Risk factors, presentations, associated anomalies, and outcomes of patients with encephalocele

Authors

  • Nawzhin Jange Jalyzada MBChB, KHCMS (Neurosurgery) trainee. Iraq, Kurdistan Region, Sulaimani City, Shar Hospital, Department of Neurosurgery.
  • Ari Sami Hussain Nadhim MBChB, FRCS, FIBMS, Consultant Neurosurgeon. Iraq, Kurdistan Region, Sulaimani City, Shar Hospital, Department of Neurosurgery.
  • Nabaz Muhamad Mustafa MBChB, FIBMS (Neurosurgery). Iraq, Kurdistan Region, Sulaimani City, Shar Hospital, Department of Neurosurgery.

DOI:

https://doi.org/10.56056/amj.2019.91

Keywords:

Congenital brain anomaly, Congenital diseases, Encephalocele, Folic acid, Hydrocephalus, Microcephalus, Neural tube defect

Abstract

Background and objectives: Encephalocele is a rare neural tube birth defect. Aim of this study was to find the risk factors and associated anomalies of patients with encephalocele, and its outcomes.

Methods: We used a case-series study for 30 patients with encephalocele who were admitted to Shahid Dr. Aso Hospital from January 2017 to July 2018. The patients’ clinical features and computed tomography findings were recorded and their outcomes classified into death, cerebral palsy, delayed milestones, and good.

Results: The genders of the patients were 11 (36.7%) male and 19 (63.3%) female with a female: male ratio of 1.7:1. There were significant associations between the site of the skull defect with microcephalus, seizure, and outcome and statistically significant association between the content of the sac and family history. The content of the sac was mostly mixed brain and cerebrospinal fluid (56.7%). The size of the sac was significantly associated with family history and hydrocephalus and all of encephaloceles were located in the midline mostly at occipital and occipito-cervical region. The outcomes were death (10%), poor (10%), delayed milestones (23.3%), and 56.7% good. There was also statistically significant association between outcome and other brain abnormality and microcephalus.

Conclusions: Encephalocele was located in midline mostly at occipito-cervical region, the size of its sac is associated with hydrocephalus, the content was mixed brain and CSF, and its site was associated with seizure. Moreover, content and size of the sac was significantly associated with family history of neural tube defect.

Downloads

Download data is not yet available.

References

Yucetas SC, Uçler N. A Retrospective Analysis of Neonatal Encephalocele Predisposing Factors and Outcomes. Pediatr Neurosurg 2017; 52:73-6.

Inan C, Sayin NC, Gurkan H, et al. A large posterior encephalocele associated with severe ventriculomegaly, cerebellar atrophy and transposition of the great arteries. J Clin Ultrasound 2018;46(9):588-90.

Jeyaraj P. Management of the frontoethmoidal encephalomenin- gocele. Ann Maxillofac Surg 2018; 8:56-60.

Arifin M, Suryaningtyas W, Bajamal AH. Frontoethmoidal encephalocele: clinical presentation, diagnosis, treatment, and complications in 400 cases. Childs Nerv Syst. 2018; 34(6):1161-8.

Bannout F, Harder S, Lee M, et al. Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection? Brain Sci. 2018; 8(3):42.

Baro V, D’Errico I, d’Avella D, Denaro L. Transethmoidal Encephalocele and High Intracranial Pressure. Pediatr Neurosurg. 2018; 53(4):286-7.

Shi C, Flores B, Fisher S, Barnett SL. Symptomatic Parietal Intradiplo- ic Encephalocele—A Case Report and Literature Review. J Neurol Surg Rep. 2017; 78:e40–e45.

Satyarthee GD, Moscote-Salazar LR, Escobar-Hernandez N, et al. A Giant Occipital Encephalocele in Neonate with Spontaneous Hemorrhage into the Encephalocele Sac: Surgical Management. J Pediatr Neurosci. 2017; 12(3):268–70.

Yhoshu E, Dash V, Bawa M. Double encephalocele: An unusual presentation. J Pediatr Neurosci 2018; 13:264-6.

Alshamrani A, Habalrih F, Altweijri I, Alsaleh S, Ajlan A. Endoscop- ic trans-nasal repair of basal encephalocele associated with morning glory syndrome. Br J Neurosurg. 2018; 10:1-3. Available from: https://www.tandfonline.com/doi/full/10.1080/02688697.2018.1494264.

Horcajadas A, Palma A, Khalon BM. Frontoethmoidal encephalocele. Report of a case. Neurocirugia (Astur). Forthcoming 2018;30. Available from: http://dx.doi.org/10.1016/j.neucir.2018.02.006.

Cortazar AZ, Martinez CM, Feliubadalo CD, Cueto MR, Serra L. Magnetic resonance imaging in the prenatal diagnosis of neural tube defects. Insights Imaging. 2013; 4(2):225–37.

Richardson S, Khandeparker R, Raghuvaram A, Mohan R. Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: a case report. J Korean Assoc Oral Maxillofac Surg. 2018; 44(2):86-90.

Gandhoke G, Goldschmidt E, Kellogg R, Greene S. Encephalocele development from a congenital meningocele: case report. J Neurosurg Pediatr. 2017; 20(5):419-22.

Lau W, Yung W, Leung W, et al. A case of “familial atretic encephalocele”. Ultrasound Obstet Gynecol. 2018; 27. doi: 10.1002/uog.20109.

Bear K, Solomon B, Roessler E, et al. Evidence for SHH as a candidate gene for encephalocele. Clin Dysmorphol. 2012; 21(3):148–51.

Greene ND, Copp AJ. Neural Tube Defects. Annu Rev Neurosci. 2014;37: 221–42.

Raeiq A. Posterior Fontanelle Encephalomeningocele in a Neonate: A Case Report. Cureus. 2018;10(3): e2315.

Wang ZI, McBride A, Grinenko O, et al. Utility of CISS sequence in detecting anteroinferior temporal encephalocele. J Neurol Sci. 2017; 381:59–61.

Rehman L, Farooq G, Bukhari I. Neurosurgical interventions for occipital encephalocele. Asian J Neurosurg. 2018; 13:233-7.

Downloads

Published

2023-06-13

How to Cite

Jalyzada, N. J. ., Nadhim, A. S. H. ., & Mustafa, N. M. . (2023). Risk factors, presentations, associated anomalies, and outcomes of patients with encephalocele. AMJ (Advanced Medical Journal) , 5(2), 1-7. https://doi.org/10.56056/amj.2019.91

Issue

Section

Articles