Bilateral endonasal endoscopic optic nerve decompression in a 7-month-old male patient with osteopetrosis: A case report with review of the literature

While several approaches have been described for optic nerve decompression, the endoscopic endonasal route is gaining favor because it provides excellent exposure of the optic canal and the orbital apex in a minimally invasive manner. Very few studies have detailed the experience with nontraumatic optic nerve decompressions, whereas traumatic cases have been widely documented. Herein, the authors describe their preliminary experience with endoscopic endonasal decompression for nontraumatic optic neuropathies (NONs) to determine the procedure's efficacy and delineate its potential indications and limits. The medical reports of patients who had undergone endoscopic endonasal optic nerve and orbital apex decompression for NONs at the Lyon University Neurosurgical Hospital in the period from January 2012 to March 2014 were reviewed. For all cases, clinical and imaging data on the underlying pathology and the patient, including demographics, preoperative and 6-month postoperative ophthalmological assessment results, symptom duration, operative details with video debriefing, as well as the immediate and delayed postoperative course, were collected from the medical records. Eleven patients underwent endoscopic endonasal decompression for NON in the multidisciplinary skull base surgery unit of the Lyon University Neurosurgical Hospital during the 27-month study period. The mean patient age was 53.4 years, and there was a clear female predominance (8 females and 3 males). Among the underlying pathologies were 4 sphenoorbital meningiomas (36%), 3 optic nerve meningiomas (27%), and 1 each of trigeminal neuroma (9%), orbital apex meningioma (9%), ossifying fibroma (9%), and inflammatory pseudotumor of the orbit (9%). Fifty-four percent of the patients had improved visual acuity at the 6-month follow-up. Only 1 patient whose sphenoorbital meningioma had been treated at the optic nerve atrophy stage continued to worsen despite surgical decompression. The 2 patients presenting with preoperative papilledema totally recovered. One case of postoperative epistaxis was successfully treated using balloon inflation, and 1 case of air swelling of the orbit spontaneously resolved. Endoscopic endonasal optic nerve decompression is a safe, effective, and minimally invasive technique affording the restoration of visual function in patients with nontraumatic compressive processes of the orbital apex and optic nerve. The timing of decompression remains crucial, and patients should undergo such a procedure early in the disease course before optic atrophy.

Osteopetrosis is a rare genetic disorder characterized by abnormal bone density and structure, often leading to vision loss due to optic canal stenosis and consequent nerve compression. Early intervention is critical to prevent irreversible damage. This case report discusses the management of bilateral optic nerve compression in an infant with osteopetrosis. A 7-month-old male with a family history of osteopetrosis presented with hepatosplenomegaly. The infant was diagnosed with osteopetrosis based on radiological findings and genetic testing. Ophthalmologic examination and magnetic resonance imaging showed evidence of bilateral optic nerve compression. Endoscopic transcaruncular optic nerve decompression was not attainable The patient underwent a bilateral expanded endoscopic endonasal medial orbital wall and optic canal decompression. This is one of the few reported cases of endoscopic endonasal optic nerve decompression surgery on an infant. Endoscopic endonasal optic nerve decompression surgery is a viable and effective treatment option for optic nerve compression in infants with osteopetrosis, especially in cases where cost of surgery is a limiting factor for patients. This approach provides direct access to the optic canal with minimal morbidity, offering significant potential for visual recovery, and an improved quality of life. Our patient represents the youngest reported infant in the literature, demonstrating the potential for undergoing this surgical approach at the earliest possible age to aid with his prognosis.

