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Intrasphenoidal encephalocoeles are acquired or congenital herniations of meninges and brain parenchyma through a structural sphenoid bone defect. Acquired causes are most common, either iatrogenic, post-traumatic, or spontaneous. However, defects in the lateral wall of the sphenoid sinus are uncommon and cephalocoeles through them relatively underexplored in current literature, warranting dedicated attention to unravel their complexities. Congenital causes such as persistence of Sternberg's canal, which can lead to lateral cephalocoeles, is a rare entity, seen in two of the presented cases, based on the location of the defect with respect to the line connecting the foramen rotundum and the vidian canal (VR line). Three cases of intrasphenoidal cephalocoeles are presented; two patients presented with watery nasal discharge without prior trauma or surgery and the third case was incidentally detected in an elderly patient with intraparenchymal haemorrhage. Imaging with CT cisternography and brain MR were performed to ascertain the exact location of the leak and confirm the presence of herniated brain tissue via the defects. Patients were evaluated by otolaryngology for transnasal endoscopic repair, which was deemed unfeasible, and referred to neurosurgery for transcranial duroplasty. Contribution: These cases provide crucial insights into the aetiology of lateral intrasphenoidal cephalocoeles, offering a practical system to classify cerebrospinal fluid (CSF) leaks based on the bony defect location. The three illustrative cases and emphasis on advanced imaging modalities refine the knowledge of their aetiology, clinical presentation and management, which hold direct clinical relevance for accurate diagnosis and tailored management of these rare anomalies.

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Congenital anomalies and defects of the skull base and calvarium encompass a broad and complex spectrum of pathologies. The clinical presentation is highly variable, and these anomalies may be discovered incidentally in asymptomatic individuals. Radiological assessment plays a pivotal role in precisely characterizing these abnormalities, facilitating the diagnostic process, and assisting in any preoperative preparation.

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BACKGROUND: Anomalies in the anatomical structure of the nasal cavity and paranasal sinuses often serve as a potential cause of spontaneous cerebrospinal fluid (CSF) leakage and may result in the development of a meningoencephalocele. In this report, the authors present a case of surgically treated intrasphenoidal meningoencephalocele attributed to the persistence of the lateral craniopharyngeal canal, which was further complicated by the occurrence of an intracerebral hematoma. OBSERVATIONS: A temporal lobe meningoencephalocele located in the lateral recess of the sphenoid sinus was successfully managed using endoscopic endonasal transpterygoid repair (EETR). However, an intracerebral hematoma developed after resection of the meningoencephalocele, necessitating additional surgical interventions. Despite this complication, the patient exhibited a favorable clinical outcome after the surgical interventions. LESSONS: This case highlights the potential risk of intracerebral hematoma associated with EETR of a lateral sphenoid sinus meningoencephalocele. A thorough examination of magnetic resonance imaging scans, especially identifying vascular structures, is crucial during surgical planning. This knowledge can help to prevent the occurrence of complications, including intracerebral hematoma.

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Background: Cerebrospinal fluid (CSF) rhinorrhea with meningoencephalocele (MEC) associated with Sternberg's canal is rare. We treated two such cases. Case Description: A 41-year-old man and a 35-year-old woman presented with CSF rhinorrhea and mild headache worsening with standing posture. Head computed tomography showed a defect close to the foramen rotundum in the lateral wall of the left sphenoid sinus in both cases. Head magnetic resonance (MR) imaging and MR cisternography revealed that brain parenchyma had herniated into the lateral sphenoid sinus through the defect of the middle cranial fossa. The intradural and extradural spaces and bone defect were sealed with fascia and fat through both intradural and extradural approaches. The MEC was cut away to prevent infection. CSF rhinorrhea completely stopped after the surgery. Conclusion: Our cases were characterized by empty sella, thinning of the dorsum sellae, and large arteriovenous malformations that suggest chronic intracranial hypertension. The possibility of Sternberg's canal in patients with CSF rhinorrhea with chronic intracranial hypertension should be considered. The cranial approach has the advantages of lower infection risk and the ability to close the defect with multilayer plasty under direct vision. The transcranial approach is still safe if performed by a skillful neurosurgeon.

