Images in neonatal medicine
Encephalocele presenting as an oropharyngeal mass A female infant, born at 31-week gestation with birth weight 1.40 kg, required emergency intubation at birth for respiratory distress syndrome. Generalised convulsion was noted 30 min after delivery. Re-intubation on day 6 revealed a mass in the posterior oropharynx (figure 1). Direct laryngoscopy and rhinoscopy found a lesion measuring 15×20 mm between the right
medial and inferior nasal turbinate (figure 2) extending to the posterior oropharynx. MRI confirmed a basal encephalocoele comprising part of the medulla and cervical cord, and protruding through the osseous defect of the basiocciput. Subsequently, the infant required tracheostomy for upper airway obstruction, and she developed Staphococcus aureus meningitis presenting as nasal discharges, which was successfully treated with a 3-week course of cloxacillin. Basal encephalocoele, together with sincipital encephalocoele, are classified as the anterior type. It is more commonly found in Southeast Asian patients with an incidence 1–1.4/ 5000.1 The anatomical position of the defects renders such lesions impossible to detect on external inspection or easily overlooked during emergency procedures. Masses in the oropharynx could be due to polyp, mucocele, neoplasm or rarely encephalocoele. It is important not to perform needle aspiration or biopsy until the nature and the extent of the mass has been determined by radiological imaging. Neurological deficits are common and depend on the size and types of neural tissues enclosed in the sac.2 Treatment requires a multidisciplinary approach. Yennie L I Pang,1 Alice K Y Siu,2 Pak C Ng1 1
Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong 2 Department of Otolaryngology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong Correspondence to Professor Pak C Ng, Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, 6th Floor, Lui Che Woo Clinical Sciences Building, Shatin, N.T., Hong Kong; [email protected]
Figure 1 Direct laryngoscopy revealed an encephalocoele (En) at the posterior oropharynx. Ep, T and P represent the epiglottis, tongue and left pyriform sinus, respectively.
Contributors YLIP wrote the manuscript and obtained parents’ consent. AKYS provided the images. PCN revised the article and finally approved the version to be published. Funding Department of Paediatrics, The Chinese University of Hong Kong. Competing interests None. Parents’ consent obtained. Provenance and peer review Not commissioned; internally peer reviewed.
To cite Pang YLI, Siu AKY, Ng PC. Arch Dis Child Fetal Neonatal Ed 2015;100: F275. Received 10 October 2014 Revised 21 November 2014 Accepted 6 December 2014 Published Online First 24 December 2014 Arch Dis Child Fetal Neonatal Ed 2015;100:F275. doi:10.1136/archdischild-2014-307689
Figure 2 Direct rhinoscopy revealed an encephalocoele (En) covered by a thin layer of membrane in the right nostril. MT, IT and S represent the right middle turbinate, right inferior turbinate and nasal septum, respectively.
REFERENCES 1 2
Tomita T, Ogiwara H. Primary (congenital) encephalocele. In: Patterson MC, Weisman LE, eds. UpToDate. Waltham, MA (updated May 2013). Nazanin B, Farideh N. Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg 2009;45:461–6.
Pang YLI, et al. Arch Dis Child Fetal Neonatal Ed May 2015 Vol 100 No 3
F275
Copyright of Archives of Disease in Childhood -- Fetal & Neonatal Edition is the property of BMJ Publishing Group and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Encephalocele presenting as an oropharyngeal mass A female infant, born at 31-week gestation with birth weight 1.40 kg, required emergency intubation at birth for respiratory distress syndrome. Generalised convulsion was noted 30 min after delivery. Re-intubation on day 6 revealed a mass in the posterior oropharynx (figure 1). Direct laryngoscopy and rhinoscopy found a lesion measuring 15×20 mm between the right
medial and inferior nasal turbinate (figure 2) extending to the posterior oropharynx. MRI confirmed a basal encephalocoele comprising part of the medulla and cervical cord, and protruding through the osseous defect of the basiocciput. Subsequently, the infant required tracheostomy for upper airway obstruction, and she developed Staphococcus aureus meningitis presenting as nasal discharges, which was successfully treated with a 3-week course of cloxacillin. Basal encephalocoele, together with sincipital encephalocoele, are classified as the anterior type. It is more commonly found in Southeast Asian patients with an incidence 1–1.4/ 5000.1 The anatomical position of the defects renders such lesions impossible to detect on external inspection or easily overlooked during emergency procedures. Masses in the oropharynx could be due to polyp, mucocele, neoplasm or rarely encephalocoele. It is important not to perform needle aspiration or biopsy until the nature and the extent of the mass has been determined by radiological imaging. Neurological deficits are common and depend on the size and types of neural tissues enclosed in the sac.2 Treatment requires a multidisciplinary approach. Yennie L I Pang,1 Alice K Y Siu,2 Pak C Ng1 1
Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong 2 Department of Otolaryngology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong Correspondence to Professor Pak C Ng, Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, 6th Floor, Lui Che Woo Clinical Sciences Building, Shatin, N.T., Hong Kong; [email protected]
Figure 1 Direct laryngoscopy revealed an encephalocoele (En) at the posterior oropharynx. Ep, T and P represent the epiglottis, tongue and left pyriform sinus, respectively.
Contributors YLIP wrote the manuscript and obtained parents’ consent. AKYS provided the images. PCN revised the article and finally approved the version to be published. Funding Department of Paediatrics, The Chinese University of Hong Kong. Competing interests None. Parents’ consent obtained. Provenance and peer review Not commissioned; internally peer reviewed.
To cite Pang YLI, Siu AKY, Ng PC. Arch Dis Child Fetal Neonatal Ed 2015;100: F275. Received 10 October 2014 Revised 21 November 2014 Accepted 6 December 2014 Published Online First 24 December 2014 Arch Dis Child Fetal Neonatal Ed 2015;100:F275. doi:10.1136/archdischild-2014-307689
Figure 2 Direct rhinoscopy revealed an encephalocoele (En) covered by a thin layer of membrane in the right nostril. MT, IT and S represent the right middle turbinate, right inferior turbinate and nasal septum, respectively.
REFERENCES 1 2
Tomita T, Ogiwara H. Primary (congenital) encephalocele. In: Patterson MC, Weisman LE, eds. UpToDate. Waltham, MA (updated May 2013). Nazanin B, Farideh N. Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg 2009;45:461–6.
Pang YLI, et al. Arch Dis Child Fetal Neonatal Ed May 2015 Vol 100 No 3
F275
Copyright of Archives of Disease in Childhood -- Fetal & Neonatal Edition is the property of BMJ Publishing Group and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
