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Total aplasia of paranasal sinus (TAPS) is extremely rare, although the fact that partial aplasia is very common. TAPS seems to be limited to only 5 case reports in the literature until now. We present the case of a 29-year-old patient who has a syndromic face appearance but whose TAPS was detected incidentally. The maxillary, sphenoid, ethmoid, and frontal sinuses were totally aplastic. Furthermore, clinodactyly and high-arched palate were observed. The patient's appearance was consistent with a syndromic face because of some findings on inspection such as hypertelorism, shortening of the palpebral fissure, protruded and wide nasal base, high arched palate and zygomatic hypoplasia. The patient's profile was more suitable for Teacher-Collins syndrome than other syndromes, however, a certain diagnosis was not made genetically. To the best of our knowledge, this is the first reported association between TAPS and a syndromic condition.

Hamamy syndrome (HS) is an autosomal recessive syndrome with a genetic origin that is very rarely observed. The syndrome with craniofacial dysmorphisms, including midface prominence, severe telecanthus, sparse lateral eyebrows, protruding ears, fronto-nasal abnormalities, lacrimal-salivary apparatus agenesis, thin upper vermillion border, myopia, mental retardation, sensorineural hearing impairment, congenital heart anomalies with intraventricular conduction delay, hypochromic microcytic anaemia and skeletal abnormalities of the long bones with recurrent fractures. In this paper, we report a case of two brothers diagnosed with HS at the ages of 25 and 18 years, visited out clinic at different times due to dental reasons. In the radiological examinations, it was observed that both brothers have sphenoid sinuses agenesia, and their sella turcica were smaller than normal. HS may be observed very rarely, and it should be kept in mind that, in addition to various symptoms, it may also cause sphenoid sinus agenesis and sella turcica hypoplasia as shown for the first time in this case report.

Purpose: Sphenoid sinuses are pneumatised structures, placed in the body of the sphenoid bone, with highly variable morphology. The strict relationships with vascular and nervous structures determine the importance of their anatomical variants in otorhinolaryngology and neurosurgery; a precise understanding of the complex anatomy and anatomic variations of these structures is pivotal for radiological diagnosis of paranasal sinuses pathology and for surgical planning, to avoid potential complications. Our aim is to describe the anatomical variants of sphenoid sinuses, and to help general radiologists and specialists in endoscopic surgery in becoming familiar with these sensitive anatomical structures., Methods: A literature search of PubMed and Embase (Elsevier) databases was performed using the keywords "sphenoid sinus" and "anatomy, "sphenoid sinus" and "anatomic variants", "sphenoid sinus", and "anatomic variations"., Results: We described the anatomical variants of the sphenoid sinuses anatomy, according to their size, shape, degree of pneumatisation, protrusion of anatomical structures into their lumen, superimposition of ethmoid cells (Onodi cells), and presence of accessory septations CONCLUSION: The information provided by this study may help in the identification and description of the anatomical variations of the sphenoid sinuses and their relationship to neurovascular structures.

Introduction: Conventional surgical approaches to the lateral aspect of a well-pneumatized sphenoid sinus are associated with significant surgical morbidity. Transorbital endoscopic approaches have recently gained favor as an alternative approach to the skull base. We describe the use of a contralateral precaruncular endoscopic approach to provide a surgical pathway to the lateral aspect of the sphenoid sinus, allowing for improved and direct visualization of the surgical field, with limited morbidity., Case Report: A 60-year-old female patient, with a spontaneous cerebrospinal fluid leak from a Sternberg canal defect in the sphenoid sinus, underwent repair of the defect at Groote Schuur Hospital (Cape Town, South Africa). A contralateral precaruncular approach, using the left medial orbital portal, was utilized to access the defect in the lateral aspect of a well-pneumatized right sphenoid sinus. Computer modeling software was used to predetermine the surgical pathway, and the case was performed under navigation guidance. Adequate surgical access was obtained to the lateral sphenoid sinus and sinus defect, with superior visualization compared to a pure transnasal or transpterygoid approach to the lateral sphenoid sinus., Conclusions: This case validates the use of computer simulation to plan and decide on the best operative approaches in skull base surgery and describes the contralateral precaruncular approach as a surgical pathway to the lateral sphenoid sinus. Advantages of the contralateral precaruncular approach include a direct trajectory toward the sinus defect, easy access with a standard 18-cm, 4-mm, 0-degree rigid endoscope, and straight instruments, with sparing of the pterygoid base and contents of the pterygopalatine fossa.

