Philippe Herman

Long‐term follow‐up of juvenile nasopharyngeal angiofibromas: Analysis of recurrences

Objectives: Juvenile nasopharyngeal angiofibroma often recurs if the tumor is large. This report is a long‐term follow‐up of these cases. It establishes the prognostic values of tumor extensions, analyzes the anatomic factors involved in recurrences, describes the spontaneous evolution of remnants based on a radiographic follow‐up, and evaluates the pertinence of complex combined surgical approaches for invasive tumors and the value of complementary endoscopy. Study Design: Retrospective review of 44 cases treated between 1985 and 1996. Methods: Statistical analysis of the correlation between recurrence and tumor extension as evaluated by systematic analysis of 18 putative tumor extensions on preoperative computed tomography scans. Results: Invasion of the skull base affected two‐thirds of the patients, and the rate of recurrence was 27.5%. Extensions to the infratemporal fossa, sphenoid sinus, base of pterygoids and clivus, the cavernous sinus (medial), foramen lacerum, and anterior fossa were correlated with more frequent recurrence. Long‐term radiographic follow‐up revealed putative residual disease in nine asymptomatic patients: these remnants gradually involuted. Conclusions: The data in the current study emphasize the prognostic value of skull base invasion and the difficulty of complete resection of extended lesions. Tumor remnants detected in symptom‐free patients should be kept under surveillance by repeated computed tomography scan, since involution may occur. Recurrent symptoms may be treated by radiotherapy (30 Gy) rather than by extended combined procedures. Endoscopic surgery should be combined with surgery for better control of skull base extensions.

Spontaneous Intracranial Hypotension: A Rare Cause of Labyrinthine Hydrops

Spontaneous intracranial hypotension should be considered as a possible cause of cochlear hydrops. We report a case of unilateral hearing loss attributed to spontaneous intracranial hypotension on the basis of characteristic abnormalities seen on magnetic resonance imaging. The diagnostic gold standards for intracranial hypotension are lumbar measurement of cerebrospinal fluid pressure and magnetic resonance imaging. The usual treatment is an autologous blood injection into the peridural spaces. The mechanism of hearing loss is thought to involve secondary perilymph depression due to a patent cochlear aqueduct. This perilymph depression would induce a compensatory expansion of the endolymphatic compartment, with a subsequent decrease in basilar or Reissners membrane compliance. Endolymphatic hydrops can occur in the course of intracranial hypotension, and not only because of abnormal endolymph production or resorption. Hydrops can thus be classified into 1) syndromes of endolymphatic origin and 2) syndromes of perilymphatic origin, in which loss of perilymph induces compensatory expansion of the endolymphatic space.

Transnasal endoscopic removal of an orbital cavernoma

The approach to posterior and medial orbital tumors is still a challenge, since poor functional results are frequent. We report a case of cavernoma successfully removed by a modified transnasal endoscopic procedure. The patient, a 56-year-old woman, complained of a decrease in vision of the left eye. Magnetic resonance imaging evidenced a lesion in the posterior part of the orbital cavity, inferior to the optic nerve, extending to the sphenoidal cleft. The lesion was isodense on T1-weighted images and showed contrast enhancement. Because of the medial location of the tumor, the patient was referred to the otolaryngology department by the neurosurgeons, and a transnasal endoscopic approach was chosen. A large exposure of the operative field was obtained, and a cavernoma was removed. Rapid relief of the symptoms was obtained. In view of this good result, we advocate the transnasal endoscopic approach in cases of inferomedial and posterior intraconal lesions as an alternative and addition to the standard techniques of orbital surgery.

Sudden sensorineural hearing loss as a revealing symptom of vestibular schwannoma

Conclusions Sudden sensorineural hearing loss (SSHL) is a frequent symptom of vestibular schwannoma (VS), often reveals small VSs and does not exhibit specific features. Therefore, every case of SSHL should be evaluated using systematic MRI to rule out VS in order to improve hearing and preservation facial nerve function. Objective SSHL leads to the discovery of a VS in a small proportion of cases (2%). However, SSHL appears to be a more frequent occurrence in the history of patients with VS (3–23% in the literature), suggesting a large disparity in the evaluation of SSHL. Material and methods A total of 139 consecutive unilateral VSs operated on between 2000 and 2002 were reviewed and analyzed regarding the prevalence, clinical and audiological features of SSHL and their relation to the size of the tumor. Results SSHL was observed in 20% of cases at some point in their VS history. The characteristics of SSHL were: (i) lack of a specific audiometric pattern, except that low-tone loss was rare; and (ii) a high rate of hearing recovery (50%). Tumor size was significantly smaller in SSHL-associated VSs compared to other VSs. In the former cases, 96% involved the internal auditory canal.