Patients with fibrous dysplasia (FD) of the anterior skull base can experience progressive visual loss and impairment. The authors reviewed their experience with endonasal decompression of the optic nerve (ON) in this patient population. Endoscopic ON decompression (EOND) is a feasible surgical approach for children with FD and visual deficit due to structural ON compression. Electronic medical records of children between 1 and 17 years of age with unilateral FD of the anterior skull base and concomitant ON compression, who required EOND between 2017 and 2022 (n = 4), were reviewed for demographic data, both pre- and postoperative imaging, and evaluations by an otolaryngologist, neurosurgeon, and ophthalmologist in a multidisciplinary fashion. EOND was found to be a safe and effective surgery for children with FD. Visual acuity was stable in 80% of the eyes postoperatively. Visual fields improved in 40% of the eyes and remained stable in the rest. EOND is beneficial for progressive optic neuropathy that is unresponsive to steroid therapy and can prevent permanent disability if performed prior to irreversible damage to the nerve. EOND can decompress the edematous ON with proper exposure of the optic canal and orbital apex, without any major complications.

For decades, treatment of compressive optic nerve neuropathy was conservative and involved the intravenous application of high-dose corticoids, which was combined with nerve growth factors in later years. If surgery was considered, transcranial and transfacial access to the orbital apex and the optic nerve were achieved using classical approaches. Over the past decade, endonasal endoscopic procedures have increasingly come into force, based on favorable experiences with functional endoscopic sinus surgery and as a result of innovative developments in endoscopy and image guidance. The purpose of this review is to address the latest literature in this field with regard to the indications, techniques, results, and complications associated with this procedure. There are basically two approaches for endoscopic optic nerve decompression: the extradural supraorbital “keyhole” approach, which provides access to the optic nerve canal roof and which is usually performed by neurosurgeons, and the endonasal endoscopic approach, which is widely used by otorhinolaryngologists. The development and application of endonasal endoscopic surgery was only possible with extensive knowledge of the topographic anatomy. The most important anatomical landmarks and topography have been described based on either cadaveric or CT scan studies, and include the opticocarotid recess, the distance between the optic nerve canal and the internal carotid artery, the ophthalmic artery, and the length of the optic nerve canal. There are two main indications for endonasal endoscopic optic nerve decompression: (1) traumatic optic nerve neuropathy (TON), and (2) optic nerve neuropathy from non-traumatic causes (nTON), such as tumorous conditions, inflammatory diseases, or idiopathic intracranial hypertension (IIH). In trauma cases, rates of postoperative visual improvement of 40–80% have been reported. Factors influencing the outcome include the severity of the injury, initial visual acuity (light perception or better vs. no light perception), the time interval between trauma and intervention, and the type of injury, such as fracture lines directly through the optic nerve canal or probable avulsion trauma. nTON cases generally have a slightly better prognosis, with vision improvement of 54–100% reported. The procedure should be performed before any optic nerve atrophy can occur. In IIH cases, all major symptoms, including vision loss, headache, visual field deterioration, and papilla edema, improved after optic nerve decompression, with rates of 60–100% reported. Earlier studies reported the occurrence of major complications such as cerebral fluid leakage, severe hemorrhage, or optic nerve injury at rates of about 3%. However, more recent studies postulate that no major complications occur, which may reflect the increasing use of the endonasal endoscopic route to the optic nerve and skull base as standard procedure, so that surgeons are becoming more comfortable with the technique. Endonasal endoscopic optic nerve decompression is a safe and highly effective treatment to reduce hydrostatic pressure on the optic nerve in cases of optic nerve neuropathy of various etiologies. In TON cases, about 50% of patients will benefit from the procedure, with vision improvement possible in some cases even with an interval of some days between trauma and surgery. Non-trauma cases have a slightly better prognosis. The intervention should be performed prior to optic nerve atrophy.