A 53-year-old man presented with a 1-week history of headache and double vision. On examination, he was found to have right cranial nerve III palsy. Computed tomography and magnetic resonance imaging of the brain showed a sellar mass with right cavernous sinus invasion. There was no evidence of anterior visual pathway compression on formal neuro-ophthalmologic examination. An elective endoscopic transsphenoidal adenectomy was performed. On access to the sphenoid sinus, he was noted to have a completely dehiscent posterior bony wall of sphenoid sinus with only a thin mucous membrane covering the sella, optic nerves, carotid arteries, and clivus (Figure 1). A focal area of mucosa was subsequently stripped from the posterior sphenoid sinus wall, and a micro-Doppler was used to localize the carotid arteries before sharp dural opening. The tumor was subsequently resected without complication. Failure to appreciate this uncommon anatomic variant (Figures 2 and 3) could result in disastrous irreversible carotid artery or optic nerve injury., (Copyright © 2019 Elsevier Inc. All rights reserved.)

In the present study, we investigated the relationship between sphenoid sinus, carotid canal, and optic canal on paranasal sinus computed tomography (PNSCT). This study was performed retrospectively. PNSCT images of 300 adult subjects (159 male, 141 female). Sphenoid sinus (pneumatisation, dominancy, septation, inter-sinus septa deviation), anterior clinoid process pneumatisation, Onodi cell, carotid and optic canals (width, dehiscence, classification) were measured. In males, type 3 pneumatised sphenoid sinus (in both sides) and in females type 2 pneumatised sphenoid sinus (right side) and type 3 pneumatised sphenoid sinus (left side) were detected more. Anterior clinoid pneumatisation was present 47.2% in males and 39.7% in females. In male group, more septation (i.e. 22.6%, ≥ 3 septa) in sphenoid sinus were detected. Onodi cell was present 26.6 and 19.1% in males and females, respectively. Carotid canal protrudation to the sphenoid sinus wall was present 23.9-32.1% in males and 35.5-36.2% in females. Dehiscence in carotid canal was detected more in females (34%) compared to males (22%). Optic canal protrudation was 33.3 and 30.5% in males and females. Type 4 optic canal was detected more in both gender. Optic canal dehiscence was detected 11.3 and 9.9% in males and females. Carotid and optic canal diameters were higher in males. In pneumatised sphenoid sinuses and in females, type 3 carotid canal (Protrudation to SS wall) (bilaterally) and type 1 optic canal type (No indentation) (ipsilateral side) were detected more. In elderly patients, carotid and optic canal width increased. When carotid canal protrudation was detected, there was no indentation in optic canals In pneumatised SS, carotid canal protrudation was observed with a greater risk in surgery. However, type 1 (non indentation) optic canal was present in highly pneumatised SS with lower risk for the surgery. In women, the risk of carotid canal protruding (about 1/3) is greater than that of males, and carotid canal dehiscence rates are also higher in females. Therefore, physicians should be very careful during the preparatory stages of the sphenoid sinus surgery. Otherwise, it may not be possible to prevent lethal carotid artery bleeds.

Objective: Arrested pneumatization of the sphenoid sinus is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of arrested pneumatization of the sphenoid sinus in paediatric patients with haemotologic diseases like sickle cell anaemia, thalassemia and leukemia. Materials and Methods: One hundred and eight paediatric patients (43 girls, 65 boys; age range: 4-18 years; median age: 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging of the head and neck between 2010 and 2017 in a single institution were included. Magnetic resonance imaging studies were retrospectively reviewed. Well-defined and fat-containing non-expansile lesions located at the sphenoid sinus region were diagnosed as arrested pneumatization of the sphenoid sinus. Medical diagnoses, lesion sizes, age and sex of the patients were recorded. Results: Five (4.6%) of 108 patients demonstrated arrested pneumatization of the sphenoid sinus (3 girls, 2 boys; age range: 9-18 years; median age: 14 years). One (1.2%) of 83 patients with acute leukemia, 2 (15%) of 13 patients with sickle cell anaemia and 2 of 12 (17%) patients with thalassemia demonstrated arrested pneumatization of the sphenoid sinus. No statistical correlation was found between arrested pneumatization of the sphenoid sinus and possible risk factors like patient age and sex. Conclusions: Patients with haemoglobinopathies like sickle cell anaemia and thalassemia involving red-blood-cells had higher prevalence of arrested pneumatization of the sphenoid sinus than patients with leukemia which involves the white-blood-cells. This supported the suggestion that regional blood-flow disorders induce arrested pneumatization of the sphenoid sinus. Correct diagnosis prevents invasive procedures like biopsy and surgery.

This anatomic image demonstrates the sphenoethmoidal (Onodi) cell (a variant of the paranasal sinuses), the identification of which is critical to prevent neurovascular injury during endoscopic approaches to the sella and adjacent regions of the skull base., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Reports of congenital anomalies of the Eustachian Tube (ET) are scarce, and often associated with chromosomal abnormalities. We report a unique case of a completely bony left Eustachian tube which communicated with the sphenoid sinus. This report details these findings and discusses the potential embryological basis and implications of such an unusual anatomy, in the context of a comprehensive literature review.

