The advent and widespread development of endonasal endoscopic techniques have recently expanded the frontiers of skull base surgery. The reduced invasiveness, wider and adjustable visualization of the operative field, and lack of postoperative cosmetic defects are well-known advantages of the endonasal endoscopic approaches compared with traditional surgical exposures both in adults and in children. The need to avoid disruption of facial growth centers and permanent tooth roots represents a further special consideration in favor of these endoscopic techniques in children. The authors report on a case of solitary myofibroma involving the ethmoid, mesial orbits, and anterior skull base with intracranial intradural expansion in a 17-month-old girl. The occurrence of such proliferative disease along the skull base is exceedingly rare. The tumor was successfully excised via an endoscopy-assisted cranionasal approach in which a transcranial microsurgical exposure was combined with endonasal endoscopic access to ensure a radical resection and optimize skull base reconstruction. To the authors' knowledge, the patient in this case is the youngest reported patient in the literature who has undergone treatment with this surgical strategy. The outcome in this patient underscores the feasibility and safety of endoscopic endonasal surgery even in toddlers and early childhood.
Ercole Galassi, Ernesto Pasquini, Giorgio Frank and Gianluca Marucci
Jamie J. Van Gompel, Giorgio Frank, Ernesto Pasquini, Matteo Zoli, Jason Hoover and Giuseppe Lanzino
Transnasal endoscopic (TNE) approaches have been proposed for the resection of anterior cranial base meningiomas. The purpose of this article was to evaluate the results of endoscopic resection of anterior cranial fossa meningiomas by reviewing available published data in addition to the authors' experience with 13 cases.
The literature was searched via OVID to identify all available disaggregate data pertaining to anterior fossa meningiomas treated primarily by TNE. In addition, the authors reviewed the records of a personal series of 13 patients who underwent TNE removal of parasellar meningiomas through a pure TNE approach.
Meta-analysis from studies included a total of 69 patients with adequate disaggregated data to summarize. Mean age (± SD) at surgery was 53.3 ± 13.0 years (range 27–80 years); 78% of the patients were women. Tumor size was skewed toward smaller lesions, with a mean volume of 24.4 ± 2.9 cm3. Intraoperative impressions were of gross-total resection in 76% of cases. The most common complication reported was CSF leakage, occurring in 32% of the cases (22 of 69). The rate of CSF leakage was not affected by size (p = 0.52), location of tumor (p = 0.9), or age (p = 0.56). There was 1 death overall. The mean duration of follow-up was 21 ± 18 months.
Transnasal endoscopic resection of anterior cranial base meningiomas is feasible in selected cases. Reported resection rates are adequate, although the follow-up in the reported series is too short (mean 21 months) to make definitive conclusions regarding the long-term effectiveness. Cerebrospinal fluid leakage is a common complication, although it appears not to be associated with additional morbidity except for the need for reoperation. Long-term results are necessary before considering TNE resection as a valid alternative, in selected cases, to the more established transcranial techniques.
Matteo Zoli, Marco Faustini-Fustini, Diego Mazzatenta, Gianluca Marucci, Eugenio De Carlo, Antonella Bacci, Ernesto Pasquini, Giuseppe Lanzino and Giorgio Frank
Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon.
The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter.
Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved.
The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.
Matteo Zoli, Giacomo Sollini, Sofia Asioli, Clarissa Ann Elisabeth Gelmi, Angelo Gianluca Corradini, Ernesto Pasquini and Diego Mazzatenta
We present the case of a 47-year-old man with left exophthalmus. MRI showed a left intraorbital intraconal cavernous malformation, located in the superoesternal quadrant and medially displacing the optic nerve. An endoscopic transpalpebral approach was performed and total removal was achieved after dissection of the lesion from the optic nerve and other orbital structures. Pathology confirmed the diagnosis of cavernous malformation. The patient was discharged neurologically intact on the second postoperative day free of complications. Follow-up MRI demonstrated radical resection of the cavernoma and resolution of the exophthalmus with an excellent esthetic result.
The video can be found here: https://youtu.be/o1a1tneZ6qk.
