Clinical Research
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Year 2024, Volume: 1 Issue: 2, 124 - 130, 14.06.2024

Abstract

References

  • Sadler TW. Langman's Medical Embryology, Lippincott Williams & Wilkins, Philadelphia 1990. p.352.
  • Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA. Cephaloceles and related malformations. AJNR Am J Neuroradiol. 1992;13(2):655-690.
  • Lo BW, Kulkarni AV, Rutka JT, et al. Clinical predictors of developmental outcome in patients with cephaloceles. J Neurosurg Pediatr. 2008;2(4):254-257. doi:10.3171/PED.2008.2.10.254
  • Mai CT, Isenburg JL, Canfield MA, et al. National population-based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019;111(18):1420-1435. doi:10.1002/bdr2.1589
  • Siffel C, Wong LY, Olney RS, Correa A. Survival of infants diagnosed with encephalocele in Atlanta, 1979-98. Paediatr Perinat Epidemiol. 2003;17(1):40-48. doi:10.1046/j.1365-3016.2003.00471.x
  • Parker SE, Mai CT, Canfield MA, et al. Updated National Birth Prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Res A Clin Mol Teratol. 2010;88(12):1008-1016. doi:10.1002/bdra.20735
  • Czeizel AE, Dudás I. Prevention of the first occurrence of neural-tube defects by periconceptional vitamin supplementation. N Engl J Med. 1992;327(26):1832-1835. doi:10.1056/NEJM199212243272602
  • Tirumandas M, Sharma A, Gbenimacho I, et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29(5):739-744. doi:10.1007/s00381-012-1998-z
  • Stoll C, Alembik Y, Dott B. Associated malformations in cases with neural tube defects. Genet Couns. 2007;18(2):209-215.
  • Chen CP, Chern SR, Wang W. Rapid determination of zygosity and common aneuploidies from amniotic fluid cells using quantitative fluorescent polymerase chain reaction following genetic amniocentesis in multiple pregnancies. Hum Reprod. 2000;15(4):929-934. doi:10.1093/humrep/15.4.929
  • Anesen D, Rosman AK, Kandasamy R. Giant Occipital Encephalocele with Chiari Malformation Type 3. J Neurosci Rural Pract. 2018;9(4):619-621. doi:10.4103/jnrp.jnrp_103_18
  • Yucetas SC, Uçler N. A Retrospective Analysis of Neonatal Encephalocele Predisposing Factors and Outcomes. Pediatr Neurosurg. 2017;52(2):73-76. doi:10.1159/000452805
  • Joó JG, Beke A, Papp C, et al. Neural tube defects in the sample of genetic counselling. Prenat Diagn. 2007;27(10):912-921. doi:10.1002/pd.1801
  • Society for Maternal-Fetal Medicine, Monteagudo A. Posterior Encephalocele. Am J Obstet Gynecol. 2020;223(6):B9-B12. doi:10.1016/j.ajog.2020.08.177
  • Tomita T, Ogiwara H. Primary (congenital) encephalocele. In: Post TW, ed. UpToDate. UpToDate; 2013. Accessed December 10, 2023. https://www.uptodate.com
  • Tsuchida T, Okada K, Ueki K. The prognosis of encephaloceles (author's transl). No Shinkei Geka. 1981;9(2):143-150.
  • Kankam SB, Nejat A, Tavallaii A, Tayebi Meybodi K, Habibi Z, Nejat F. Hydrocephalus in patients with encephalocele: introduction of a scoring system for estimating the likelihood of hydrocephalus based on an 11-year experience from a tertiary center. J Neurosurg Pediatr. 2023;31(4):298-305. doi:10.3171/2022.12.PEDS22475
  • Protzenko T, Dos Santos Gomes Junior SC, Bellas A, Salomão JFM. Hydrocephalus and occipital encephaloceles: presentation of a series and review of the literature. Childs Nerv Syst. 2021;37(11):3437-3445. doi:10.1007/s00381-021-05312-7
  • Nagy MR, Saleh AE. Hydrocephalus associated with occipital encephalocele: surgical management and clinical outcome. Egypt J Neurosurg. 2021;36:1-7.
  • Lorber J, Schofield JK. The prognosis of occipital encephalocele. Z Kinderchir Grenzgeb. 1979;28(4):347-351.
  • Basaran Gundogdu E, Kilicarslan N. Encephalocele: Retrospective Analysis and Our Clinical Experience. BSJ Health Sci. September 2022;5(3):370-378. doi:10.19127/bshealthscience.1087914
  • Akyol ME, Celegen I, Basar I, Arabacı O. Hydrocephalus in encephalocele. Eur Rev Med Pharmacol Sci. 2022;26(15):5399-5405. doi:10.26355/eurrev_202208_29407
  • Kiymaz N, Yilmaz N, Demir I, Keskin S. Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg. 2010;46(1):6-11. doi:10.1159/000314051
  • Nejat A, Berchi Kankam S, Heidari V, et al. The Predictors of Seizures in Patients with Encephalocele: An 11-Year Experience from a Tertiary Hospital. Pediatr Neurosurg. 2023;58(6):410-419. doi:10.1159/000534140
  • Nagao T, Hirokawa M. Diagnosis and treatment of macrocytic anemias in adults. J Gen Fam Med. 2017;18(5):200-204. doi:10.1002/jgf2.31
  • Merrell BJ, McMurry JP. Folic Acid. StatPearls Publishing; 2023. Accessed December 12, 2023. https://www.ncbi.nlm.nih.gov/books/NBK554487/

