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Abstract

Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.
 
Case Reports



     



Encephalocele presenting as lower lid
swelling: A rare case report
Vaibhav Kumar Jain, Vikas Kanaujia,
Priyadarshini Mishra, Kumudini Sharma


   

         
       
      
      
       
      

Key words: 

    


    





    
    

       
    
  

Case Report
     


  

     
    
     
     



    
    
   


   
  
    







Cite this article as: Jain VK, Kanaujia V, Mishra P, Sharma K. Encephalocele
presenting as lower lid swelling: A rare case report. Indian J Ophthalmol
2018;66:453-4.
This is an open access article distributed under the terms of the Creative
Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work non‑commercially, as long as the
author is credited and the new creations are licensed under the identical terms.
For reprints contact: reprints@medknow.com
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DOI:
10.4103/ijo.IJO_894_17
PMID:
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        


       
et al.  
    


      





 


     


[Downloaded free from http://www.ijo.in on Monday, February 26, 2018, IP: 47.8.126.95]
  

    

Discussion



    
     
  
    

  
    
     
    
     
       
     

 A


 
Conclusion
    


     

   



Declaration of patient consent
     
    



       

Financial support and sponsorship

Conicts of interest

References
 
 

     

    


 


 

 

    

        


Figure 1: Case description of anterior encephalocele. (a) Clinical
picture showing the right eye lower lid swelling. (b) Computed
tomography head and orbit (coronal view) showing anterior fossa
skull base defect along the cribriform plate on the right side along
with herniation of intracranial soft tissue into the ethmoid air sinuses
and medial aspect of the right orbit through a medial orbital wall
defect (black arrow). (c) Postoperative photo showing the resolution
of lower lid swelling
b
c
a
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... Encephalocele clinical manifestations can vary from mild to severe headaches, CSF rhinorrhea, meningitis, ataxia, and other severe neurological symptoms. Situations such as allergic rhinitis, nasal polyps, migraines, or benign lid swelling may predispose to the misdiagnosis of the encephalocele [17,[29][30][31] . Spontaneous CSF rhinorrhea and meningitis represent the most crucial signs that can bring physicians closer to the diagnosis of encephalocele [32] . ...
Article
Introduction and importance Spontaneous cerebrospinal fluid (CSF) rhinorrhea without a history of head trauma is rare. The authors describe a scarce case of spontaneous, nontraumatic, delayed CSF rhinorrhea due to ethmoidal encephalocele associated with frontoparietal giant meningioma. Case presentation A 49-year-old male complained of a slowly growing skull mass lasting 10 years since a blunt head trauma and progressive hemiparesis 3 months ago and was referred to our center. There was a heterogeneous extra-axial mass (77×77×70 mm) with calcified components at his right frontal lobe, demonstrating notable adjacent parenchymal edema and a midline shift to the left. There was a sizeable extra-axial mass (113×95×80 mm) in his right front-temporoparietal lobe associated with adjacent invasion to calvarium leading to the lytic and sclerotic appearance of the calvarial bone showing intense heterogeneous contrast enhancement and an adjacent enhancing dural tail. A considerable mass effect on the adjacent frontotemporal cortex is evident, with mild parenchymal edema leading to a notable midline shift to the left, right uncal herniation, and left ventriculomegaly. On postoperation day 5, the patient developed a recurrent CSF leak exacerbated by hydrocephalus. He was rescheduled to undergo endoscopic endonasal surgery and dural repair on postoperative day 7. The postoperative course was uneventful. Clinical discussion Frontoparietal lobe encephaloceles represent the least common cause of spontaneous CSF rhinorrhea. Early diagnosis and surgical management remain crucial to minimize the subsequent complications. Conclusion The ventricular anatomy and CSF fluid dynamics alteration following tumor surgical resection seem to contribute to an environment by which a herniated ethmoidal encephalocele developed a delayed nontraumatic CSF leak.
... Clinical manifestations of encephalocele can include mild to severe headaches, neurological symptoms, ataxia, meningitis, CSF rhinorrhea, etc. There is also a possibility of misdiagnosis of the encephalocele symptoms with situations such as allergic rhinitis, nasal polyps, migraines, or benign lid swelling [13][14][15][16] . The most important signs that can bring physicians closer to the diagnosis of encephalocele are spontaneous CSF rhinorrhea and meningitis. ...
Article
Full-text available
Encephalocele is a protrusion of the intracranial contents through a cranium defect. Encephalocele is divided into primary (congenital) and secondary (acquired) classes. From an epidemiological point of view, primary encephalocele is much more prevalent than secondary cases. Furthermore, among the secondary causes of this condition, iatrogenic encephalocele is recognized as a rare phenomenon. In this case report, we introduce a case of secondary encephalocele in a 30-year-old pregnant female who came to our emergency center at 5 weeks of gestation with a vague headache in her forehead and a runny nose. she reported a history of nasal polypectomy 9 months ago and a 10-day hospitalization for meningitis 5 months prior to admission. MRI of the patient's brain showed evidence of cerebral parenchymal herniation to the right nasal cavity, which was suggestive of encephalocele. She was scheduled for endoscopic transnasal reconstruction, and during the operation, a significant right-sided posterior ethmoidal roof defect with CSF leak and encephalocele was revealed. Eventually, the skull defect was successfully repaired with a vascularized flap, and the patient was discharged in good general condition.
... Abnormal protrusion of a part of the brain and its meninges through a defect in the skull bones is termed meningoencephalocele. A case of an anterior meningo-encephalocele presenting as a small swelling in the right lower eyelid was reported; if it was mistreated as a lachrymal swelling it might result in meningitis and cerebrospinal fluid leakage [6]. The nasal dermoid cyst and median nasal sinus are rare congenital anomalies that are attributed to early embryonic inclusion of the ectodermal germ layer during closure of the anterior neuropore of the neural tube [7]. ...