Domenico Catapano, Chris A. Sloffer, Giorgio Frank, Ernesto Pasquini, Vincenzo A. D’Angelo and Giuseppe Lanzino
The authors compare the views afforded by the operating microscope and the endoscope in the direct endonasal extended transsphenoidal approach to the sellar, suprasellar, and parasellar regions.
Five formalin-fixed, silicone-injected adult cadaveric heads were studied. A direct endonasal transsphenoidal approach was performed via the right nostril, pushing aside the nasal septum. The approach was performed with the microscope first, then with the endoscope. For each step (sellar, suprasellar, and clival), the exposure afforded by direct microscopic view was measured and then compared with that obtained using the endoscope. The direct endonasal approach provides a slightly off-midline view. Although the microscope provides an adequate view of the mid-line structures and part of the contralateral parasellar areas, the addition of the endoscope allows for a more panoramic view and permits widening of the approach in all directions.
An adequate exposure of the sellar, suprasellar, and infrasellar/upper clival regions can be achieved via a simple, direct endonasal approach. From a direct endonasal route, there is a preferential visualization of the structures contralateral to the approach. The endoscope affords a more panoramic view that extends the area covered by the operating microscope.
Matteo Zoli, Diego Mazzatenta, Adelaide Valluzzi, Gianluca Marucci, Ph.D., Nicola Acciarri, Ernesto Pasquini and Giorgio Frank
In the last decade the indications for the endoscopic endonasal approach have been progressively expanded to include lesions that have not been traditionally considered amenable to resection through the transsphenoidal route. In this study, the authors analyze their experience with hypothalamic gliomas treated via the endoscopic endonasal approach.
Consecutive cases of hypothalamic gliomas treated since 2007 via an endoscopic endonasal approach were reviewed. Preoperative and postoperative neuroimaging as well as endocrinological, neurological, and visual symptoms were analyzed to assess the surgical outcome. Signs and symptoms of hypothalamic dysfunction including body mass index (BMI), memory, sleep-wake rhythm, and polyphagia were prospectively collected pre- and postoperatively to assess hypothalamic function. Quality of life was evaluated using the Katz scale.
In the initial phase the endoscopic endonasal approach was adopted in 3 cases with a palliative intent, to obtain a biopsy sample or for debulking of the mass followed by radio- or chemotherapy. In 2 later cases it was successfully adopted to achieve gross-total tumor resection. Complications consisted of 2 postoperative CSF leaks, which required an endoscopic endonasal reintervention. Visual deficit improved in 3 cases and normalized in the other 2. Four patients developed diabetes insipidus, and 3 an anterior panhypopituitarism. All patients had a moderate increase in BMI. No patients presented with any other signs of hypothalamic damage, and their quality of life at follow-up is normal.
Despite the limitations of a short follow-up and small sample, the authors’ early experience with the endoscopic endonasal approach has revealed it to be a direct, straightforward, and safe approach to third ventricle astrocytomas. It allowed the authors to perform tumor resection with the same microsurgical technique: dissecting the tumor with 2 hands, performing a central debulking, and controlling the bleeding with bipolar coagulation. The main limitations were represented by some anatomical conditions, such as the position of the chiasm and the anterior communicating artery complex and, finally, by the challenge of watertight plastic repair. To definitively evaluate the role of this approach in hypothalamic gliomas, a comparison with transcranial series would be necessary, but due to the rarity of these cases such a study is still lacking. The authors observed that more aggressive surgery is associated with a worse endocrinological outcome; thus they consider it to be an open question (in particular in prepubertal patients) whether radical removal is an advisable goal for hypothalamic gliomas.
Matteo Zoli, Laura Milanese, Rocco Bonfatti, Marco Faustini-Fustini, Gianluca Marucci, Giovanni Tallini, Corrado Zenesini, Carmelo Sturiale, Giorgio Frank, Ernesto Pasquini and Diego Mazzatenta
In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach.
All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered.
Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9–80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7–159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate.
The EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.