Early Clinical Outcomes of Congenital Encephalocele and Mortality Risk Factors: A Tertiary Center Experience

Year 2024, Volume: 1 Issue: 2, 124 - 130, 14.06.2024

Abstract

Objective: To investigate the early clinical outcomes of infants with congenital encephalocele
Methods: We investigated newborns diagnosed with congenital encephalocele and treated in our hospital. We recorded data of the patients regarding the delivery history, anthropometric features, and clinical outcomes. We used conventional statistical methods and Bayesian models.
Results: We included 18 patients (%61.1 female, 38.9% male) in the study. The median birth week was 38 (36.3-39) weeks, and the mean birth weight was 2837+/-816 grams. 83.3% of the patients had undergone an operation with a median of 5 days. The defect diameter was more than 5 cm in 61.1% of the patients, and brain parenchyma was positive in the sac in half of the patients. 22.2% of the patients needed ventriculoperitoneal shunt insertion. The overall survival resulted in 61.1% in all patients and 73.3% in operated. There were statistically significant differences in terms of birth weight (p<0.001, 3150 v.s 2470 grams) and birth week (38.6 v.s 34.6 weeks, p=0.021), as deceased patients had lower birth weight and birth week.
In Bayesian Kendall’s tau calculations, Neural tissue involvement, defect diameter more than 5 cm, birth weight (very strong evidence), operation within three days of life and birth week (strong evidence), and shunt need and seizures (moderate evidence) had an impact on mortality. There was no significant risk factor for hydrocephalus development, but there was a correlation between hydrocephalus and shunt need. Also, Dandy walker deformity correlated with shunt need (moderate evidence), but the birth week and neural tissue involvement were independent of shunt need (moderate evidence).
Conclusion: The poor prognostic factors for mortality were defect diameter larger than 5 cm, neural tissue involvement in the sac, lower birth week, lower birth weight, and seizures, and the good prognostic factor was operation before postnatal three days of life.

