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Preoperative sagittal (left) and coronal (right) T1weighted MR images demonstrating a transsphenoidal cephalocele.
Source publication
Transsphenoidal encephaloceles are rare and the transsellar variety is the least common. We present a 1-year-old male patient with transsellar transsphenoidal encephalocele which herniated into the oral cavity through the congenital split palate. The patient was operated on using a combined transcranial and transpalatal approach without mortality o...
Context in source publication
Context 1
... 1-year-old male with a diagnosed transsphenoidal encephalocele was referred to us in 2005. There was a history of a slowly progressive mass in the mouth since birth and his attending pediatrician advised neurosurgical repair after cranial MRI. He had been born at the term with a body weight of 2750 g. He was the first child of his parents and there was no consanguinity. His mother had no history of drug or another chemical agent using during pregnancy. On examination the child was alert and playful. His body weight was 8500 g, body height was 67 cm and the head circumference was 44 cm. He had no neurological deficit. His mental milestones were normal. He had a mass, which was partially compressible, in the mouth 4 cm in diameter and herniating from the nose through the split soft and hard palate. A transsellar transsphenoidal rhino-oral wide encephalocele was diagnosed on the MRI (Figure 1). The CT scan with bone window settings, including a paranasal sinus view, revealed a large defect in the planum sphenoidale and sella turcica (Figure 2). There was no other cerebral malformation. The results of laboratory examinations including hematological, endocrinological and chromosomal studies were ...
Citations
... The incidence of encephalocele ranges from 1 in 3000 to 1 in 5000 thousand live births [1]. The incidence of basal cephaloceles is thought to be 1 in 35,000 live births, making them rare anomalies. ...
... Most trans-sphenoidal meningoencephaloceles are unearthed in the first year of life as a result of symptoms like respiratory distress brought on by pharyngeal or nasal obstruction, feeding issues, hypertelorism, a mass in the oral cavity, and endocrine anomalies [1,20]. Corpus callosum agenesis occurs in approximately half of the afflicted individuals, as does hydrocephalus and pituitary hypoplasia. ...
... Additionally, although this method has the advantage of effectively repositioning herniated tissue, some studies indicate a morbidity rate of up to 70% and a mortality rate of up to 50% [2,4,27]. The most common complications are associated with the removal of functioning neural tissue, anosmia, intracerebral hemorrhage, and frontal lobe dysfunction [1,3,4]. The repair of basal defect can be done with either an autologous graft or titanium mesh. ...
Trans-sellar trans-sphenoidal encephalocele is a rare congenital anomaly, with only around 20 cases having been documented in literature around the world. Surgical repair of these defects in the pediatric population commonly uses either the transcranial or the transpalatal approach, with the choice of approach being individualized based on the clinical features, age, and associated defects present in the patient. Here, we document a case of a 4-month-old child who presented to us with nasal obstruction, who was diagnosed with this rare entity and successfully underwent a transcranial repair for the same. We also provide a systematic review of all existing case reports that have described this rare condition in the pediatric population, as weel as the different surgical approaches used in each case.
... The incidence of encephalocele ranges from 1 in 3000 to 1 in 5000 thousand live births [1] . The incidence of basal cephaloceles is thought to be 1 in 35,000 live births, making them rare anomalies. ...
... Most transsphenoidal meningoencephaloceles are unearthed in the rst year of life as a result of symptoms like respiratory distress brought on by pharyngeal or nasal obstruction, feeding issues, hypertelorism, a mass in the oral cavity, and endocrine anomalies [1,5] . A third of the patients have congenital anomalies such as hypertelorism, median nasal ssure, broad nasal root, and cleft lip or palate. ...
Trans sellar trans sphenoidal encephalocele is a rare congenital anomaly, with only around 10 cases having been documented in literature around the world. Surgical repair of these defects in the pediatric population commonly uses either the trans cranial or the trans palatal approach, with the choice of approach being individualized based on the clinical features, age, and associated defects present in the patient. Here we document a case of a 4-month-old child who presented to us with nasal obstruction, who was diagnosed with this rare entity and successfully underwent a transcranial repair for the same.
... The incidence of congenital encephalocele is approximately 1 in 3000-5000 live births.[28] Trans-sphenoidal encephaloceles comprise less than 5% of all basal meningo-encephaloceles and has an estimated incidence of 1 in 700000 live birth.[161719] We performed a comprehensive search in the available English literature from 1970 to April 2011. ...
... The management of the trans-sphenoidal encephalocele require a multidisciplinary approach.[172224] Trans-sphenoidal encephalocele have been treated by the trans-cranial, trans-palatal, and trans-sphenoidal approaches. ...
Transsphenoidal encephaloceles represent a rare clinical entity with wide range of symptoms in adult. Such lesions require accurate diagnosis and surgical treatment. The incidence of congenital encephalocele is very low occurring in approximately 1 in 3000-5000 live births. Even though 63 similar cases have been reported in the literature not all of them have been discussed completely. Due to the rare occurrence of these lesions, we will focus on the main clues in the diagnosis and management of such lesions, which are challenging.
We intend to present our experience with two cases of trans-sphenoidal meningoencephalocels, one located medially and the other herniating through the Sternberg's canal. The younger was 17 and the elder was 47 years old and both of them presented with cerebrospinal fluid (CSF) leakage. Both patients were treated successfully using pure endoscopic endonasal approach.