Mucocele of the Sphenoid Sinus: A Late Complication of Transsphenoidal Pituitary Surgery

In an 18-month period, 3 cases of sphenoidal mucocele following pituitary surgery were diagnosed at our institution. Only 1 case of this late-onset disorder has yet been reported as a pitfall of the transsphenoidal route. Symptoms include recurrent headache and visual complications. Diagnosis was delayed because of misinterpretation of the magnetic resonance imaging findings, which actually showed the development of a sphenoid mucocele long before clinical symptoms occurred. These 3 cases suggest that attention should be focused not only on the sella turcica, but also on the sphenoid sinus, in analyzing the magnetic resonance imaging data. The treatment consists of endoscopic transnasal marsupialization, since the mucocele is lined by normal epithelium with an inflammatory reaction that will heal with drainage. At the time of surgery, prevention would require either endoscopic control of the mucosal remnants, in case of sinus exclusion, or leaving the sphenoid sinus air-filled under a sealed sella.

Experimental model for investigating trans-mucosal gas exchanges in the middle ear of the rat

Objective The total pressure in the middle ear depends on the air composition of this gas pocket, i.e. on gas exchanges occurring through either the Eustachian tube (ET) or mucosa. The aim of this study was to develop an experimental model to investigate the exclusive role of trans-mucosal gas exchanges in the middle ear (ME). Material and Methods Both tympanic membranes of 20 Sprague–Dawley rats were punctured under general anesthesia. Rats were divided into two equal groups. Group 1 had no ET obstruction. In Group 2, the ET was blocked, after velar incision, by cauterization and application of cyanoacrylate glue into the lumen. One open transparent glass tube containing a droplet of colored water was placed horizontally and connected hermetically to each ear canal. The ME was then flushed with room air through the tube. Variations in ME gas volume were measured by reading the displacement of the liquid droplet in the horizontal tube. The kinetics of variations in gas volume between groups were displayed and statistically compared using a two-sided t-test. Results The pattern of variations in ME gas volume with time was similar in the two groups. Both were characterized by a decrease with three phases and an elimination rate of ≈0.152±0.026 μl/min. There was no significant difference in the mean rate of ME volume changes between the two groups. Conclusion This experimental model allows investigation of trans-mucosal gas exchanges. These exchanges exhibit an absorptive function resulting in a negative pressure that must be compensated, under physiological conditions, by air flow through the ET.

Aneurysmal bone cyst in the sphenoid bone : Treatment with minimally invasive surgery

Aneurysmal bone cysts are vascular lesions that destroy and expand bone. We report a recently treated case of an aneurysmal cyst of the sphenoid bone. A 14-year-old girl presented with frontal headaches, bouts of nausea, and vomiting. Computed tomography and magnetic resonance imaging showed typical features of an aneurysmal bone cyst. Arterial embolization was undertaken before surgery. The endoscopic transnasal procedure used allowed the complete removal of the aneurysmal bone cyst. This use of minimally invasive surgery makes this case of interest to surgeons of the skull base and sinuses.

Nasal haemangiopericytoma : report of two cases with literature review

Haemangiopericytoma (HPC) is a rare vascular tumour produced by proliferation of pericytes. One hundred and thirty-three cases of HPC have been reported in the nose and the paranasal sinuses. We present two more cases. Wide surgical excision via lateral rhinotomy, midfacial degloving, or endonasal removal is the treatment of choice. Radiotherapy has been used post-operatively in cases of incomplete removal. Life-long follow-up is required to evaluate local recurrence and late metastases.

Intrasphenoidal transsellar encephalocele repaired by endoscopic approach.

Spontaneous sphenoidal encephaloceles are rare entities. In the case of intrasphenoidal encephaloceles, most defects are temporosphenoidal and occur in the lateral wall of the sphenoidal sinus. There have been to our knowledge only 7 reports of medial, transsellar encephaloceles in the literature. We report a case of intrasphenoidal transsellar encephalocele that was successfully managed through an endoscopic approach with complementary lumboperitoneal shunting. This disorder presents a challenge in surgical management because of the involvement of opticochiasmatic structures, the hypothalamopituitary axis, and the delicate vasculature of the circle of Willis in and around the encephalocele, besides proximity to the cavernous sinus. Rhinologists should be aware of these malformations, since the optic nerve or chiasma may be totally exposed in the sphenoidal sinus in the course of this disease. This case illustrates the need for magnetic resonance imaging in case of sphenoidal abnormalities, as well as the possibility of endonasal repair without a transcranial approach for large intrasphenoidal encephaloceles.

A rare clival and sellar fracture with pneumatocephalus

We present a case of clival and sellar complex fracture produced by an indirect mechanism. This previously healthy patient had an occipital trauma followed by epistaxis. CT showed a clival and sellar fracture with pneumatocephalus. The probable fracture mechanism was contre-coup injury, linked to cerebral shock-wave transmission. This type of fracture is generally observed in the anterior part of the skull base, in a low resistance area. Severe osteoporosis probably accounted for the unusual fracture site in this patient. A mechanism of direct clival transmission is discussed, together with the usual complications of sphenoid injuries.