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Sphenoethmoidal Encephalocele: A Case Report

Authors:
D S Acherman
D S Acherman
D S Acherman
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Diederik K Bosman at Academisch Medisch Centrum Universiteit van Amsterdam
Diederik K Bosman
Chantal M A M van der Horst at Academisch Medisch Centrum Universiteit van Amsterdam
Chantal M A M van der Horst
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Objective: This article documents the characteristics and treatment of an infant patient with a sphenoethmoidal encephalocele. An extracranial, transpalatal approach was used to eliminate the encephalocele.
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... The surgical management of transsphenoidal encephalo- celes is typically attempted via either transcranial, transoral, or transnasal endoscopic approaches 10 , with most surgeons opting for the transoral route for cases of transsphenoidal encephaloceles with cleft palate, because there is less risk of damaging the functioning tissues within the wall of the en- cephalocele, and the herniated sac can be separated from the nasal mucosa, enabling adequate closure. ...
... Surgery is usually indicated in symptomatic cases or in cases in which life threatening complications, like CSF leaks, meningitis, or visual disturbance secondary to the lesion, might be result from failure to intervene 10 . Furthermore, the prognosis in cases treated by neurosurgical intervention is reserved, with only slightly more than half of treated cases presenting a favorable evolution, as described in a study by Macfarlane et al. 11 who demonstrated that only 59% of 114 cases resulted in normal development. ...
Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: A case report
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  • Apr 2018
About one-third of patients with transsphenoidal basal encephaloceles have associated congenital anomalies, including cleft palate. Moreover, they are often plagued by symptomatic exacerbations in the form of upper respiratory obstructions, cerebrospinal fluid leaks, meningitis, etc., with few patients being asymptomatic. We herein present a rare asymptomatic case of transsphenoidal basal encephalocele in an 18-month-old child with cleft palate and highlight a modified version of two-flap palatoplasty.
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... In our third case, snoring was a sign of airway obstruction due to the meningocele. Noisy breathing sounds have been reported as a clue to the diagnosis [9,10]. ...
Congenital basal meningoceles with different outcomes: A case series
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  • Dec 2017
  • J Med Case Rep
Background: Basal meningoceles are rare congenital defects and often clinically occult until they result in life-threatening complications. Therefore, it is important to know the diagnostic clues to early diagnosis. Case presentation: We describe three cases of congenital basal meningocele in a 3-year-old Japanese boy, a 1-month-old Japanese baby boy, and a 10-month-old Japanese baby girl. One of our patients died of sepsis due to traumatic rupture of the meningocele during nasal suction. His meningocele remained undiagnosed until it resulted in the fatal complication. The other patients underwent surgical repair without any complications. Their meningoceles were complicated by midfacial anomalies including cleft palate and hypertelorism, or a sign of nasal obstruction such as snoring. Conclusions: These clinical features may be a clue to the early diagnosis of congenital basal meningocele, which enables its safe preoperative management and provides an opportunity for surgical repair before the condition results in serious complications.
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The Orbit
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  • Sep 2023
The orbit represents a unique confluence of embryonic tissue sources: (1) neural crest from several neuromeric levels and (2) paraxial mesoderm from 5 of the 7 somitomeres. It is the anatomic surround which protects and permits the function of the visual apparatus, par cranium and part facial skeleton. The evolutionary history of the primate orbit reflects a series of changes in the size, shape and position of its osseous developmental fields which lead to the development of binocular gaze. This phraseology is deliberate: as these fields morphed in their relative position to each other, the visual apparatus was carried into a frontal position. Stereopsis was accompanied by concomitant changes in circuitry and the visual fields. The acquisition of color vision tops off the story. The learning objective of this chapter is to gain an appreciation for how developmental fields evolved over time to achieve this end. This chapter will deal with the anatomic components of the orbit, emphasizing neuromeric relationships, to better understand both the how and the why of the orbit. The reader is advised to study carefully the various figures. In the case of the orbital ones, note changes of their position in the overall architecture of the skull. Neurovascular structures will be discussed in the light of the twin embryonic systems of the orbits: derivatives of the primitive internal carotid system and those structures supplied by the evolutionary newcomer, the stapedial system. The evolutionary advantages of binocular vision are an additional theme of this chapter. Although the basic neuroanatomy of the mammalian orbit varies little between rabbit and man, the functional repositioning of the orbit into its frontalized state permitted an increasing complexity of depth perception and motor coordination culminating in the “invention” of the opposable thumb-permitting hominis to produce tools and gain mastery of the environment.