The aim of this report is to investigate the pathogenesis and surgical treatment of encephalocele located in the sphenoid sinus and presented with spontaneous rhinorrhea. The data of a patient with sphenoid sinus encephalocele was analyzed in association with the review of literature. The patient admitted to our clinic with rhinorrhea from the left nasal cavity. Cranial magnetic resonance imaging revealed bone defects in the left sphenoid sinus wall, and the presence of brain tissue and cerebrospinal fluid (CSF) in the sphenoid sinus. The patient underwent dural repair via left pterional approach. Rhinorrhea disappeared after surgery. The pathogenesis of the encephalocele in the sphenoid sinus is not clear. It may be related to the presence of lateral craniopharyngeal canal, extra-gasification in the sphenoid sinus, high intracranial pressure or other factors. It is very important to diagnose accurately the source of CSF fistula in preoperative period, choose the specific operation technique, and follow-up the patient for a long time period.

Frank-ter Haar syndrome is a genetic disease that is transmitted by autosomal recessive pattern with characteristic features such as megalocornea or glaucoma, a prominent coccyx, heart defects, developmental delays, brachycephaly, a wide anterior fontanel, finger flexion deformities, full cheeks and micrognathia. Dentomaxillofacial features of this syndrome are not well documented in the literature. We present of a 21-year-old male with Frank-ter Haar syndrome and some features that may be linked with this syndrome not reported before in the literature.

Objectives: This case presents a previously undescribed clinical scenario of spontaneous cerebrospinal fluid (CSF) leaks secondary to a lateral sphenoid sinus recess skull base dehiscence and contralateral Sternberg's canal. This case report aims to characterize the presentation and successful management of these lesions., Methods: The electronic medical record was used to collect information pertaining to the patient's clinical history., Results: The patient was a middle-aged, obese female with persistent clear rhinorrhea as her only presenting symptom. Neuroradiologic studies localized the defect to the lateral sphenoid sinus recess. CSF opening pressures were within normal limits, but radiographic findings were consistent with elevated intracranial pressure. After an endoscopic transnasal transsphenoidal approach failed to resolve the CSF leak, a transpterygoid approach facilitated CSF leak resolution. The patient then did well for the following 2 years, but later developed a CSF leak through a contralateral Sternberg's canal. An endoscopic suprapterygoid procedure and ventriculoperitoneal shunt placement led to CSF leak resolution., Conclusion: This case demonstrates one of the only published examples of a sphenoid sinus CSF leak secondary to Sternberg's canal as it was originally described in the literature. Wide endoscopic surgical exposure and intracranial pressure management ultimately led to CSF leak resolution., (© The Author(s) 2015.)

Agenesis of paranasal sinuses is an uncommon finding mainly seen in the frontal and maxillary sinuses. Agenesis of sphenoid sinuses is an extremely rare condition. In this article, we present an 18-year-old female patient suffering from headache and nasal obstruction without any skeletal or cranial anomalies. Axial, coronal and sagittal computed tomography revealed absent sphenoid sinuses. Surgeons should be aware of sphenoid sinus agenesis, when transsphenoidal hypophysectomy and endoscopic sinus surgery are scheduled, in particular.

Agenesis of the sphenoid sinuses is a very rarely encountered anatomic variation. Findings on magnetic resonance imaging can mislead the radiologist and clinician. Therefore, the gold standard for diagnosis is computed tomography (CT) of the paranasal sinuses. We present the case of a 28-year-old man who was admitted to our hospital with a 3-month history of headache. CT of the paranasal sinuses revealed isolated bilateral sphenoid sinus agenesis.

Spontaneous nasal liquorrhea (SNL) is a pathological condition previously regarded as a casuistic one but increasingly frequently described in the current literature. The present review is focused on the causes of SNL, such as the anomalous anatomical structure of the nasal cavity and paranasal sinuses. One of the variants of the anatomical structure is the so-called Sternberg canal. Its anatomical structure and formation in the course of embryonic development are described. The presence of the Sternberg canal in the adult subjects is considered to be the main cause underlying SNL and meningoencephalocele originating from the lateral parts of the sphenoidal sinus. The main variants of the endoscopic endonasal approach for the treatment of the fluid fistulas located in the lateral parts of the sphenoidal sinuses are described. A series of the original authors' observations is reported including 173 patients presenting with SN l who were operated in 1999-2011.