Ideal surgical approach for such patients is still not clear due to lack of adequate experience in the literature, it is suggested that full preoperative imaging studies might lead the surgeons to undertake minimally invasive skull base approaches in similar patients.
BACKGROUND
Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis.
OBJECTIVE
To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue.
METHODS
A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac.
RESULTS
We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula.
CONCLUSION
The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.
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.
OBJECTIVE
Congenital transsphenoidal encephaloceles are rare malformations, and their surgical treatment remains challenging. This paper reports 3 cases of transsphenoidal encephalocele in 8- to 24-month-old infants, who presented mainly with airway obstruction, respiratory distress, and failure to thrive.
METHODS
The authors discuss the surgical management of these lesions via a minimally invasive endoscopic endonasal approach, as compared with the traditional transcranial and transpalatal approaches. A unique endonasal management algorithm for these lesions is outlined. The lesions were repaired with no resection of the encephalocele sac, and the cranial base defects were reconstructed with titanium mesh plates and vascular nasoseptal flaps.
RESULTS
Reduction of the encephalocele and reconstruction of the skull base was successfully accomplished in all 3 cases, with favorable results.
CONCLUSIONS
The described endonasal management algorithm for congenital transsphenoidal encephaloceles is a safe, viable alternative to traditional transcranial and transpalatal approaches, and avoids much of the morbidity associated with these open techniques.
Object:
Middle fossa floor dehiscence (MFFD) can present as multiple syndromes depending on dehiscence location, tissue herniation, and dural integrity. The authors propose a classification system for MFFD with the potential to guide clinical decision making.
Methods:
A retrospective analysis of the electronic medical records (years 1995-2012) of patients who had undergone temporal craniotomy for the surgical repair of an MFFD syndrome at a single institution was undertaken. Reviewed data included demographic, operative, presentation, and outcome details. Middle fossa floor dehiscence was classified as follows: Class A, bony dehiscence without herniation of the brain and/or meninges; Class B, herniation of the brain and/or meninges through the middle fossa floor without CSF leakage; Class C, dehiscence with CSF leakage without meningitis; or Class D, dehiscence with meningitis.
Results:
Fifty-one patients, 22 males and 29 females, were included in the analysis. The mean age was 48.7 ± 15.5 years, mean body mass index was 32.65 ± 6.86 kg/m(2), and mean symptom duration was 33 ± 42 months. Seven patients underwent repeat surgery for symptomatic recurrence; therefore, there were 58 surgical encounters. Repair included bony reconstruction with hydroxyapatite with or without resection of encephaloceles and/or repair of a dural defect. According to the MFFD classification system described, 15, 8, 27, and 8 cases were categorized as Class A, B, C, and D, respectively. The prevalence of hearing loss was 87%, 63%, and 70% in Classes A, B, and C, respectively. Vestibular symptoms were more prevalent in Class A. Seven patients reported persistent symptoms at the last follow-up. Transient complications were similar in each classification (13%-25%), and a single permanent complication related to anesthesia was observed. There were no mortalities or severe neurological morbidities in the series.
Conclusions:
Middle fossa floor dehiscence has a spectrum of clinical presentations. A classification system may help to clarify the diagnosis and guide therapy. Surgery, the mainstay of treatment, is safe and well tolerated.
Basal encephaloceles are rare, accounting for about 1.5 % of all encephaloceles. Transsphenoidal encephaloceles represent less than 5 % of basal encephaloceles. Respiratory and feeding difficulties due to mass effect in the oral or nasal cavity and episodes of recurrent meningitis are the main clinical features. Diagnosis is established in the first year of life, but without characteristic facies, the diagnosis can be delayed to adolescence or adulthood. We report the case of a 10-year-old boy who presented with short stature and eventually was diagnosed with a growth hormone deficiency because of mass effect of transsphenoidal encephalocele. Unusual presentation of an encephalocele as a short stature is described.
Background
Intrasphenoidal encephalocele (ISE) is a rare clinical entity. The incidence of congenital encephalocele is very low. Accurate diagnosis and surgical approach is of critical value.
Case Reports
We present a case of intrasphenoidal encephalocele in a 40-year-old man. He complained of cerebrospinal fluid (CSF) rhinorrhea and recurrent meningitis. In images of computed tomography (CT) and magnetic resonance imaging (MRI), intrasphenoidal encephalocele herniating through a defect of the left lateral sphenoid sinus wall was determined. Incisional biopsies were taken by endoscopic transnasal approach and histopathological examination revealed an encephalocele. In the differential diagnosis, ISE can be taken for inflammatory or malignant sinusoidal soft tissue masses. ISE is differentiated from other entities by demonstrating continuity with normal brain tissue.
Conclusions
MRI clearly demonstrates that the herniating soft tissue is isointense with brain and continuous with brain tissue via the sphenoid sinus, thereby the treatment decision-making process is very important.
Respiratory distress in an infant is a common cause of admission in neonatal intensive care facility. Obstructive lesions of the airway constitute a minority of problems in the new born but present a diagnostic challenge. We present a 30-day-old male infant admitted with respiratory distress who was diagnosed to have an oropharyngeal cystic mass which on further evaluation by computed tomography and magnetic resonance imaging revealed a transalar transsphenoidal meningoencephalocele herniating into the oral cavity through a congenital split palate. The patient was operated successfully using a transpalatal approach leading to complete resolution of respiratory distress.
