Total Aplasia of Paranasal Sinus Associated With a Syndromic Condition.

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.

Sphenoid sinus agenesis and sella turcica hypoplasia: very rare cases of two brothers with Hamamy syndrome.

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.

Sphenoid sinuses: pneumatisation and anatomical variants-what the radiologist needs to know and report to avoid intraoperative complications.

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.

Contralateral Precaruncular Approach to the Lateral Sphenoid Sinus-A Case Report Detailing a New, Multiportal Approach to Lesions, and Defects in the Lateral Aspect of Well-Pneumatized Sphenoid Sinuses.

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.

Completely Dehiscent Posterior Sphenoid Sinus Wall During Endoscopic Pituitary Surgery.

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.

Carotid canal and optic canal at sphenoid sinus.

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.

Arrested pneumatization of the sphenoid sinus mimicking skull base tumours: MRI prevalence in children with haematologic diseases.

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.

The Onodi Cell: An Anatomic Illustration.

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.

Eustachian tube communicating with sphenoid sinus: report of a novel anatomical variant.

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.

Lateral Craniopharyngeal Canal: An Anatomical Cause of Spontaneous Rhinorrhea.

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--additional findings?

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.

Reparación endoscópica de encefalocele esfenoidal, secundario a persistencia del canal de Sternberg / Endoscopic repair of sphenoid encephalocele, secondary to persistence of Sternberg's canal

Las fístulas de líquido cefalorraquídeo (FLCR) nasales se producen por la comunicación entre el espacio subaracnoídeo y el tracto aerodigestivo. Pueden ser adquiridas (secundarias a trauma o iatrogenia) o espontáneas. Éstas últimas pueden ser de origen congénito, tumoral o idiopáticas. El canal lateral craneofaríngeo o de Sternberg se produce por la falta de fusión de los puntos de osificación del seno esfenoidal durante el período embrionario. Esta región, ubicada posterolateral a la pared inferior del seno esfenoidal, queda cubierta solamente por tejido conectivo, siendo la zona de menor resistencia de la base de cráneo. La persistencia de este canal puede causar FLCR, especialmente cuando se asocia a elevación de la presión intracraneana y extensa neumatización. La cirugía endoscópica nasal es la técnica de elección para la resolución de estos casos. En el presente artículo se realiza una revisión de la técnica quirúrgica endoscópica nasal para la resolución quirúrgica de encefaloceles secundarios a persistencia del canal de Sternberg, en base a dos casos clínicos que fueron sometidos a cirugía endoscópica nasal.

Nasal cerebrospinal fluid leaks are caused by a nasal communication between the subarachnoid space and the aerodigestive tract. They may be acquired (secondary to trauma or iatrogenic) or spontaneous. The latter can be congenital, tumoral or idiopathic. The lateral craniopharyngeal canal or Sternberg canal is caused by the lack of fusion of the points of ossification of the sphenoid sinus during the embryonic period. This region, located posterolateral to the bottom wall of the sphenoid sinus, is covered only by connective tissue, being the area of least resistance of the skull base. The persistence of this canal can cause cerebrospinal fluid leaks, especially when associated with elevated intracranial pressure and extensive pneumatization. Nasal endoscopic surgery is the technique of choice for solving these cases. This article is a review of the endoscopic surgical technique of encephaloceles secondary to persistence of Sternberg canal, based on two clinical cases.

A Unique Case of Bilateral Recurrent Sphenoid Sinus Cerebrospinal Fluid Leaks: Primary Acquired Leak Within the Lateral Sphenoid Sinus Recess, Followed by a Leak via Sternberg's Canal.

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.

[Sphenoid sinus agenesis]. / Sfenoid sinüs yoklugu.

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.

[Spontaneous nasal liquorrhea and the Sternberg canal: scientific hypothesis and personal experience].

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.

The significance of magnetic resonance imaging and computed tomography findings in sphenoid sinus agenesis.

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.

Unusual presentation of an asymptomatic pseudomeningocele within the sphenoid sinus.

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.

Surgical treatment of transsphenoidal encephaloceles: transpalatal versus combined transpalatal and transcranial approach.

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.