Osteopetrosis is a rare genetic disorder characterized by impaired osteoclast function and excessive bone density, often leading to compressive optic neuropathy due to bony overgrowth of the optic canal. Timely surgical intervention is critical for preventing permanent vision loss in affected patients. This review summarizes the available literature on the surgical techniques of endoscopic endonasal optic nerve decompression (EEOND) and the transcaruncular approach in osteopetrosis patients. EEOND is a minimally invasive technique that offers excellent visualization, advanced instrumentation, and access to the optic nerve through the nasal corridor and requires mastering the anatomy of the sphenoid bone to achieve success. However, challenges arise from the dense and brittle nature of the bone in osteopetrosis, complicating the procedure. The transcaruncular approach provides a targeted route to the medial optic canal with minimal external scarring. However, its limited scope may not suffice for extensive decompression in severe cases. Early surgical intervention correlates with better visual outcomes, particularly in pediatric patients who are at higher risk for rapid progression of vision loss. Integrating advanced imaging and hybrid surgical techniques may enhance decompression efficacy. In conclusion, both EEOND and the transcaruncular approach are valuable for managing optic nerve compression in osteopetrosis, each with distinct advantages and limitations. Ongoing advancements in surgical techniques and a multidisciplinary approach are essential to optimize patient outcomes.

Idiopathic intracranial hypertension (IIH) is a syndrome characterized with increased intracranial pressure and normal cerebrospinal fluid (CSF) composition. Treatment is mainly conservative, whereas CSF diversion surgery is the most frequently used surgical intervention. Endoscopic endonasal optic nerve decompression (EOND) is a newer surgical treatment of this patient group. This study presents a single clinic's case series with comparative results of unilateral an bilateral EOND with or without optic nerve fenestration. Sixteen patients with IIH syndrome who underwent 18 EOND procedures by a single neurosurgeon were evaluated with MRI and digitally subtracted angiography preoperatively. Both preoperative and postoperative visual acuity and perimetry and fundoscopy examinations were routinely performed. All patients underwent sphenoidotomy using the endoscopic binostril approach; however, unilateral or bilateral optic nerve decompression and accompanying optic sheath fenestration was determined on a case-by-case basis, after which all patients were also evaluated for the outcome of headaches. Most of the patients were female, and the mean age was 30.28 ± 9.78 years. CSF pressure was increased in all patients (406.43 ± 112.91 mm of H 2 O), and the follow-up period was 61.72 ± 21.67 months. In patients with unilateral EOND, visual fields improved in 83%, visual acuity in 70%, headache in 75%, and papilledema in 27% of cases. In patients with bilateral EOND, perimetry improved in 86%, visual acuity in 43%, headache in 50%, and papilledema in 57% of cases. EOND is an effective surgical option in the treatment of IIH. Bilateral decompression is preferable in patients with bilateral visual involvement, and optic nerve fenestration may prove to be helpful in patients with IIH whose primary complaint is headache.

Objective To evaluate visual outcomes and potential complications for optic nerve decompression using an endoscopic endonasal approach (EEA) for fibrous dysplasia. Design Retrospective chart review of patients with fibrous dysplasia causing extrinsic compression of the canalicular segment of the optic nerve that underwent an endoscopic endonasal optic nerve decompression at the University of Pittsburgh Medical Center from 2010 to 2013. Main Outcome Measures The primary outcome measure assessed was best-corrected visual acuity (BCVA) with secondary outcomes, including visual field testing, color vision, and complications associated with the intervention. Results A total of four patients and five optic nerves were decompressed via an EEA. All patients were symptomatic preoperatively and had objective findings compatible with compressive optic neuropathy: decreased visual acuity was noted preoperatively in three patients while the remaining patient demonstrated an afferent pupillary defect. BCVA improved in all patients postoperatively. No major complications were identified. Conclusion EEA for optic nerve decompression appears to be a safe and effective treatment for patients with compressive optic neuropathy secondary to fibrous dysplasia. Further studies are required to identify selection criteria for an open versus an endoscopic approach.

