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Nasal Encephalocele – a Rare Case Report NP Ansari* , MR Hasibur** , MH Siddiqui¶

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Nasal Encephalocele – a Rare Case Report NP Ansari* , MR Hasibur** , MH Siddiqui¶ Journal of Pakistan Association of Dermatologists 2012; 22 (4):373-375. Case Report Nasal encephalocele – a rare case report NP Ansari* , MR Hasibur** , MH Siddiqui¶ *Department of Pathology, Community Based Medical College, Bangladesh **Department of Dermatology, Community Based Medical College, Bangladesh ¶Department of Ophthalmology, Community Based Medical College, Bangladesh Abstract Encephalocele is the herniation of the cranial contents through a bone defect in the skull. Nasal encephalocele is the herniation of cranial content in the nasal area. It is a congenital abnormality. It is one of the cause s of craniospinal dysraphism , u sually present at birth with symptom of obstruction or other complication s. It present s as an external swelling on the nose. Clinically , there can be many differential diagnos es. An accurate diagnosis can reduce life threating intracranial complication s and permit better management. For proper management , a multidiciplinary approach is needed. Approximately 80% of encephalocele exit the cranial cavity in the region of the occiput. Extraoccipital encephalocoele may protrude through defects in other portions of the cranial vault. We present a case of encephalocele in a 6-month -old girl who presented with a congenital swelling on the left lateral side of the nose with complaints of watering from the eye of same side. Key words Nasal encephalocele. Introduction any of these locations must be carefully evaluated for the evidence of extension into the A nasofrontal mass is a rare anomaly of infan cy cranial cavity. Result s of neuroimaging stud ies and may cause serious complication s. Incidence are often decisive in the classification of a given of nasal encephalocele is 1 in 5000 live births .1 lesion because both the heterotropia and true An accurate diagnosis permits proper encephalocele often consist solely of aggregatd management and prevent s potentially life astroglia embedded in fibrous tissue. We report a thretening intracranial complication. A diverse case of nasal encephalocele and discuss the group of condition s is observe d in the clinical presentation, diagnosis and management developmental anomalies of the nose, these of such a case. include nasal dermoid, gliomas, encephalocoele, nasal clefts, proboscis lateralis, arrhinia, Case report polyrrhinia, nasopharyngeal teratoma and epignathus .2 Encephaloceles and glial A 6-month -old girl admitted to the department heterotropias (commonly called nasal gliomas) of ophthalmology with the complaints of a are related malformational tumour -like swelling over the left side of the nose and conditions usually affecting newborn and older watering from the eye of the same side. The infants .3 Prior to biopsy or resection, a swelling had been present since birth. The baby congenital subcutaneous or submucosal mass in was born at full term and had a normal vaginal Address for correspondence delivery. She wa s the first child of her parents. Professor MR Hasibur Department of Dermatology, Community Based On physical examination, there was a Medical College, Bangladesh subcutaneous annular swelling on the left lateral E-mail: [email protected] Mobile : +88 01727428441 side of nose with watering from the eye. The 373 Journal of Pakistan Association of Dermatologists 2012; 22 (4):373-375. On g ross examination , the specimen consisted of a soft cyst like structure measuring 2x2x1.5cm. The cut section showed a tiny empty cavity and wall grey white in appearance. Two blocks were embedded. Microscopic examination show ed a mass of mature astrocytes along with areas of fibrosis. No distinct anaplastic features were seen. It was compatible with nasal encephalocele . Figure 1 Photograph of the patient showing left Discussion lateral nasal mass. Nasal encephalocele are typically identified in association with a discernible cranial bone defect. Encephalocele s are classified as occipital when found at or posterior to the lambdoid suture, parietal when found between the lambdoid and the coronal suture and anterior when they occur anterior to the coronal suture. Occipital encephalocele s are the commonest