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Background: A spontaneous cerebrospinal leak from Sternberg's canal with meningoencephalocele is a very rare clinical entity. Endoscopic repair of the defect is challenging and crucial in identifying the defect. The aim of this case report is to highlight the presence and management with endoscopic surgery in repairing Sternberg canal. Case: 40-year-old woman presents with spontaneous CSF rhinorrhea with no predisposing factors. CT imaging and MRI showed osteodural defect in the lateral recess of sphenoid with meningoencephalocoele lateral to the foramen rotundum. Endoscopic transethmoidal - transphenoidal - transpterygoid approach was used to repair the defect, and patient is well post-operative with least complication from the intervention surgery. Conclusion: Endoscopic approach proved to be the best and safest method in localizing the defect and closure of the leak. Angled scopes and image guided system were used to identify the precise location of the leak. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03347-z.

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A case of nasopharyngeal glial heterotopia with persistent craniopharyngeal canal is being reported. These lesions though rare should be considered in the differential diagnosis of neonates who present with nasal obstruction. Careful radiological evaluation for a persistent craniopharyngeal canal and differentiating the nasopharyngeal mass from brain tissue is of paramount importance.

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Persistent craniopharyngeal canal (PCC) is a rare congenital anomaly that appears as a linear well-corticated canal running from the sella through the clivus and into the nasopharynx. Case reports of this anomaly have shown it is associated with a range of craniofacial defects, pituitary abnormalities, and meningoencephaloceles. It predisposes patients to bacterial meningitis. In this case a 46-year-old gentleman presenting for preoperative planning for surgical drainage of Potts Puffy tumor was found to have a PCC on CT and MRI. Imaging also demonstrated the presence of chronic inflammation and a fistula extending from the tract into the sphenoid sinus. This unusual presentation of a PCC with a sphenoid sinus fistula broadens the potential clinical presentations of PCC and further emphasizes the ability of this anomaly to serve as a conduit for CNS infection.

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Craniopharyngeal canal persistence is a congenital abnormality characterized by a bone canal extending from the nasopharynx to the pituitary fossa. Ascension of microorganisms through the channel can occur, triggering meningitis.

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BACKGROUND: The lateral craniopharyngeal or Sternberg's canal (SC) originates from superior orbital fissure (SOF) and traverses the sphenoid body into the nasopharynx. A remnant of the canal, Sternberg's defect (SD), has been debated as a source of cerebrospinal fluid (CSF) leak. The canal was described in 1888, and there is limited accurate visual illustration in the literature. OBJECTIVE: To provide a detailed anatomic and radiological illustration of the canal in pediatric and adult population including the mechanism undermining the incidence of the canal, and the possibility of the canal as a source of CSF leak. METHODS: A total of 195 high-resolution computed tomographies (CT) of patients (50 3-yr-old, 20 5-yr-old, and 125 adults) and 43 dry adult skulls (86 sides) were analyzed for a canal matching the description of the SC. RESULTS: A SC was identified in 86% of the 3-yr-old and 40% of 5-yr-old patients. The diameter and length were 2.12 mm and 12 mm, respectively. The incidence of the canal decreased with age as sinus pneumatization extended into the sphenoid sinus. Only 0.8% of the adult skull on CT had the canal. The canal was not present on the dry adult skulls examination, but SD was found in 4.65%. CONCLUSION: SC exists with high incidence in the pediatric group. Sinus pneumatization obliterates the canal in the adult population, leaving a defect in 4.65% of cases, which given the location and related anatomic structures, is unlikely to be a source of CSF leak.

