Retrospective evaluation of operated meningomyelocele cases

Year 2023, , 133 - 141, 30.08.2023

Ali Özen , Selahattin Akar

https://doi.org/10.30569/adiyamansaglik.1313886

Abstract

Aim: In this study, it was aimed to retrospectively evaluate the preoperative, peroperative and postoperative data of operated meningomyelocele patients.
Materials and Methods: Meningomyelocele patients who were operated between January 2018 and March 2020 were found from the hospital database and examined retrospectively. Demographic data, birth data, neurological status, surgical findings, radiological data, postoperative follow-ups and results were evaluated.
Results: Ten of the patients were girls and 11 were boys. Twenty patients were operated in the first 48 hours. One patient was operated on the 6th postpartum day. Hydrocephalus developed in 18 of the patients and a ventriculoperitoneal shunt was inserted. Three patients had skin necrosis, one patient had wound infection and cerebro-spinal fluid fistula. In one patient, shunt revision was performed due to shunt dysfunction during follow-up. All patients were discharged from the neonatal intensive care unit with recovery.
Conclusions: Meningomyelocele patients are at risk of meningitis, sepsis, and neurological deficits that may progress from the time of birth. Multidisciplinary close follow-up and early surgery are important in reducing complications.

Keywords

Spina bifida, Meningomyelocele, Hydrocephalus, Chiari Malformation

Opere edilen meningomyelosel olgularının retrospektif değerlendirilmesi

Abstract

Amaç: Bu çalışmada opere edilen meningomyelosel hastalarının pre-operatif, peri-operatif ve post-operatif verilerinin retrospektif değerlendirilmesi amaçlanmıştır.
Gereç ve Yöntem: Ocak 2018 – Mart 2020 tarihleri arasında opere edilen meningomyelosel hastaları hastane arşivinden bulunarak retrospektif olarak incelenmiştir. Demografik veriler, doğum verileri, nörolojik durumları, ameliyat bulguları, radyolojik verileri, ameliyat sonrası takipleri ve sonuçları değerlendirilmiştir.
Bulgular: Hastaların 10’u kız ve 11’i erkekti. Yirmi hasta ilk 48 saatte opere edildi. Bir hasta postpartum 6.günde opere edildi. Hastaların 18’inde hidrosefali gelişti ve ventriküloperitoneal şant takıldı. Üç hastada cilt nekrozu, bir hastada yara yeri enfeksiyonu ve beyin omurilik sıvısı fistülü görüldü. Bir hastada takiplerinde şant disfonksiyonu nedeni ile şant revizyonu yapıldı. Hastaların hepsi şifa ile yenidoğan yoğun bakım ünitesinden taburcu oldu.
Sonuç: Meningomyelosel hastaları doğum anından itibaren menenjit, sepsis, ilerleyebilecek nörolojik defisit riski altındadır. Hastalarda multidisipliner yakın takip ve erken cerrahi, komplikasyonları azaltmada önemlidir.

