Pediatrics and Neonatology (2014) 55, 404e406

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CASE REPORT

A Rare Presentation of a TransethmoidalEncephalocele at Birth

Anand Vishwanath Upasani, Dhiren Nanjibhai Patel*,Sudhir Bhisham Chandna

Department of Pediatric Surgery, Sheth V. S. General Hospital, Smt. NHL Municipal Medical College,Ahmedabad, Gujarat, India

Received Oct 17, 2011; received in revised form Jan 15, 2012; accepted Dec 3, 2012Available online 4 February 2013

Key Wordsnasal mass;neonate;transethmoidalencephalocele

* Corresponding author. F-42, TakVishweshwar Mahadev, Vastrapur, AhIndia.

E-mail address: drdhirenpatel70@y

1875-9572/$36 Copyright ª 2013, Taiwhttp://dx.doi.org/10.1016/j.pedneo.2

Transethmoidal encephalocele is a very rare condition. Herein, we report the case of a neo-nate with a transethmoidal encephalocele, who presented with an externally visible intranasalmass at birth. Clinical suspicion of intracranial extension was confirmed by radiological ima-ging. A bifrontal craniotomy was done to divide the narrow communicating duct. The masswas delivered through the nostril and duraplasty was completed. The postoperative recoverywas uneventful.Copyright ª 2013, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rightsreserved.

1. Introduction

Basal encephalocele has an incidence rate of one in every20,000e40,000 live births. It presents as a smooth masswithin the nasal cavity, the nasopharynx, or the pter-ygopalatine fossa.1e3 Transethmoidal encephalocele is asubtype of basal encephalocele. It is even rarer and con-tributes only 8% of all the cases of anteriorencephaloceles.4

shashila Apartments, Nearmedabad 380 015, Gujarat,

ahoo.co.in (D.N. Patel).

an Pediatric Association. Publish012.12.015

2. Case Report

A full-term vaginally delivered male child weighing 3 kgpresented at birth with a polypoidal mass protruding out ofthe left nostril (Figure 1). The child was born of a non-consanguineous marriage. The antenatal and obstetrichistory was uneventful. The head circumference at birthwas 34.5 cm and the anterior fontanelle was not tense.Skull, spine, and limb movements were normal. Examina-tion of the oral cavity and an ophthalmic evaluation wereboth unremarkable. There was no associated hypertelorismor cerebrospinal fluid (CSF) leak. The results of a braincomputed tomography (CT) scan revealed the presence of a3 � 3 � 2.5-cm soft tissue mass containing fluid components(Figure 2). The mass extended across the left cribriform

ed by Elsevier Taiwan LLC. All rights reserved.

Figure 1 Preoperative photograph showing a polypoidal softtissue mass protruding from the left nostril.

Figure 3 Histologic image showing stratified squamous epi-thelium of the nasal cavity (a), multiple blood vessels showingcongestion and proliferation (b), and glial tissue (c).

Transethmoidal encephalocele in a newborn 405

plate into the left nasal cavity and protruded out from theleft nostril. These findings were suggestive of a trans-ethmoidal meningoencephalocele. The newborn was sub-jected to a bifrontal craniotomy on the 5th day of life. Anarrow connecting duct was found extending up to the leftcribriform plate. No obvious bony defect was seen. Theconnecting duct was divided, dura repaired, and the massdelivered through the nostril. The wide and deformedleft nostril was left uncorrected for natural remodeling.Microscopic analysis revealed the presence of glial tissues

Figure 2 Computed tomography (sagittal view) showingextranasal mass (a), intranasal mass (b), connecting duct (c),cribriform plate (d), and frontal cortex (e).

with vascular congestion and proliferation (Figure 3). Thepostoperative recovery was uneventful. There was no CSFleak or meningitis, and the patient continues to do well 2years after the operation.

3. Discussion

Basal encephalocele is formed by herniation of meningesand brain matter through a defect in the anterior skullbase. It is a late neurulation defect that results fromdisturbance in the separation of surface ectoderm andneuroectoderm in the midline around the 4th week of ges-tation.5,6 There can be other associated midline anomaliessuch as hypertelorism, broad nasal root, cleft lip, and cleftpalate.7 A variety of terminologies and classificationshave been used to classify encephaloceles.8 Rahbar et alhave classified basal encephalocele into transethmoidal,sphenoethmoidal, trans-sphenoidal, and frontosphenoidaltypes.1 The transethmoidal type herniates through theregion of the cribriform plate.1,5,7 The incidence rate ofbony defects noted to be as high as 21%.5 However, no bonydefect was seen in this case. Intracranial or subarachnoidcommunication might not be demonstrated if the con-nection is lost during development.5

Although the age at presentation varies from earlyinfancy to adolescence, very few cases have been reportedin the neonatal period. Mahapatra and Agrawal4 havereported three neonates with anterior encephaloceles whopresented at birth, and Rahbar et al1 have reported a 2-day-old neonate with a nasal encephalocele. In addition,transethmoidal encephaloceles are commonly known topresent with recurrent meningitis, CSF rhinorrhea, or as anintranasal mass with respiratory distress.4,9 To the best ofour knowledge, this is the first reported case of a trans-ethmoidal encephalocele in a neonate with an uncompli-cated externally visible intranasal mass.

Intranasal mass can be confusedwith nasal polyps, leadingto disastrous consequences.9,10 Other differential diagnosesinclude a dermoid, a hemangioma, or a nasal glioma.1,5 The

406 A.V. Upasani et al

diagnosis of transethmoidal encephalocele can be confirmedby a CT (as in this case) or by magnetic resonance imag-ing.2,5,11 The preferred management is surgical excision asearly as possible to reduce the chances of infection anddeformity.1 Frontal craniotomy is done to resect the intra-cranial component and repair the dura, followed by excisionof the extracranial component using a suitable approach. Thetransnasal endoscopic approach in experienced hands pro-vides good exposure and reduces patient morbidity.1,5

Postoperative complications reported in other studieswere CSF leak, meningitis, convulsions, and epiphora.1,4

However, the postoperative recovery in this patient wasuneventful.

In conclusion, there is a need to increase awarenessabout the possibility of an intracranial extension of anovertly localized intranasal mass. In addition, every casepresenting as a congenital intranasal mass should be eval-uated in detail, including by suitable radiological imaging,before embarking on a surgical treatment or an invasiveintranasal procedure.

Conflicts of interest

The authors have no conflicts of interest relevant to thisarticle.

Acknowledgments

We sincerely thank Dr Pradip Rathod, Dr Kirti Prajapati, andDr Manas Dash for their help in proofreading andphotography.

References

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