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PURPOSE: The clivus is a part of the sphenoid bone. It may show some anatomical variations such as fossa navicularis magna (FNM), canalis basilaris medianus (CBM) and craniopharyngeal canal (CPC). These variations have been associated with conditions like meningitis and tumors of skull base. Inadequate information about these structures may pose a risk of inaccurate diagnosis resulting in unwarranted interventional procedures. Hence, the knowledge about the prevalence of these variations is important. Thus, the objective of this study was to determine the prevalence of fossa navicularis magna, craniopharyngeal canal and canalis basilaris medianus and its types utilizing cone-beam computed tomography (CBCT) images. METHODS: Retrospectively, a total of 350 CBCT scans were evaluated for the presence of FNM, CBM, its types and CPC. The analysis was done by two observers independently. Cohen's kappa statistics was used to determine the interobserver agreement. RESULTS: FNM was noted in 19.4% cases, CBM in 9.7% cases, whereas CPC was not identified in any case. Type 5 was the most common type of CBM. There was no significant difference (p > 0.05) between genders and age groups for the prevalence and a highly significant (p < 0.01) substantial agreement between observers for the prevalence of FNM and highly significant (p < 0.01) moderate agreement for the prevalence of CBM and its types was obtained. CONCLUSION: FNM, CBM, CPC, albeit rare anatomical variations of clivus, knowledge of these structures is important for radiologists, anatomists and surgeons to avoid misdiagnosis and provide awareness to the individual of a higher possibility of meningitis or tumors of the skull base.

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BACKGROUND: A persistent craniopharyngeal canal (PCC) is a rare but treatable anatomical abnormality that causes recurrent meningitis and should be considered as a differential diagnosis. CASE REPORT: We report a case of an 8-year-old boy who presented with recurrent meningitis associated to his PCC. Surgical repair was performed, and no further episodes occurred.

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We present the case of a 5-year-old girl who had six episodes of meningitis. She also had panhypopituitarism and was found to have a persistent craniopharyngeal canal (CPC) as the cause of her recurrent meningitis. Role of neuroradiology and a high index of suspicion by the clinical team are highlighted here. Persistent CPC is a rare cause of recurrent meningitis. We discuss the approach to the child with recurrent meningitis.

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Objectives A persistent craniopharyngeal canal (CPC) is a rare embryologic remnant that presents as a well-corticated defect of the midline sphenoid body extending from the sellar floor to the nasopharynx. Our case series aims to describe three unique presentations of this congenital anomaly and their subsequent management. Design Retrospective review. Setting Tertiary academic medical center. Participants Patients who underwent endoscopic transnasal surgical repair of a CPC lesion. Main Outcome Measures Resolution of symptoms and surgical outcomes. Results A total of three patients were identified. The clinical presentation varied, however, all cases prompted further imaging which demonstrated a persistent CPC and associated pathologic lesion. The presentation of a persistent CPC with nasal obstruction and subsequent iatrogenic cerebrospinal fluid leak as in Case 1 demonstrates the importance of imaging in this work-up. Cases 2 and 3 in the series were representative of the larger subset of patients in the literature who present with the defect incidentally but still warrant surgical management. Nonetheless, a standard approach to diagnosis with preoperative imaging and subsequent transnasal endoscopic repair of the skull base defect was undertaken. Conclusion The persistent CPC is a rare congenital anomaly associated with diverse pathology and careful review of preoperative radiology is critical to the management. When warranted, subsequent surgical repair and reconstruction is associated with excellent postoperative outcomes.

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Nasopharyngeal gliomas are congenital masses of heterotopic glial tissue. Our case report describes an infant with respiratory distress produced by a nasopharyngeal glioma with a persistent craniopharyngeal canal (CPC). The identification of CPC is important for medical and surgical management of possible pituitary dysfunction and preventing operative and post-operative complications.