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Single Surgeon’s Experience in Repair of Median Cleft Lip: A Case Series of 5 Median Craniofacial Dysplasia Patients
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Median craniofacial dysplasia is a rare congenital anomaly with a broad spectrum of severity, which can be classified as hypoplasia, dysraphia, and hyperplasia, depending on the involved tissue amount. A retrospective chart review was performed of patients with median craniofacial dysplasia who underwent repair of the upper lip median cleft between January 2013 and February 2020. The median cleft of the upper lip was present in 5 cases. The average age at operation was 11 months. Two patients had a median notch in the vermilion, 2 patients had an incomplete median cleft lip, and 1 patient had a complete median cleft lip with the absence of columella, prolabium, and premaxilla. A variety of surgical correction was performed for each case, including simple rhombus-shaped excision, modified version of straight-line repair, and columella reconstruction using an intranasal dorsal flap and bilateral cleft margin flaps. Each case needs to be carefully assessed with individualization for appropriate surgical treatment.
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Spontaneous symptomatic orbital meningoencephalocele in an adult patient. Case report and review of the literature
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  • J CLIN NEUROSCI
Symptomatic spontaneous meningoencephalocele (MEC) is a very rare entity in adults and there have been no reported cases of spontaneous MEC through the orbital roof in an adult. We report a 41-year-old woman who presented with a left eyelid swelling for several weeks without any history of trauma. Brain magnetic resonance imaging (MRI) showed a MEC through the orbital roof causing a significant blepharocele in this young patient. Supraorbital craniotomy was performed to repair the bone defect. The symptoms resolved immediately after surgery. Even though blepharocele is a rare manifestation of spontaneous orbital MEC it should be considered in the differential diagnosis for appropriate surgical management.
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Cerebrospinal Fluid Rhinorrhea and Seizure Caused by Temporo-Sphenoidal Encephalocele
Article
Full-text available
  • Apr 2015
This case report describes the symptoms and clinical course of a 35-year-old female patient who was diagnosed with a temporo-sphenoidal encephalocele. It is characterized by herniation of cerebral tissue of the temporal lobe through a defect of the skull base localized in the middle fossa. At the time of first presentation the patient complained about recurrent nasal discharge of clear fluid which had begun some weeks earlier. She also reported that three months earlier she had for the first time suffered from a generalized seizure. In a first therapeutic attempt an endoscopic endonasal approach to the sphenoid sinus was performed. An attempt to randomly seal the suspicious area failed. After frontotemporal craniotomy, it was possible to localize the encephalocele and the underlying bone defect. The herniated brain tissue was resected and the dural defect was closed with fascia of the temporalis muscle. In summary, the combination of recurrent rhinorrhea and a first-time seizure should alert specialists of otolaryngology, neurology and neurosurgery of a temporo-sphenoidal encephalocele as a possible cause. Treatment is likely to require a neurosurgical approach.
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The Transpalatal Approach to Repair of Congenital Basal Skull Base Cephaloceles
Article
  • Apr 2014
Basal skull base herniations, including meningoceles and encephaloceles, are rare and may present with characteristic facial and neurologic features. The traditional craniotomy approach has known morbidity, and nasal endoscopy may not allow for control of large posterior basal defects, especially in newborns. We present two cases of successful repair of basal transsphenoidal meningoceles using an oral-transpalatal approach. The first patient with an intact palate presented with respiratory distress, and a palatectomy was performed for access to the skull base. The second patient had a large basal herniation that was reduced through a congenital midline cleft palate, and a calvarial bone graft was used to repair the defect. A literature search revealed 10 previous successful cases using the transpalatal repair, which allows for excellent access, low morbidity, and a team-oriented method to skull base surgery.
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Acquired Anterior Basal Encephalocele in Idiopathic Hypertrophic Pachymeningitis
Article
  • Jan 2009
This report describes a 49-year-old woman diagnosed as idiopathic hypertrophic pachymeningitis (IHCPM) with imaging evidence of diffuse dural sinus thrombosis. Over the years, secondary to the raised intracranial pressure, she had developed an anterior basal encephalocele through the defects in the cribriform plate of the ethmoid bone. The relation between elevated intracranial pressure and encephalocele is discussed.
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The Spectrum of Median Craniofacial Dysplasia
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  • Feb 2011
Given the multiple permutations in craniofacial malformations, classification of median craniofacial dysplasia or midline Tessier no. 0 to 14 clefts has been difficult and disjointed. In this review, the authors present a summary of normal embryology, prior terminology, and their proposed new classification system. Median craniofacial dysplasia has tissue agenesis and holoprosencephaly at one end (the hypoplasias), frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, and abnormal splitting or clefting and normal tissue volume (dysraphia) occupying the middle portion of the spectrum. These three distinct subclassifications have different forms of anomalies within their groups.