Luigi Maria Cavallo, Giorgio Frank, Paolo Cappabianca, Domenico Solari, Diego Mazzatenta, Alessandro Villa, Matteo Zoli, Alfonso Iodice D'Enza, Felice Esposito and Ernesto Pasquini
Despite their benign histological appearance, craniopharyngiomas can be considered a challenge for the neurosurgeon and a possible source of poor prognosis for the patient. With the widespread use of the endoscope in endonasal surgery, this route has been proposed over the past decade as an alternative technique for the removal of craniopharyngiomas.
The authors retrospectively analyzed data from a series of 103 patients who underwent the endoscopic endonasal approach at two institutions (Division of Neurosurgery of the Università degli Studi di Napoli Federico II, Naples, Italy, and Division of Neurosurgery of the Bellaria Hospital, Bologna, Italy), between January 1997 and December 2012, for the removal of infra- and/or supradiaphragmatic craniopharyngiomas. Twenty-nine patients (28.2%) had previously been surgically treated.
The authors achieved overall gross-total removal in 68.9% of the cases: 78.9% in purely infradiaphragmatic lesions and 66.3% in lesions involving the supradiaphragmatic space. Among lesions previously treated surgically, the gross-total removal rate was 62.1%. The overall improvement rate in visual disturbances was 74.7%, whereas worsening occurred in 2.5%. No new postoperative defect was noted. Worsening of the anterior pituitary function was reported in 46.2% of patients overall, and there were 38 new cases (48.1% of 79) of postoperative diabetes insipidus. The most common complication was postoperative CSF leakage; the overall rate was 14.6%, and it diminished to 4% in the last 25 procedures, thanks to improvement in reconstruction techniques. The mortality rate was 1.9%, with a mean follow-up duration of 48 months (range 3–246 months).
The endoscopic endonasal approach has become a valid surgical technique for the management of craniopharyngiomas. It provides an excellent corridor to infra- and supradiaphragmatic midline craniopharyngiomas, including the management of lesions extending into the third ventricle chamber. Even though indications for this approach are rigorously lesion based, the data in this study confirm its effectiveness in a large patient series.
Matteo Zoli, Giacomo Sollini, Laura Milanese, Emanuele La Corte, Arianna Rustici, Federica Guaraldi, Sofia Asioli, Luigi Cirillo, Ernesto Pasquini and Diego Mazzatenta
Surgical treatment of orbital lesions is challenging because complex approaches with a high risk of postoperative sequelae are required. Recently, minimally invasive endoscopic approaches through endonasal (EEA) and transpalpebral (ETP) routes have been proposed. The objective of this study was to assess outcomes of EEA and ETP in the authors’ series of patients with orbital lesions.
Data from all patients who underwent operations for an orbital tumor through an endoscopic approach at the authors’ institution from 2002 to 2018 were retrospectively collected. All patients underwent preoperative MRI and ophthalmological evaluation, which was repeated 3 months after surgery and then at regular follow-up intervals. A systematic review of the literature was also performed using Medline, Embase, and Web of Science databases.
The series includes 23 patients (14 males); the mean patient age was 48 ± 23.9 years. Most of the lesions were intraconal (n = 19, 83%). The more frequent histotype was cavernous hemangioma (n = 5, 22%). Exophthalmos was the most common symptom (21 of 23 patients). EEA was performed in 16 cases (70%) and ETP in 7 (30%). The aim of the surgery was achieved in 94% of the cases after an EEA (successful biopsy in 5 of 6 cases and radical resection in all 10 remaining patients), and in 86% after an ETP (successful biopsy in 2 cases and radical tumor resection in 4 of 5 cases). Complications consisted of 3 cases (13%) of transitory diplopia. One recurrence (4%) was observed at follow-up (mean 59 ± 55 months).
The EEA and ETP have demonstrated to be safe and effective for tumors located respectively in medial and lateral quadrants, permitting one to approach orbital lesions endoscopically at 360°. Innovative surgical tools, including intraoperative ultrasonography, may be useful to potentially reduce surgical morbidity. Larger series are needed to validate these preliminary suggestions.