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PURPOSE: Several skull-base foramina including foramen meningo-orbitale, craniopharyngeal canals, canaliculus innominatus, foramen vesalius, palatovaginal canals, and canalis basalis medianus are visible on cone-beam computed tomographs. A good understanding of the anatomical variants of these foramina is important to accurately diagnose fractures, understand the paths that infections may take, and identify associated anomalies. We used cone-beam computed tomography to measure the incidences of skull-base foramen variants in a normal population. METHODS: A total of 350 subjects (200 females, 150 males, 6-30 years of age) were included. The prevalences of foramen meningo-orbitale, craniopharyngeal canals, canaliculus innominatus, foramina vesalius, palatovaginal canals, and canalis basalis medianus were evaluated by age and gender. RESULTS: Subject age ranged from 6 to 30 years (mean age ± SD = 15.1 ± 4.08). Foramen meningo-orbitale, craniopharyngeal canals, canaliculus innominatus, foramen vesalius, palatovaginal canal, and canalis basalis medianus were observed in 51 (14.6%), 19 (5.4%), 60 (17.1%), 145 (41.1%), 34 (9.7%), and 15 (4.3%) patients, respectively. CONCLUSIONS: Skull-base foramina are important clinically and radiologically. Imaging of such variants via cone-beam computed tomography is valuable for both physicians and patients. Few studies of skull-base foramina have used cone-beam computed tomography. Additional research is required for a fuller understanding of this phenomenon.

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PURPOSE: This study determined the prevalence of fossa navicularis magna (FNM), canalis basilaris medianus (CBM), and craniopharyngeal canal (CPC), the size of FNMs, and types of CBM using 3D computed tomography (CT) images. METHODS: A total of 1059 3D images [649 cone beam computed tomography (CBCT) and 410 CT] were evaluated in this study. The prevalence of FNM, CBM, and CPC, length, width, and depth of FNM, and type of CBM were assessed. RESULTS: Overall, FNM was identified in 7.6%, CPC in 0.3%, and CBM in 2.5% of the study group. Type 2 (0.1%) and Type 6 (0.1%) are the least common CBM types. There was no significant difference between genders for depth and width measurements (p > 0.05), however, the length of FNM was significantly higher in males than females in CBCT images (p = 0.02). CONCLUSION: FNM, CBM, and CPC are rare anatomical variants of clivus. However, they can facilitate spread of infection to the skull base or vice-versa. These types of anatomical variations should be known by radiologists to avoid unnecessary diagnosis and treatment procedures and to distinguish anatomic variations from pathological conditions.

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BACKGROUND: A persistent craniopharyngeal canal (PCC) is a rare cause of cerebrospinal fluid rhinorrhea in children. The condition often coexists with other midline facial defects, such as cleft palate. Children with PCC may also have pituitary dysfunction or neoplasms, such as craniopharyngiomas within the canal. CASE DESCRIPTION: A 5-year-old girl presented with bacterial meningitis and active cerebrospinal fluid rhinorrhea from her left nostril. Imaging showed a large nasopharyngeal meningoencephalocele, communicating with the subarachnoid space through a persistent craniopharyngeal canal. An endonasal approach was chosen to excise the PCC and meningoencephalocele and to repair the resulting skull base defect. CONCLUSIONS: The extended endonasal approach can be used to treat PCC with nasopharyngeal encephaloceles in young children. The approach is suitable to address both conditions at the same time. The extended endonasal approach avoids potentially morbid transfacial approaches and can help in earlier recovery after surgery.

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Trans -sellar trans-sphenoidal encephalocele is an extremely rare entity. We present the case of an 18-month old boy who presented with a trans-sellar, trans-sphenoidal encephalocele associated with cleft lip, cleft palate and microphthalmia. This patient was treated successfully by a trans-cranial extra-dural route. In this paper, we discuss the clinico-radiological findings as well as various surgical options in managing these rare lesions and briefly review the literature.

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INTRODUCTION: Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed. CASE PRESENTATION: We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst. He also has ectopic neurohypophysis and optic atrophy. CONCLUSION: The presence of orbital and optic tract malformations, craniofacial and intracranial anomalies, and tumors can accompany craniopharyngeal canals (CCs). MRI is helpful in the evaluation of PCC and associated anomalies in these patients.

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We describe the neuroimaging findings of an 11-year-old boy who presented with mild occipital headache and precocious puberty. This child was found to have a combination of various midline anomalies including a Chiari type 1 malformation, corpus callosum agenesis and patent craniopharyngeal canal with adjacent intracranial dermoid cyst.

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