Objective: We report the rare case of a 55-year-old woman with an asymptomatic left sphenoid sinus pseudomeningocele mimicking a mucocele., Case Report: A 55-year-old woman was found to have an incidental mass in the left sphenoid sinus on computed tomography. Magnetic resonance imaging confirmed the presence of a mucocele. A left endoscopic sphenoidotomy was performed to drain the mucocele. Despite an anatomical puncture through a stenosed sphenoid ostium, alarmingly, the opening leaked cerebrospinal fluid. A dehiscent lateral wall was identified with a dural opening communicating with the sphenoid sinus. This was immediately repaired with a free nasal septal mucosal graft. Six months post-operatively, the patient remained free from cerebrospinal fluid leakage., Conclusion: Pseudomeningoceles are extremely rare in the absence of trauma or iatrogenic injury. Surgeons should be alert to their presence as they can mimic a unilateral mucocele or nasal polyp. Endoscopic management lends itself to a single-stage repair.

Object: Transsphenoidal encephalocele (TSEC) is a rare developmental anomaly with herniation of neural elements including pituitary gland or optic apparatus, through a defect of sphenoid bone. To prevent obstructive dyspnea, endocrine dysfunction, or visual disturbance, surgical correction is usually performed. However, the optimal operative approach has not been determined yet. The authors present their surgical experiences with transpalatal, transcranial, or combined approach., Methods: The authors retrospectively analyzed surgical results in patients with TSEC. The charts were reviewed for demographic data, clinical presentation, surgical therapy, and clinical outcomes., Results: Seven consecutive patients with TSEC were surgically treated between March 2004 and February 2012. The mean age was 23.9 months (range 2-69 months). Four patients with TSEC who had a bone defect within the sphenoid bone were successfully treated using a transpalatal approach. Two patients with an extensive sphenoethmoidal bone defect that was treated either by a transpalatal or transcranial approach experienced several relapses with a prolapse of the encephalocele. One patient with an extensive sphenoethmoidal bone defect was successfully treated by combined transpalatal and transcranial approach without relapse for 3.6 years., Conclusions: The combined transpalatal and transcranial approach is useful for TSEC with an extensive sphenoethmoidal bone defect to dissect the sac and expose the bone defect circumferentially. If dissection is not sufficient, relapse with a prolapse of the encephalocele can ensue. The transpalatal approach is sufficient for TSEC in which a bone defect is limited within the sphenoid bone.

Background: The sphenoid sinus is a frequent target of paranasal sinus surgery. Because of the high risk of injuring the surrounding structures (e.g. internal carotid artery, optical nerve) a preoperative imaging is absolutely necessary., Purpose: To analyze the possibilities of cone-beam computed tomography (CBCT), which is especially quite a new technique in ENT, in the evaluation of the sphenoid sinus, its surrounding structures, and the corresponding anatomical variations., Material and Methods: This was a retrospective, single-centre study of 580 patients (1160 sides = cases). The Accu-I-Tomo-F17 was used. Pneumatization of sphenoid sinus, course of internal artery, course of optical nerve, and dehiscence of the bony canals were evaluated., Results: In the case of pneumatization a type I (completely missing or minimal sphenoid sinus) was found in two patients (0.3%), type II (posterior wall of sphenoid sinus is in front of the anterior wall of the sella) in 38 patients (6.6%), type III (posterior wall is between anterior and posterior wall of sella) in 332 patients (57,2%), type IVa (posterior wall is behind the posterior wall of sella without air dorsal the sella) in 104 patients (17.9%), and type IVb (similar to type IVa but with air dorsal the sella) in 104 patients (17.9%). In 1025 cases (89.5%) a smooth course of the internal carotid artery was found whereas a free course could be detected in 120 cases (10.5%). Defects of the bony canal of the optical nerve were found in 16.7% and of the internal carotid artery in 2.7% of the cases. The optical nerve showed a free course through the sphenoid in 151 cases (13.7%) and a smooth course in 1007 cases (87.0%)., Conclusion: CBCT could evaluate all relevant anatomic structures and answer the questions of different anatomical variants. A modified classification of the pneumatization of the sphenoid sinus could be described. Frequencies of anatomical variations are in accordance with the current literature of CT research.

Endoscopic surgical techniques allow treating various pathological diseases of the cranial base, including cerebrospinal fluid leaks (CSF). These approaches have a high success rate and reduce surgical time and morbidity. However, when they are located in the lateral wall of the sphenoid, the surgery is more complex. In our case, the CSF appeared to be related with a Sternberg's canal. Predisposing factors were also associated with an extensive unilateral pneumatization of the sinus and benign intracranial hypertension. The closure of the leak was achieved using an endoscopic transethmoidal transpterygoid approach for proper exposition of the lateral wall of the sphenoid. After five years of monitoring, the patient has had no more episodes of rhinoliquorrhea., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

We present the case report on a 66-year-old male patient who appeared in our clinic with recurrent epistaxis. Anterior rhinoscopy during acute bleeding yielded no information on the origin of hemorrhage. On renewed epistaxis, angiography was performed on which an arteriovenous malformation in the sphenoid sinus could be identified as the cause of epistaxis. Embolization of afferent vessels successfully reduced the malformation and stopped the bleeding. In cases of recurrent epistaxis, especially in combination with an unremarkable anterior rhinoscopy, angiography is recommended for the detection of life-threatening vessel malformations.