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Space-occupying lesion of the nasopharynx and skull base
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  • Oct 2010
  • HNO
Wir berichten über eine 73-jährige Patientin, die sich mit einer Raumforderung im Bereich des Nasenrachens, der Nasenhaupthöhlen sowie beidseitigen Paukenergüssen vorstellte. In der durchgeführten Magnetresonanztomographie zeigte sich eine bilateral-symmetrische liquorintense Raumforderung der Schädelbasis, einer transsphenoidalen Meningozele entsprechend. Da diese Diagnose einen Zufallsbefund darstellt und keine Beschwerden vorliegen, besteht keine Indikation für eine mit hohem Risiko behaftete operative Intervention.
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Congenital hypothalamic hamartoma associated with severe midline defect: A developmental field defect. Report of a case
Article
  • Jan 1986
  • Am J Med Genet
We describe a median “cleft” face anomaly (MCFA) with congenital hypothalamic hamartoma in a newborn girl. The MCFA was associated with a frontal midline skull lipoma and a complex congenital heart defect. Possible pathogenetic mechanisms are discussed, and a review of the pertinent literature is given. It is concluded that probably all malformations in our patient are disturbances of a single developmental field defect, ie, the midline.
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Anterior Basal Encephalocele of the Neonatal and Infantile Period
Article
  • Sep 1986
: Diagnosis of occult basal encephalocele is not difficult if the peculiar clinical and radiological signs of this anomaly are borne in mind. Recent surgery for transsphenoidal encephalocele has had better results than realized. However, high surgical risks may still be encountered in transsphenoidal encephalocele of the early infantile period, because the pituitary-hypothalamic structures are usually incorporated in the herniated encephalocele of this age group. Surgical indications for and operative approaches to transsphenoidal encephalocele in the infantile period are discussed on the basis of the authors' failure in transcranial repair, which resulted in early postoperative death due to hypothalamic dysfunction. Reviewing the reported cases of anterior basal encephalocele, a high correlation between transsphenoidal encephalocele, particularly in the pediatric age group, and allied malformations of the face, eye, and brain was disclosed. This characteristic malformation complex may be explained by a common pathogenetic mechanism operating in the embryonal period at about the stage of the anterior neuropore closure and occurring in the ventral surface of the cephalic end of the neural tube. Three cases of transsphenoidal encephalocele diagnosed in the neonatal period with progressive obstruction in the nasopharyngeal airway are also reported. A characteristic malformation complex consisting of median cleft face syndrome, optic nerve dysplasia, and agenesis of the corpus callosum was associated in two cases; the other patient had an extremely rare combination of septooptic dysplasia. Two patients died pre- and postoperatively, respectively; the other patient did not undergo operation because of grave multiple anomalies. (Neurosurgery 19:468-478, 1986) Copyright (C) by the Congress of Neurological Surgeons
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Sphenoethmoidal encephalocele: A case report with review of the literature
Article
  • Feb 1977
A rare case of sphenoethmoidal encephalocele with detailed pathological studies is reported. Only a few cases have been documented and were reviewed. The etiology and embryology of the congenital malformation remains speculative with an agreement on their development at an early embryonic stage. The mass presenting in the nasopharyngeal cavity remains often undetected unless causing respiratory embarrassment and is frequently misdiagnosed. Thorough radiological investigations detect the bone defect and outline the extent of the herniated mass and associated cerebral abnormalities. Small lesions have been successfully repaired. Larger ones prove less amenable to surgery and carry a high mortality.
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Frontoethmoidal meningoencephalocele: A common and severe congenital abnormality in South East Asia
Article
  • Jul 1992
  • Arch Dis Child
The clinical features of 23 cases of fronto-ethmoidal meningoencephalocele are described in two South East Asian populations. The clinical, embryological, and epidemiological evidence may support an early teratogenic insult in the aetiology of this congenital defect.
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Transfacial Surgical Treatment and Anthropometric Considerations of Frontoethmoidal Meningoencephaloceles
Article
  • Dec 1989
Meningoencephaloceles and meningoceles are projections of the meninges with or without cerebral tissue, respectively, through a cranial bony defect. Generally, meningoceles are soft and depressible, whereas meningoencephaloceles are solid and pulsatile. They can present through the calvarium or facial bones.
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Transethmoidal encephalomeningocele
Article
  • Jan 1986
  • SURG NEUROL
A newborn child with a basal meningoencephalocele of the transethmoidal type is reported with an intranasal obstruction and hypertelorism as the principal abnormalities. This disease has been reported in the literature with various facial and central nervous system deformities and was considered as a type of median cranioencephalic dysraphia. Precise diagnosis of this disease seems extremely important for classification and has a direct bearing on the prognosis.
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