References

  • Sadler TW. Langman's Medical Embryology, Lippincott Williams & Wilkins, Philadelphia 1990. p.352.
  • Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA. Cephaloceles and related malformations. AJNR Am J Neuroradiol. 1992;13(2):655-690.
  • Lo BW, Kulkarni AV, Rutka JT, et al. Clinical predictors of developmental outcome in patients with cephaloceles. J Neurosurg Pediatr. 2008;2(4):254-257. doi:10.3171/PED.2008.2.10.254
  • Mai CT, Isenburg JL, Canfield MA, et al. National population-based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019;111(18):1420-1435. doi:10.1002/bdr2.1589
  • Siffel C, Wong LY, Olney RS, Correa A. Survival of infants diagnosed with encephalocele in Atlanta, 1979-98. Paediatr Perinat Epidemiol. 2003;17(1):40-48. doi:10.1046/j.1365-3016.2003.00471.x
  • Parker SE, Mai CT, Canfield MA, et al. Updated National Birth Prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Res A Clin Mol Teratol. 2010;88(12):1008-1016. doi:10.1002/bdra.20735
  • Czeizel AE, Dudás I. Prevention of the first occurrence of neural-tube defects by periconceptional vitamin supplementation. N Engl J Med. 1992;327(26):1832-1835. doi:10.1056/NEJM199212243272602
  • Tirumandas M, Sharma A, Gbenimacho I, et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29(5):739-744. doi:10.1007/s00381-012-1998-z
  • Stoll C, Alembik Y, Dott B. Associated malformations in cases with neural tube defects. Genet Couns. 2007;18(2):209-215.
  • Chen CP, Chern SR, Wang W. Rapid determination of zygosity and common aneuploidies from amniotic fluid cells using quantitative fluorescent polymerase chain reaction following genetic amniocentesis in multiple pregnancies. Hum Reprod. 2000;15(4):929-934. doi:10.1093/humrep/15.4.929
  • Anesen D, Rosman AK, Kandasamy R. Giant Occipital Encephalocele with Chiari Malformation Type 3. J Neurosci Rural Pract. 2018;9(4):619-621. doi:10.4103/jnrp.jnrp_103_18
  • Yucetas SC, Uçler N. A Retrospective Analysis of Neonatal Encephalocele Predisposing Factors and Outcomes. Pediatr Neurosurg. 2017;52(2):73-76. doi:10.1159/000452805
  • Joó JG, Beke A, Papp C, et al. Neural tube defects in the sample of genetic counselling. Prenat Diagn. 2007;27(10):912-921. doi:10.1002/pd.1801
  • Society for Maternal-Fetal Medicine, Monteagudo A. Posterior Encephalocele. Am J Obstet Gynecol. 2020;223(6):B9-B12. doi:10.1016/j.ajog.2020.08.177
  • Tomita T, Ogiwara H. Primary (congenital) encephalocele. In: Post TW, ed. UpToDate. UpToDate; 2013. Accessed December 10, 2023. https://www.uptodate.com
  • Tsuchida T, Okada K, Ueki K. The prognosis of encephaloceles (author's transl). No Shinkei Geka. 1981;9(2):143-150.
  • Kankam SB, Nejat A, Tavallaii A, Tayebi Meybodi K, Habibi Z, Nejat F. Hydrocephalus in patients with encephalocele: introduction of a scoring system for estimating the likelihood of hydrocephalus based on an 11-year experience from a tertiary center. J Neurosurg Pediatr. 2023;31(4):298-305. doi:10.3171/2022.12.PEDS22475
  • Protzenko T, Dos Santos Gomes Junior SC, Bellas A, Salomão JFM. Hydrocephalus and occipital encephaloceles: presentation of a series and review of the literature. Childs Nerv Syst. 2021;37(11):3437-3445. doi:10.1007/s00381-021-05312-7
  • Nagy MR, Saleh AE. Hydrocephalus associated with occipital encephalocele: surgical management and clinical outcome. Egypt J Neurosurg. 2021;36:1-7.
  • Lorber J, Schofield JK. The prognosis of occipital encephalocele. Z Kinderchir Grenzgeb. 1979;28(4):347-351.
  • Basaran Gundogdu E, Kilicarslan N. Encephalocele: Retrospective Analysis and Our Clinical Experience. BSJ Health Sci. September 2022;5(3):370-378. doi:10.19127/bshealthscience.1087914
  • Akyol ME, Celegen I, Basar I, Arabacı O. Hydrocephalus in encephalocele. Eur Rev Med Pharmacol Sci. 2022;26(15):5399-5405. doi:10.26355/eurrev_202208_29407
  • Kiymaz N, Yilmaz N, Demir I, Keskin S. Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg. 2010;46(1):6-11. doi:10.1159/000314051
  • Nejat A, Berchi Kankam S, Heidari V, et al. The Predictors of Seizures in Patients with Encephalocele: An 11-Year Experience from a Tertiary Hospital. Pediatr Neurosurg. 2023;58(6):410-419. doi:10.1159/000534140
  • Nagao T, Hirokawa M. Diagnosis and treatment of macrocytic anemias in adults. J Gen Fam Med. 2017;18(5):200-204. doi:10.1002/jgf2.31
  • Merrell BJ, McMurry JP. Folic Acid. StatPearls Publishing; 2023. Accessed December 12, 2023. https://www.ncbi.nlm.nih.gov/books/NBK554487/
There are 26 citations in total.

Details

Primary Language English
Subjects Intensive Care
Journal Section Research Articles
Authors

Akan Yaman 0000-0002-7550-6765

İbrahim Kandemir 0000-0002-1720-9711

Publication Date June 14, 2024
Submission Date March 25, 2024
Acceptance Date May 15, 2024
Published in Issue Year 2024 Volume: 1 Issue: 2

Cite

Vancouver Yaman A, Kandemir İ. Early Clinical Outcomes of Congenital Encephalocele and Mortality Risk Factors: A Tertiary Center Experience. Cerasus J Med. 2024;1(2):124-30.