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Bilateral meningoencephaloceles with cerebrospinal fluid rhinorrhea after facial advancement in the Crouzon syndrome.

Panuganti BA, Leach M, Antisdel J - Allergy Rhinol (Providence) (2015)

Bottom Line: Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair.Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair.Clinicians should consider CSF shunt placement and carefully weigh the advantages of the transcranial approach versus endonasal, endoscopic techniques.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Head and Neck Surgery, Division of Rhinology and Sinus Surgery, Saint Louis University School of Medicine, St. Louis, Missouri, USA.

ABSTRACT

Background: Cerebrospinal fluid (CSF) rhinorrhea and encephaloceles are rare complications of craniofacial advancement procedures performed in patients with craniofacial dysostoses (CD) to address the ramifications of their midface hypoplasia including obstructed nasal airway, exorbitism, and impaired mastication. Surgical repair of this CSF rhinorrhea is complicated by occult elevations in intracranial pressure (ICP), potentially necessitating open, transcranial repair. We report the first case in otolaryngology literature of a patient with Crouzon syndrome with late CSF rhinorrhea and encephalocele formation after previous LeFort III facial advancement surgery.

Objectives: Describe the case of a patient with Crouzon syndrome who presented with CSF rhinorrhea and encephaloceles as complications of Le Fort III facial advancement surgery. Review the literature pertaining to the incidence and management of post-operative CSF rhinorrhea and encephaloceles. Analyze issues related to repair of these complications, including occult elevations in ICP, the utility of perioperative CSF shunts, and the importance of considering alternative repair schemes to the traditional endonasal, endoscopic approach.

Methods: Review of the literature describing CSF rhinorrhea and encephalocele formation following facial advancement in CD, focusing on management strategies.

Results: CSF rhinorrhea and encephalocele formation are rare complications of craniofacial advancement procedures. Occult elevations in ICP complicate the prospect of permanent surgical repair, potentially necessitating transcranial repair and the use of CSF shunts. Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair. Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair.

Conclusion: Otolaryngologists should be aware of the possibility of occult elevations in ICP and sinonasal anatomic abnormalities when repairing CSF rhinorrhea in patients with CD. Clinicians should consider CSF shunt placement and carefully weigh the advantages of the transcranial approach versus endonasal, endoscopic techniques.

No MeSH data available.


Related in: MedlinePlus

Intact skull base after transcranial repair. CT of the head, showing repair of anterior skull base with bilateral calvarial bone grafts in place.
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Figure 3: Intact skull base after transcranial repair. CT of the head, showing repair of anterior skull base with bilateral calvarial bone grafts in place.

Mentions: The patient had no immediate or delayed complications after this surgery. Several months later, the patient once again developed symptoms of CSF rhinorrhea and recurrence of right encephalocele. The patient ultimately underwent open repair with a pericranial flap with a calvarial bone graft used for definitive anterior skull base repair (Fig. 3). Importantly, an extraventricular drain was placed intraoperatively and was kept in place during her entire inpatient stay. It was discontinued before discharge. To date, the patient remains asymptomatic, without clinical or endoscopic evidence of encephalocele or CSF leak recurrence.


Bilateral meningoencephaloceles with cerebrospinal fluid rhinorrhea after facial advancement in the Crouzon syndrome.

Panuganti BA, Leach M, Antisdel J - Allergy Rhinol (Providence) (2015)

Intact skull base after transcranial repair. CT of the head, showing repair of anterior skull base with bilateral calvarial bone grafts in place.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4541637&req=5

Figure 3: Intact skull base after transcranial repair. CT of the head, showing repair of anterior skull base with bilateral calvarial bone grafts in place.
Mentions: The patient had no immediate or delayed complications after this surgery. Several months later, the patient once again developed symptoms of CSF rhinorrhea and recurrence of right encephalocele. The patient ultimately underwent open repair with a pericranial flap with a calvarial bone graft used for definitive anterior skull base repair (Fig. 3). Importantly, an extraventricular drain was placed intraoperatively and was kept in place during her entire inpatient stay. It was discontinued before discharge. To date, the patient remains asymptomatic, without clinical or endoscopic evidence of encephalocele or CSF leak recurrence.

Bottom Line: Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair.Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair.Clinicians should consider CSF shunt placement and carefully weigh the advantages of the transcranial approach versus endonasal, endoscopic techniques.

View Article: PubMed Central - PubMed

Affiliation: Department of Otolaryngology, Head and Neck Surgery, Division of Rhinology and Sinus Surgery, Saint Louis University School of Medicine, St. Louis, Missouri, USA.

ABSTRACT

Background: Cerebrospinal fluid (CSF) rhinorrhea and encephaloceles are rare complications of craniofacial advancement procedures performed in patients with craniofacial dysostoses (CD) to address the ramifications of their midface hypoplasia including obstructed nasal airway, exorbitism, and impaired mastication. Surgical repair of this CSF rhinorrhea is complicated by occult elevations in intracranial pressure (ICP), potentially necessitating open, transcranial repair. We report the first case in otolaryngology literature of a patient with Crouzon syndrome with late CSF rhinorrhea and encephalocele formation after previous LeFort III facial advancement surgery.

Objectives: Describe the case of a patient with Crouzon syndrome who presented with CSF rhinorrhea and encephaloceles as complications of Le Fort III facial advancement surgery. Review the literature pertaining to the incidence and management of post-operative CSF rhinorrhea and encephaloceles. Analyze issues related to repair of these complications, including occult elevations in ICP, the utility of perioperative CSF shunts, and the importance of considering alternative repair schemes to the traditional endonasal, endoscopic approach.

Methods: Review of the literature describing CSF rhinorrhea and encephalocele formation following facial advancement in CD, focusing on management strategies.

Results: CSF rhinorrhea and encephalocele formation are rare complications of craniofacial advancement procedures. Occult elevations in ICP complicate the prospect of permanent surgical repair, potentially necessitating transcranial repair and the use of CSF shunts. Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair. Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair.

Conclusion: Otolaryngologists should be aware of the possibility of occult elevations in ICP and sinonasal anatomic abnormalities when repairing CSF rhinorrhea in patients with CD. Clinicians should consider CSF shunt placement and carefully weigh the advantages of the transcranial approach versus endonasal, endoscopic techniques.

No MeSH data available.


Related in: MedlinePlus