BACKGROUNDCongenital optic canal stenosis causing compressive optic neuropathy is a rare disorder that presents unique diagnostic and treatment challenges. Endoscopic endonasal optic nerve decompression (EOND) has been described for optic nerve compression in adults and adolescents but has never been reported for young children without pneumatized sphenoid sinuses. The authors describe preoperative and intraoperative considerations for three patients younger than 2 years of age with congenital optic canal stenosis due to genetically confirmed osteopetrosis or chondrodysplasia.OBSERVATIONSSerial ophthalmological examinations, with a particular focus on object tracking ability, fundoscopic examination, and visual evoked potential trends in preverbal children, are important for detecting progressive optic neuropathy. The lack of pneumatization of the sphenoid sinus presents unique challenges and requires the surgical creation of a sphenoid sinus with the use of neuronavigation to determine the limits of bony exposure given the lack of easily identifiable anatomical landmarks such as the opticocarotid recess. There were no perioperative complications.LESSONSEOND for congenital optic canal stenosis is safe and technically feasible even given the lack of pneumatization of the sphenoid sinus in young patients. The key operative step is surgically creating the sphenoid sinus through careful bony removal with the aid of neuronavigation.https://thejns.org/doi/10.3171/CASE23559

Langerhans cell histiocytosis (LCH) arising from the skull base is a rare acute optic neuropathy cause. We describe a clinical approach for the purpose of optic nerve decompression and simultaneous lesion excision. This is the first case of visual improvement following decompression operation via endoscopic endonasal approach for acute optic neuropathy that is produced by LCH. An 11-year-old boy presented with a 2-week history of visual deterioration. Radiological evidence of compressive optic neuropathy by an expansile soft tissue mass around the optic canal was observed. The patient underwent an endoscopic endonasal optic nerve decompression and subtotal tumor removal. His visual impairment improved dramatically after the operation. Acute optic neuropathy can be elicited by LCH arising in the sphenoid-ethmoid bone. Early decompression is the key to vision salvage. An endoscopic endonasal approach may be a good therapeutic option in this situation.

Many approaches for optic nerve decompression have their own advantages and disadvantages. The aim of this study was to perform endoscopic decompression of optic nerve through the supraorbital keyhole and evaluate its feasibility. Ten adult cadaveric heads were studied using 4-mm, 0- and 30-degree rigid endoscopes to perform the optic nerve decompression through the supraorbital keyhole extradural approach. Furthermore, the relevant measurements about the optic canal were recorded. Through the supraorbital keyhole, the endoscope was introduced into the extradural space, and the following structures could be exposed and identified: the sphenoid ridge, the anterior clinoid process, the roof of the optic canal and the falciform ligament. The roof and lateral wall of the optic canal were removed using a drill under the endoscope, and more sufficient decompression could be achieved by further incising the falciform ligament and optic sheath. After measurement, the distance from the zygomatic process of the frontal bone to the optic canal was 59.32 ± 2.27 mm, the distance from the upper midpoint of the posterior foramen of the optic canal to the internal carotid artery was 3.80 ± 0.93 mm. According to the cadaveric study, it is feasible to perform the endoscopic decompression of optic nerve with a clear view through the supraorbital keyhole extradural approach. Our study may provide a minimally invasive and safe option for the optic nerve decompression.

Objective To investigate the therapeutic efficacy of endoscopic optic nerve decompression (EOND) on patients with traumatic optic neuropathy (TON) and their related prognostic factors. Methods The clinical data of 103 eyes with TON,admitted to our hospital from January 1995 to February 2001 (n=53) and from March 2001 to December 2009 (n=50),were retrospectively analyzed; the clinical outcomes of these patients after being performed EOND were observed. Univariate analysis (Chi-square test) and logistic regression were performed to analyze the prognostic factors of TON underwent EOND therapy. Results The total effective rate of 103 eyes was 37.86％ (39/103); the effective rate of eyes with residual vision was 83.3％ (20/24),which was significantly higher than that of those without residual vision (24.05％,19/79,P＜0.05).Univariate analysis and multiple factor logistic regression,respectively,showed that 3 variables significantly increased the risk of the visual acuity:no residual vision, interval from injury to surgery for more than 3 d and ethmoid and/or sphenoid sinus hematocele after injury.Other factors such as age,disturbance of consciousness,optic canal fracture,performing of sheathotomy,hormone level and interval from injury to surgery for more than 7 d were not correlated with the efficacy (P＞0.05).Comparing fractures at different sites and with different severities,the efficacy of surgery was also different. The logistic regression equation is P (1)=1/ [1+e-(-2139+2.839X3+1.372X5+2.263X9)]. Conclusion The total therapeutic efficacy of EOND on patients with TON is not satisfactory, especially for those without light perception. The prognostic factors of TON were very complex and interactive; no light perception, interval from injury to surgery for more than 3 d,hemorrhage within the ethmoid and/or sphenoid sinus and lateral or multiple fracture of optic canal are factors affecting the prognosis of EOND for patients with TON. The indication for EOND must be individualized. Key words: Traumatic optic neuropathy; Endoscopy; Optic nerve decompression; Prognostic factor