Purpose: In adults, sphenoid sinus agenesis is an extremely rare anomaly. The objective of this study was to investigate the prevalence of sphenoid sinus hypoplasia and agenesis using dental volumetric computed tomography (DVCT) in a population of Turkish individuals., Materials and Methods: DVCT scans in the axial, coronal and sagittal planes of the sphenoid sinus of 384 patients were examined for evidence of sphenoid sinus agenesis and hypoplasia., Results: In the DVCT scans, bilateral agenesis of sphenoid sinus was not seen. Unilateral agenesis of sphenoid sinus was seen in 0.26% of the sample, and sphenoid sinus hypoplasia was seen in 0.52%. Unilateral hypoplasia of the sphenoid sinus was observed in 0.26% of the sample, and bilateral hypoplasia of sphenoid sinus was observed in 0.26%., Conclusion: In this study, we found a low frequency of sphenoid sinus agenesis. Compared with sphenoid sinus agenesis, the frequency of sphenoid sinus hypoplasia was higher. DVCT may be used as a diagnostic tool to investigate the paranasal sinus.

Objective: To report the case of a woman who had combined aplasia of sphenoid, frontal, and maxillary sinuses accompanied by ethmoid sinus hypoplasia., Methods and Results: A 47-year-old woman presented with complaints of headache, nasal obstruction, and postnasal drainage. She had no previous history of either facial trauma or systemic diseases affecting the skeletal system. She had a history of functional endoscopic sinus surgery twice in an other institution. However, the surgeries did not significantly alter her symptoms. Coronal and axial computed tomographic scans of the nose and the paranasal sinuses showed poorly developed frontal, sphenoid sinuses, ethmoid cells, and very severe and profound hypoplasia (aplasia) of bilateral maxillary sinuses., Conclusions: To our knowledge, this patient seems to be the first case having combined aplasias of the sphenoid, frontal, and maxillary sinuses with hypoplastic ethmoid cells without any systemic or skeletal disease.

The absence of sphenoidal sinuses (SS) in an adult is an extremely rare condition. We investigated in agenesis of the sphenoid, maxillary, ethmoid, and frontal sinuses in 20 male adult cadavers. In a 50-year-old man, bilateral absence of the SS was observed. On the macroscopic examination, opening of the SS was not found on the lateral wall of the nasal cavity. There were multiple small mucosal grooves between the sphenoidal rostrum and the superior nasal turbinates. The bulging of the sphenoidal rostrum at the choanal level was flat. The body of the sphenoid bone was normal and consisted of a symmetrical bony structure with a total lack of pneumatization. Other paranasal sinuses agenesis was not observed. Surgeons should also consider the possibility of sphenoidal agenesis before transsphenoidal hypophysectomy. As a supplement to the traditional classification, agenesis of the SS can be described as the fourth type of pneumatization.

Objective: Sphenoid sinus agenesis is a very rare entity. In this study, we evaluated the CT scans of 1193 patients and detected cases with sphenoid sinus agenesis despite the absence of craniofacial anomalies or syndromes., Methods: The CT scans of 1193 patients who had undergone endoscopic sinus surgery in our clinic between 1999 and 2008 were retrospectively evaluated. Of the patients, 652 (54.6%) were female and 541 (45.3%) were male. The mean age of the patients was 32 years (range: 13-75 yrs.)., Results: In this study, we identified eight patients (0.67%) with sphenoid agenesis. Three patients (37.5%) had bilateral, 3 patients (37.5%) had left, and 2 patients (25%) had right sphenoid agenesis. None of the patients had craniofacial anomalies or syndromes., Conclusion: Sphenoid sinus agenesis is rarely seen in the absence of craniofacial anomalies or syndromes. In our study, its incidence was 0.67%. Awareness of sphenoid sinus agenesis and/or hypoplasia is very important, particularly when transphenoidal hypophysectomy is planned.