type in the western Europe, while anterior ones Figure 2 Microscopic examination revealed presence are more frequent in certain estern countries .4 of glial tissue with a fibrillary background. Extraoccipital encephaloceles are more commonly of sincipital or basilar types .5 mass was superficial on the left lateral side of Sincipital encephaloceles present as visible the nose and measuring 3.5x2 cm. It wa s firm in facial swelling in the region of the forehead, consistency. The mass increased in size during nasal bridge, or orbit, whereas basal variants are crying or coughing. The baby had no other situated in the nasal cavity, sphenoid sinus, physical and mental abnormalities. nasopharynx, or pterygopalatine fossa and therefore are not externally evident .5 Nasal Computerized tomography showed there was encephalocele s are anterior encephaloceles skull defect at floor of the anterior cranial fossa where meningeal herniation occurs through a at cribiform plate of the ethmoid on left side midline defect in the floor of the anterior cranial lateral to nasal septum. The herniated fossa. During the intrauterine life, due to some intracranial contents through this defect form ed unknown etiological factor inappropiate closure a soft tissue mass at left side of the root of the of the anterior neuropore occur s which causes an nose. outpouching of the meninges and the neural tissue.6 The case was referred to the neurosurgery department where she was operated with The common clinical differential diagnos es excision and repair. The tissue was then sent for include nasal glioma, nasal dermoid, histopathological examination. hemangioma and lipoma. Nasal glioma is a 374 Journal of Pakistan Association of Dermatologists 2012; 22 (4):373-375. congenital benign tumour like condition of the brain must be done to confirm composed of heterotropic neural tissue within encephalocele before an invasive procedure to the nasal region. It has no connection with the avoid intracranial complications. Surgical cranial cavity. Nasal dermoid show s epithelial excision and repair is the treatment of choice. lined cavities or sinus tracts with variable number of skin appendages. Hemangioma s are References the most frequent vascular tumour in infancy.7,8,9 1. Turgect M, Ozean OE, Benti K, Ozen T. Nasal encephalocele usually present s with Congenital nasal encephalocele: a review of 35 cases. J Craniomaxillofac Surg. external swelling on the nose since birth. 1995 ;23 :1-5. Symptom s usually present with obstruction or 2. Chang KC, Leu YS . Nasal glioma:A case rhinorhea. Swelling is usually soft and skin over report . Ear Nose Throat J . 2001;80 :410 -1. 3. Fletcher CDM,Carpenter G,McKee PH. the swelling is normal. The swelling increase s in Nasal glioma. A rarity . Am J Dermatopathol. size on coughing and straining. 1986 ;8:341 -6. 4. Mood GF. Congenital anterior herniations of brain. Ann Oto Rhinol Laryn . 1938 ;47 ;391 - When a midline nasal swelling is present, no 401. invasive procedure nor surgery is performed 5. Rosai J, Ackermans LV, eds. Sugical until a n intracranial connection has been Pathology, 10th ed n. New York: Elsvier; 2011. excluded using CT scaning or MRI, due to the 6. Ramchandra CR, Phelps PD. Nasal risk of meningitis or cerebrospinal fluid leakage. encephaloceles associated with unilateral Computer tomography is good for delineating absence of cochlea. J Laryngol Otol. 19 77; 91 :813 -7. bony abnormalities but not for detecting an 7. Levine MR, Kellis A, Lash R. Nasal glioma intracranial component. Magnetic resonance masquerading as a capillary hemangioma. imaging is better for delineating soft tissue Ophthal Plast Reconstr Surg. 1993;9:132 -4. 8. Hoeger PH, Schaefer H, Ussmueller J, details, including any intracranial connection. It Helmke K . Nasal glioma presenting as also minimises the level of exposure to ionising capillary hemangioma. Eur J Pediatr radiation.10 . 2001;160 :84 -7. 9. Dasgupta NR, Bentj ML. Nasal glioma; identification and differentiation from Conclusion hemangiomas. J Craniofac Surg . 2003;14 :736 -8. A nasofrontal mass is a rare anomaly and may 10. Verney Y, Zanolla G, Teixeira R, Oliveira LC. Midline nasal mass in infancy:a nasal cause serious intracranial complications. It glioma case report. Eur J Pediatr Surg. should be carefully examined. CT scan or MRI 2001;11 :324 -7. 375 .
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