Article
Full-text available
Anterior encephaloceles are rare conditions. Except for a few places from South East Asia, no large series has been published in the World literature. At AIIMS, we have managed 133 cases over a 40-year-period from 1971 to 2010. Frontoethmoidal type was the most frequent, noticed in 104 patients, followed by nasopharyngeal nasal in 12 and orbital encephaloceles in 6 patients. Ten patients were adults over the age of 18 years and 15 patients were between 5 and 18 years of age. Swelling over the nose was reported in all 104 patients with frontoethmoid type. In nasopharyngeal type, patients presented with respiratory problem. Patients with orbital mass had proptosis, on the side of encephalocele. Computed tomography (CT)/Magnetic resonance imaging (MRI) was performed in 127 patients, which was able to delineate the bone defect and associated brain anomalies. All the patients were subjected to repair of encephalocele. Patients with hypertelorism required orbital osteotomies and correction of deformity. There were four deaths, all prior to 2000. No death was encountered in the last 10 years. CSF leak was the commonest postoperative complication, noticed in 24 patients. Overall cosmetic outcome was good.
Article
Full-text available
Anterior encephalocele is a rare condition, and only a few large series have been published in the literature. Surprisingly, the incidence is much higher in Southeast Asian countries, including some parts of India. While the reported incidence in the West is between 1:35,000 and 1:40,000 live births, it is as high as 1:5,000 live births in Thailand. We present a series comprising 92 cases of anterior encephaloceles treated over a 30-year period (1971-2000). Frontoethmoidal encephaloceles are the commonest type, followed by the nasopharyngeal and orbital type. Among the frontoethmoidal encephaloceles, nasoethmoid is the commonest type, and these patients present with swelling over the bridge of the nose with significant hypertelorism and orbital deformities. The nasopharyngeal type remains occult and presents with nasal obstruction or CSF rhinorrhea. Rarely, the patient may present with meningitis. Since 1978, computed tomography (CT) scans have regularly been performed in our patients. CT scans delineate the skull defect and associated brain anomalies. There was associated hydrocephalus present in 12 patients and agenesis of the corpus callosum in 5 patients. In all patients, one-stage repair of the encephalocele and correction of bony anomalies by appropriate osteotomy was undertaken. Since 1988, in cases of frontoethmoidal encephalocele with significant hypertelorism, medial advancement of the medial half of the orbits on either sides was carried out, instead of a classical Tessier's operation. Postoperative morbidity included CSF leak in 20 patients, wound infection in 2 and chest infection in 3. There were 3 deaths in our study. The overall cosmetic outcome was good.
Article
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.
Article
ENCEPHALOCELES are lesions that usually cause signs and symptoms that are misleading.1 The cranial contents may herniate through one of the natural openings, such as the optic foramen or the sphenoid fissure, or through an embryonic defect in the bony orbit.2 Anteriorly, a transethmoidal defect may occur through the lamina cribrosa and present as an orbital tumor. This report describes a case of an ethmoid encephalocele presenting as an orbital tumor. Report of a Case This 62-year-old white woman was in good health until three weeks prior to admission, when a tender swelling developed rather suddenly in the superior nasal aspect of her right orbit (Fig 1). On the previous day, she had been mowing her lawn and had gotten grass in her nose causing her to sneeze and have hayfever like symptoms. Because of her past history of sinus trouble, she was considered to have acute ethmoiditis
Article
Summary A case report of fronto-ethmoidal meningoencephalocele is described.
Article
Anterior encephalocele is a rarely reported CNS malformation with a geographical predilection for South-East Asia. The paucity of literature prompted us to analyse our results with hemiorbital advancement and classical Tessier's operation in 103 cases over 32 years (1971-2002). In our series, the frontoethmoidal subtype was the most common (80%), followed by the orbital (8%), transethmoidal (8%), transsellar (3%) and interfrontal types (1%). All patients with classical frontoethmoidal encephalocele had swelling over the nose or inner canthus since birth, with varying degrees of hypertelorism; and those with the nasopharyngeal subtype commonly presented with nasal obstruction and cerebrospinal fluid (CSF) rhinorrhoea. Neurofibromatosis was present in seven patients, all of whom had an orbital encephalocele. The diagnostic workup included a computerised tomography scan of the head in 96 patients and magnetic resonance imaging of the brain in 16 patients. The associated findings on imaging were hydrocephalus (15%), corpus callosum agenesis (7%), arachnoid cyst (3%), porencephalic cyst (3%) and single ventricle (3%). A classical Tessier's operation was performed in the initial 30 cases. However, since 1988, we have been performing a single stage hemiorbital advancement and repair of the encephalocele. There were three deaths, one due to pneumonia and two due to fulminant meningitis. Twenty-two patients (22%) had postoperative CSF leak, of which five required lumboperitoneal shunt placement. We believe that hemiorbital advancement offers satisfactory results with less morbidity than the Tessier's operation.
Article
An 11-day-old infant was referred for a large, fleshy swelling at the right medial canthus, which was noticed at birth. CT of the skull showed protrusion of brain tissue within the mass, from which a diagnosis of meningoencephalocele was made. The mass was subsequently resected by a team of physicians represented by ophthalmology, otolaryngology, and neurosurgery. Follow-up showed good cosmetic and functional results. Meningoencephaloceles usually need prompt attention to ensure a good visual and cosmetic result. Imaging before surgery and pathologic analysis are both necessary steps to successful management. Prognosis is excellent unless associated with other comorbid conditions such as Dandy-Walker syndrome, holoprosencephaly, or agenesis of the corpus callosum.