Aim: The aim of this study was to assess the septation of the sphenoid sinuses and relationship between the number and position of septa in the adult Polish subpopulation., Methods: A retrospective study of the CT examination of the paranasal sinuses 207 patients (101 male, 106 female) were performed using spiral multirow CT scanner (Siemens Somatom Sensation 10) with the following parameters: the extent fully covering all paranasal sinuses, detector configuration 10 x 0.75 mm, feed 4.1 mm, reconstruction thickness 1 mm, reconstruction increment 0.7 mm, kernel H60s for bone structures, or reconstruction thickness 4 mm, reconstruction increment 4 mm, kernel H31s for soft tissue structures. Secondary coronal and sagittal MPR's (multiplanar reconstructions) were then generated from original axial slices. No intravenous contrast media was required. Siemens CARE Dose 4D option was used to maximally reduce the X-ray dose. Since the aim of this study was to evaluate anatomical variations of sphenoidal sinuses septation only patients with no diseases within sphenoidal sinuses were included. All the subjects were Polish, with a mean age of 44 years, ranging from 18 to 84 years., Results: In our study there was only one septum in 41%, two septa in 33%, three septa in 22% and four septa in 4%. The total amount of sinuses depending on total amount of septa (including accessory septa) and it can be different in the anterior and posterior part of the sphenoidal sinus. We also notice that the highest and the broadest septum (main septum) was completely bony in 57% whilst in 47% it was partially membranous., Conclusions: Performing CT of paranasal sinuses before surgery is essential to avoid potential complications resulting from anatomical variations.

Temporosphenoidal encephalocele (TSE) is a rare entity caused by herniation of the anteromedial temporal lobe into the sphenoid sinus (SS) through a middle fossa (MF) defect. A 45-year-old woman presented with a spontaneous TSE manifesting as a 4-year history of recurrent cerebrospinal fluid rhinorrhea and meningitis. Coronal computed tomography showed a skull defect in the superior wall of the right lateral recess of the SS. This homogeneous intrasphenoidal lesion appeared hypointense on T(1)-weighted magnetic resonance (MR) imaging and hyperintense on T(2)-weighted MR imaging. The patient underwent a frontotemporal craniotomy and extradural MF exploration. The encephalocele was amputated and the temporal base dura primarily sutured and reinforced with fat graft. The MF hole was plugged with temporalis fascia and a calvarial graft layered over the bone defect. Histological examination confirmed meningoencephalocele. Rhinorrhea resolved and the patient remained asymptomatic. Resection of an anteromedial TSE and closure of the craniodural defect in the MF floor via a less invasive extradural skull base approach is effective.

A fistula of the lesser sphenoid wing sinus is a rare dural arteriovenous fistula resulting from a connection between the middle meningeal artery and recipient vein in the sinus of the lesser sphenoid wing. In this report, MRI/magnetic resonance angiography of a 54-year-old man who presented with sudden-onset glaucoma and proptosis revealed a fistula in this anatomic location. Drainage patterns here may account for the absence of serious complications and optimistic prognosis following embolization. Care in diagnosis is required to avoid superfluous procedures, because classic signs of the more common carotid-cavernous fistula are absent.

Hypoplasia and aplasia of maxillary sinus, maxillary sinus septae, ethmomaxillary sinus, superior meatus-draining maxillary sinus, and over-pneumatization are the variations of maxillary sinuses. Findings such as uncinate process abnormality, orbital enlargement, sphenomaxillary plate, canine fossa elevation, infraorbital fissure enlargement, thickening of the sinus wall and mucosal pathologies can be seen together with these variations. The aim of this study is to determine the incidence and morphology of the anatomical variations of maxillary sinus and accompanying structures. A total of 330 consecutive paranasal sinus computed tomography scans of the patients presenting with sinonasal complaints at our ENT department were assessed for maxillary sinus anatomical variations and related structures. There were 134 (20.3%) anteriorly and 17 (2.5%) posteriorly localized maxillary sinus bony septa. The position of antral septa was frequently vertical at anterior, and horizontal at posterior. We found significant correlation between the anteriorly localized maxillary sinus septa and infraorbital fissure enlargement. Maxillary sinus hypoplasia was found in 31 (4.6%) maxillary sinuses. Seven (1.0%) ethmomaxillary sinuses were assessed. Significant correlation was found between maxillary sinus hypoplasia and orbital enlargement. Presence of mucosal pathology was significant in patients with maxillary sinus septa, but there was no difference when it was compared with the mucosal pathology in the nonseptated group. There was no significant correlation between other anatomical variations and mucosal pathologies. Determining the anatomical variations of maxillary sinus and accompanying structures in patients planning endoscopic sinus surgery will significantly help with providing surgical orientation and preventing possible complications.

Neurofibromatosis type 1 (NF1) has been infrequently associated with different cerebrovascular conditions that may lead to either ischemic or hemorrhagic stroke. Intracranial dural arteriovenous fistulas have not been described in NF1 patients. In this paper we present a unique case of an 8-year-old child with florid NF1 that presented a greater sphenoid wing dural arteriovenous fistula draining directly through the superior ophthalmic vein and causing exophthalmos. The fistula was cured by direct trans-superior ophthalmic vein approach, by means of detachable coils. Only a few cases of dural fistulas in the lesser sphenoidal wing region have been described in the literature but a fistula of the greater sphenoid wing has not been previously described.

