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Case 188

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Female child, 5 years old, was hospitalized for 15 days due to pneumococcal meningitis, during which reported hearing loss on the left. Is diagnosed with asthma and allergic rhinitis with important nasal discharge on the right. Makes constant use of nasal corticosteroids, with little response. Born at term, appropriate weight for the gestational age, vaccination up to date. Prior history of hospitalization for community-acquired pneumonia at age two, and two episodes of meningitis at 14 months and 2 years of age, without sequelae. The mother also reported frequent episodes of bacterial sinusitis and denies previous surgery, history of trauma or other comorbidities.

Analyzing the images and clinical data presented, what is the most likely diagnosis?

a) Nasal Glioma

25%

b) Meningoencephalocele

25%

c) Dermoid Cyst

25%

d) Ossifying Fibroma

25%
   

Image analysis

Image 1: Detail of magnetic resonance imaging (MRI), coronal view, T2-weighted, revealing brain mass in the ethmoid region (red circle).

 

Image 2: Detail of magnetic resonance imaging (MRI), sagittal view, T2-weighted, showing image component (arrow) with signal similar to that of the cerebrospinal fluid (compare with the subarachnoid space and the lateral ventricle), inferring the presence of an intracranial communication.

Highlights

- Meningoencephalocele is a herniation of the brain and of the meningeal content through a bone defect, which can be of minimal or of great extent.

- Frequent meningitis associated with unilateral rhinorrhea (liquid clear as "rock water") may be the only signs of a skull base defect with fistula.

- Imaging studies are important for the investigation of meningoencephalocele: to assess the origin of the lesion, the extent of the bone defect, intracranial connection and the contents of the hernial sac.

- Computed tomography is effective for detecting the extent of the cranial defects, while the MRI evaluates the extent of the neural hernia. If they are inconclusive and the suspicion of cerebrospinal fluid leak is still strong, proceed with cisternography.

- Do not biopsy a nasal injury without imaging due to the risk of intracranial communication and the consequent risk of cerebrospinal fluid leakage.

- After the establishment of the diagnosis the patient should receive immunization against pneumococcus and haemophilus.

- The treatment of meningoencephalocele is surgical.

References

- Alves, Adriana L. et al. Displasia fibrosa: relato de três casos. Rev. Bras. Otorrinolaringol., São Paulo, v. 68, n. 2, Mar. 2002Dolhnikoff, M. et al. Meningites de repetição por malformação de base de crânio com fístula. Rev Paul Pediatria 2005; 23(3); 154-7

- Fanny E. Morón, Michael C. Morriss, Jeremy J. Jones, et al; Lumps and Bumps on the Head in Children: Use of CT and MR Imaging in Solving the Clinical Diagnostic Dilemma. RadioGraphics, 2004; 24:6, 1655-1674.

- Giannetti A.V.Fístula liquórica espontânea primária da base anterior do crânio: aspectos clínicos e fisiopatológicos. Belo Horizonte. Tese [Doutorado em Medicina, Ciências Aplicadas à Cirurgia e a Oftalmologia] – Universidade Federal de Minas Gerais; 2009.

- Granato L, Souza Neto O, Destailleur D, Padula F, Lessa
R M, Meningoencefalocele volumosa intranasal. Braz J
Otorhinolaryngol, 2000, 66 (1), 72-6.

- Manual de otorrinolaringologia e cirurgia de cabeça e pescoço / coordenadores Fernando Freitas Ganança, Paulo Pontes. –Barueri : Manole, 2011.

Authors

Ana Júlia Furbino Dias Bicalho, sixth year medical student at UFMG.

E-mail: anajuliabicalho[at]gmail.com

 

Kellen Alete Souza, fifth year medical student at UFMG.

E- mail: kesouzamed[at]gmail.com

 

Matheus Soares Buissa, fifth year medical student at UFMG.

E-mail: matheussbuissa[at]gmail.com

 

Lucas Tomazelli de Souza Ferreira, fifth year medical student at UFMG.

E-mail: lucastomazelli[at]hotmail.com

Supervisors

Helena Becker, professor of the Otorhinolaringology Department of UFMG’s School of Medicine.

E-mail:  helenabecker.becker[at]gmail.com

 

Fernanda Moura Teatini, neuroradiologist at UFMG’s Hospital das Clínicas.

E-mail: fernandateatini[at]live.com

Acknowledgements

To Dr Nelson José Vieira, Radiologist and Professor of the Department of Anatomy and Imaging of UFMG’s School of Medicine, for his willingness and attention.

Reviewers

André Guimarães, Letícia Horta, Bárbara Queiroz and professor Viviane Parisotto.

Translated by

Bárbara de Queiroz e Bragaglia, 5th year medical student at UFMG.

E-mail: barbara.bragaglia[at]gmail.com

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