated dural defects were identified in the orbital roof. In addition, a bony dehiscence was noted in the lacrimal sac fossa communicating with the nasopharynx. The pneumocephalus was relieved by gentle irrigation of the subdural space with saline to allow for reexpansion of the brain. The dural defects were then primarily repaired with DuraMatrix (Stryker Corporation, Kalamazzo, MI, U.S.A), a collagen-engineered membrane dural substitute, and a fat graft harvested from the abdomen was utilized to cover the bony orbital defects. Finally, the previous temporalis muscle flap was revised to provide reinforcement of the newly placed fat graft. The patient remained intubated to facilitate 100% inspired oxygen during recovery in the intensive care unit. Subsequent serial CT scans demonstrated decreased air volume within the intracranial space and frontal lobe expansion (Fig. 2), which was mirrored clinically by the patient’s rapid mental status improvement. The patient has done well, with no evidence of tumor recurrence or focal neurologic deficits 1 year after surgery.
DISCUSSION Air within the intracranial space has many well-described clinical signs, with headache being the most common presenting complaint. Other signs and symptoms include cerebrospinal fluid (CSF) rhinorrhea, meningismus, seizures, altered mental state, and hemiparesis.7 Peri- and intraoperative risk factors that have been cited for development of pneumocephalus include head position during surgery, surgical duration, nitrous oxide anesthesia, hydrocephalus, hyperventilation, spinal or epidural anesthesia, barotrauma, continuous CSF drainage via lumbar drain, infection, and neoplasms. Additional factors include incomplete reconstruction following skull base procedures and positive pressure events such as coughing, sneezing, nose blowing, or Valsalva maneuvers.8,9 A small amount of pneumocephalus (“nontension pneumocephalus”) is a common finding following intracranial surgery and typically resolves with observation alone. Tension pneumocephalus is a significantly more serious finding and is a neurosurgical emergency.6 In tension pneumocephalus, intracranial air causes a mass effect, most commonly on the frontal lobes. Radiographically, this may manifest as the “Mount Fuji sign.”6 Compression and distortion of the frontal lobes by increased pressure results in widening and separation of the frontal lobes in addition to compression; on axial CT images, these features mimic the silhouette of Mount Fuji (Fig. 1).6,10 Interestingly, the volume of air seen on CT is not an accurate predictor of tension pneumocephalus.10 There are 2 postulated theories that explain the development of tension pneumocephalus. The first is the “inverted bottle” mechanism, where intracranial negative pressure created by drainage of CSF is relieved by influx of air. Alternatively, the “ball-valve” theory suggests that positive pressure induced during episodes of sneezing, coughing, or vomiting force air through cranial defects.11 In our case, the bony defects in the lacrimal sac fossa and the orbital roof may have facilitated the influx of air beneath the soft tissue flaps; a slight positive pressure through this tract most likely occurred postoperatively with
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e3