Maxillary sinus hypoplasia (MSH) is classified into three types depending upon embryological development of the sinus and uncinate process. Type III MSH is characterized by a near-absence of the uncinate process and an almost absent cleft-like sinus. Bilateral maxillary sinus aplasia or severe hypoplasia with associated paranasal sinus abnormalities is extremely rare. Two cases with severe maxillary sinus hypoplasia/aplasia (one of them with other associated paranasal sinus abnormalities) are presented in this article. This extremely rare abnormality should be kept in mind to prevent misdiagnosis and possible complications during endoscopic sinus surgery.

Transspheinoidal meningoencephalocele is a rare presentation of dysraphism of the neural tube. It is characterized by the herniation of the neural tissue through a bony defect in the sphenoid bone. The clinical presentation is variable. It may be assymptomatic or it may include an upper airway obstruction, rhinorrhea, meningitis, hypothalamic dysfunction and optic anomalies. The surgical treatment is controversial. We describe the case of a 7-year-old boy who presented a pulsate structure filling the palate, palate digenesis and hypertelorism. The diagnosis of transsphenoidal transpalatal meningoencephalocele was confirmed by a computerized tomography and a magnetic resonance imaging. The child was operated on by the transpalatal/transspheinoidal approach with a good result.

Basilar expansion of the sphenoidal sinus (BESS) was studied in order to demonstrate its critical relevance in endoscopic or microscopic endonasal surgical interventions, including access to the sphenoidal sinus itself or in transsphenoidal pituitary approaches. Direct evaluation of anatomical specimens (25 dry skulls and 25 formalin-fixed hemi heads) and the use of computerized tomography (CT) (50 dry skulls and 750 patients) showed a high BESS frequency (69%). The authors considered BESS to be critical when the posterior wall of the clivus was 2-mm thick and found a high incidence of this important anatomical variation (44%). This study also evaluated the relationship between the sinonasal septa, the clivus, and the internal carotid arteries, and a considerable regularity in the location of these structures was seen. The septa were anatomically related to the internal carotid arteries in 55% and to the clivus in 33% of the cases. In conclusion, the high frequency of critical BESS here described is relevant to endoscopic or microscopic endonasal surgical interventions, including access to the sphenoidal sinus itself or in transsphenoidal pituitary approaches.

Paranasal sinus agenesis is an unusual clinical condition. The frontal sinus agenesis is more frequently seen, whereas agenesis of the sphenoid sinus is extremely rare. This report presents three new cases of sphenoid sinus agenesis. The absence of the sphenoid sinus was demonstrated by computed tomography (CT) in all cases.

We describe CT scans of a case with bilateral aplasia of frontal and sphenoid sinuses with symmetrical hypoplasia of the ethmoid cellules and maxillary sinuses. This case appears to be first in the English-language literature with these combined findings.

A 22-year-old female patient presented to our hospital complaining of a headache. Evaluation with magnetic resonance imaging (MRI) demonstrated the absence of sphenoid sinus. Agenesis of sphenoid sinus is a rare entity; it usually occurs with syndromes. A case of sphenoid sinus agenesis without any anomaly or syndrome is reported., ((c) 2005 Wiley-Liss, Inc.)

The absence of a maxillary or sphenoid sinus in an adult is an extremely rare condition. We investigated maxillary and sphenoid sinus aplasia in adult Turkish individuals using computed tomography (CT). We examined CT scans in the axial and coronal planes of the paranasal sinuses in 1,526 patients. The CT scans of a 21-year-old male were notable for bilateral maxillary sinus aplasia. Another patient, a 20-year-old female, had CT scans that showed the unilateral absence of a maxillary sinus. Two additional cases showed the unilateral absence of one sphenoid sinus. Bilateral absence of the sphenoid sinuses was not observed in our study. The clinical implications of maxillary and sphenoid sinus aplasia will be further discussed.

Encephaloceles are uncommon and can arise from congenital, traumatic, or spontaneous origins. Approximately 80% of all cerebrospinal fluid rhinorrheas are caused by head injuries. Spontaneous or nontraumatic encephaloceles or cerebrospinal fluid leaks have been the least common in most series, accounting for only 3% to 5% of all cerebrospinal fluid leaks. There is a high incidence of meningitis and brain abscess. Thus, early diagnosis is very important. We present an adult patient with uncomplicated nontraumatic cerebrospinal fluid rhinorrhea that was caused by spontaneous trans-sphenoidal encephalocele.