Background: With advances in endoscopic surgical techniques, endonasal decompression of the medial orbit and optic canal for compressive optic neuropathies is occurring more frequently. In the past, there has been debate in the literature regarding the capacity of this procedure to adequately decompress the optic nerve, primarily due to its inability to unroof the optic canal or resect the intracanalicular portions of skull base tumors. Our objective in the present study is to review the outcomes, in terms of preservation and restoration of visual function, of patients who have undergone endoscopic endonasal optic nerve decompression, and to compare those outcomes to patients undergoing open cranial procedures.

Craniopharyngiomas are benign tumors and account for approximately 5.6–13% of all intracranial tumors in children. Diagnosis of pediatric craniopharyngioma is often delayed until the tumor becomes relatively large and manifests severe visual and/or endocrine disturbance. Endoscopic endonasal approaches have recently been introduced to surgery for craniopharyngioma. These techniques, however, have rarely been utilized in patients affected with craniopharyngioma as young as 1 year old. This report documents a 12-month-old male infant with sellar craniopharyngioma who presented with acute total vision loss. To increase the chances of visual recovery, an endoscopic endonasal optic nerve decompression was performed as an urgent procedure. After decompression, which resulted in improvement of his visual disturbance, gross total resection of the tumor was undertaken through an anterior interhemispheric approach at a later date. Tumor mass reduction through an endoscopic endonasal transsphenoidal approach followed by secondary radical total resection under craniotomy was considered to be useful in cases such as this when urgent optic nerve decompression is required.

Traumatic optic neuropathy (TON) can be classified into direct or indirect types. Direct optic injury usually results from optic nerve avulsion ,laceration or compression by fracture, fracture segment impingement or a resultant hematoma. Indirect optic injury is caused by increased intracanalicular pressure resulting in ischemia and disruption of neurofeedback channels. The prognosis of TON is usually quite poor. To date, no standardized treatment protocol has been developed for TON. In this study we are assessing the visual improvement in patients with direct TON who underwent endoscopic optic nerve decompression in the last 10 years. A retrospective study of 32 cases of optic nerve decompression for direct TON in the last 10 years. Preoperative and postoperative visual assessment were done and followed up for 3 months. There was complete improvement in vision in 17% of patients when optic nerve decompression was done within 72h of trauma; whereas 31% cases had only partial improvement when done between 3 and 7 days. And there was no improvement when done after 7 days. Endoscopic optic nerve decompression is a minimally invasive surgery for direct traumatic optic neuropathy; with minimal or no complications when done by an experienced ENT surgeon. Other important prognostic factors include timing of surgery and preoperative visual status.

Endoscopic endonasal optic nerve decompression (EEOND) is indicated in traumatic and endocrine orbitopathies as well as in idiopathic intracranial hypertension. We present a patient with bilateral nonspecific inflammatory orbitopathy (NSIO) and optic nerve compression presenting with acute severe visual loss. Bilateral orbital and optic nerve decompression was performed as an emergency procedure with a favorable ophthalmological result. EEOND is an effective and safe technique in acute orbitopathies and should be taken in consideration in atypical clinical settings causing severe acute visual loss.