Objectives: Our goal was to determine the incidental Lund score for pediatric patients without chronic rhinosinusitis (CRS)., Methods: Pediatric patients with computed tomography scans of the orbit or brain were identified. Patients with a clinical history of sinonasal disorders or skeletal facial trauma were excluded. The remaining scans were scored according to the Lund-MacKay system. Pneumatization of the frontal and sphenoid sinuses was also recorded. A scaled Lund score was computed, and the mean Lund score for this normal population was determined., Results: One hundred ninety-two computed tomography scans were examined (mean age, 9.0 years). The frontal sinuses and sphenoid sinuses were absent in 40.1% and 1.6% of cases, respectively. The mean scaled Lund score in this pediatric population without CRS was 2.81 (95% confidence interval, 2.40 to 3.22). This result differed statistically from a score of 0 (P < 0.001). Only 37 (19.3%) patients had completely radiographically normal sinuses., Conclusions: The mean incidental Lund score in the absence of CRS approaches 3, and this should be considered when determining the diagnostic likelihood of CRS in pediatric patients.

Sphenoidal encephalocele is a rare congenital anomaly. We report a case of sphenoidal encephalocele presenting with cerebral spinal fluid rhinorrhea. A 53-years-old man suffered recurrent fever and rhinorrhea. Computed tomography demonstrated a low density area extending from the left middle cranial fossa into the left sphenoidal sinus. Bone defect was identified in the medial surface of the left middle cranial fossa. Magnetic resonance imaging with contrast medium demonstrated peripheral enhancement of the lesion. We diagnosed the lesion as sphenoidal encephalocele. Surgical repair and amputation of the encaphalocele was performed. Sphenoidal encaphalocele should be considered as a possible diagnosis for recurrent fever and rhinorrhea.

Objective: To present a 17-year-old adolescent patient with lost right eye visual field and intermittent headache since the age of 14 years. The cranium was prominent at the right frontal region. The patient had no history of head trauma or infection and his past medical story was unremarkable. His mental status was normal. Computed tomography scanning revealed an abnormal occurrence of a common cavity deformity of the frontal, ethmoid, and sphenoid sinuses. No other lesions or deformities were present. Such common cavity deformity of these paranasal sinuses has not previously been reported in the English literature.

We present a 50-year-old woman with agenesis of the sphenoid sinuses. She was referred for evaluation of the aeration status of the sphenoid sinus and related nasal structures before endoscopic transsphenoidal hypophysectomy for pituitary microadenoma. Axial and coronal computed tomography (CT) scans revealed absence of the sphenoid sinuses and the right posterior ethmoidal cell. Agenesis of sphenoid sinuses documented using CT scans is uncommon. One case with a history of Hand-Schuller-Christian disease has been reported in the English language literature. In the case reported here agenesis of the sphenoid sinuses was not accompanied by craniofacial anomaly or skeletal disease.

Background: Encephaloceles are rare cranio-facial developmental anomalies., Case Report: In this paper, we discuss the case of an 8-month-old girl affected by a transsphenoidal meningoencephalocele and try to give landmarks of the state of art in the surgical treatment of this rare pathology.

Background: Transsphenoidal meningoencephalocele is a rare congenital anomaly, reported only in 14 adult patients. It may be subdivided into intrasphenoidal, extending into the sphenoid sinus, and true transsphenoidal, traversing the floor of the sinus and protruding into the nasal cavity or nasopharynx., Methods: We present the eighth case of true transsphenoidal meningoencephalocele reported in an adult. We review the literature and highlight the differences in clinical presentation and therapeutic management between patients with intrasphenoidal and true transsphenoidal meningoencephalocele., Conclusion: Adult patients with intrasphenoidal meningoencephalocele often present with rhinorrhea and should be considered for transsphenoidal repair, whereas patients with true transsphenoidal meningoencephalocele should not undergo surgery, in view of the complex anatomy and the slow progression of symptoms.

Purpose: To measure and compare the size of the sphenoid sinuses in patients with cystic fibrosis (CF) to patients with inflammatory sinonasal disease, and to correlate the size with number of CF mutations in each patient., Material and Methods: Ninety-six CF patients aged 5-47 years (median 19 years) and 130 control patients aged 7-51 years (median 32 years) were examined using coronal CT of the paranasal sinuses. In each patient, the CT image with the largest coronal area of the sphenoid sinuses was scanned into a Macintosh computer with image processing and analysis software. Largest coronal area and largest circumference of the right and left sphenoid sinuses were automatically measured. Additionally, antero-posterior extension of the sphenoid sinuses was calculated from the lateral scanograms. CF patients were grouped according to number of confirmed mutations (CF-0, CF-1, or CF-2)., Results: CF patients generally had small sphenoid sinuses. The largest differences for all parameters were observed between the CF-2 and the control groups (p<0.0001). No CF-2 patient had pneumatization beyond the presphenoid. The CF-0 and CF-1 groups consisted of two populations, one overlapping the CF-2 group and another overlapping the control group., Conclusion: Hypoplasia of the sphenoid sinuses is a characteristic finding in CF patients. When pneumatization of the basisphenoid is present, the existing CF diagnosis should be questioned.

The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.