Keywords

Spina bifida, Meningomyelosel, Hidrosefali, Chiari Malformasyonu

References

• Kaufman BA. Neural tube defects. Pediatr Clin North Am. 2004;51(2):389-419. doi:10.1016/S0031-3955(03)00207-4
• Pinar H, Tatevosyants N, Singer DB. Central nervous system malformations in a perinatal/neonatal autopsy series. Pediatr Dev Pathol. 1998;1(1):42-48. doi:10.1007/S100249900005
• Kibar Z, Capra V, Gros P. Toward understanding the genetic basis of neural tube defects. Clin Genet. 2007;71(4):295-310. doi:10.1111/J.1399-0004.2007.00793.X
• De Marco P, Merello E, Mascelli S, Capra V. Current perspectives on the genetic causes of neural tube defects. Neurogenetics. 2006;7(4):201-221. doi:10.1007/S10048-006-0052-2
• Wolujewicz P, Steele JW, Kaltschmidt JA, Finnell RH, Ross ME. Unraveling the complex genetics of neural tube defects: From biological models to human genomics and back. Genesis. 2021;59(11). doi:10.1002/DVG.23459
• Shaer CM, Chescheir N, Schulkin J. Myelomeningocele: a review of the epidemiology, genetics, risk factors for conception, prenatal diagnosis, and prognosis for affected individuals. Obstet Gynecol Surv. 2007;62(7):471-479. doi:10.1097/01.OGX.0000268628.82123.90
• Boyd PA, Wellesley DG, De Walle HEK, et al. Evaluation of the prenatal diagnosis of neural tube defects by fetal ultrasonographic examination in different centres across Europe. J Med Screen. 2000;7(4):169-174. doi:10.1136/JMS.7.4.169
• Liptak GS, Dosa NP. Myelomeningocele. Pediatr Rev. 2010;31(11):443-450. doi:10.1542/PIR.31-11-443
• Tomatir AG, Vural BK, Acikbas I, Akdag B. Registries of cases with neural tube defects in Denizli, Turkey, 2004-2010. Genet Mol Res. 2014;13(4):8537-8543. doi:10.4238/2014.OCTOBER.20.30
• İlhan Ö, Alkan Özdemi̇r S, Özdemi̇r N, et al. Meningomyelosel Olgularında Prognozu Etkileyen Faktörler ve Eşlik Eden Anomaliler: 40 Olguluk Deneyim Prognostic Factors And Accompanying Anomalies In Neonates With Meningomyelocele: Experience Of 40 Patients. Jinekoloji - Obstetrik ve Neonatoloji Tıp Dergisi. 2017;14(4):163-167.
• Alkan Özdemir S, Özdemir N, Arun Özer E, et al. Meningomyeloselli 50 yenidoğanın prospektif-gözlemsel değerlendirilmesi. Ege Tıp Dergisi. 2017;56(3):135-139. doi:10.19161/ETD.391545
• Okurowska-Zawada B, Kozerska A, Zelazowska B, Kułak W, Wasilewska A, Wysocka J. Serum 25-hydroxyvitamin D, osteocalcin, and parathormone status in children with meningomyelocele. Neuropediatrics. 2012;43(6):314-319. doi:10.1055/S-0032-1327126
• Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers. 2015;1. doi:10.1038/NRDP.2015.7
• Volpe J. Neural tube formation and prosencephalic development. In: Volpe J, ed. Neurology of the Newborn. WB Saunders; 2001:3-44.
• Lewis D, Tolosa JE, Kaufmann M, Goodman M, Farrell C, Berghella V. Elective cesarean delivery and long-term motor function or ambulation status in infants with meningomyelocele. Obstetrics and gynecology. 2004;103(3):469-473. doi:10.1097/01.AOG.0000113624.94710.CE
• Bartonek Å, Saraste H, Knutson LM. Comparison of different systems to classify the neurological level of lesion in patients with myelomeningocele. Dev Med Child Neurol. 1999;41(12):796-805. doi:10.1017/S0012162299001607
• Oi S, Matsumoto S. A proposed grading and scoring system for spina bifida: Spina Bifida Neurological Scale (SBNS). Childs Nerv Syst. 1992;8(6):337-342. doi:10.1007/BF00296565
• Glard Y, Launay F, Viehweger E, Hamel A, Jouve JL, Bollini G. Neurological classification in myelomeningocele as a spine deformity predictor. J Pediatr Orthop B. 2007;16(4):287-292. doi:10.1097/01.BPB.0000165181.66291.53
• Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993-1004. doi:10.1056/NEJMOA1014379
• Houtrow AJ, MacPherson C, Jackson-Coty J, et al. Prenatal Repair and Physical Functioning Among Children With Myelomeningocele: A Secondary Analysis of a Randomized Clinical Trial. JAMA Pediatr. 2021;175(4). doi:10.1001/JAMAPEDIATRICS.2020.5674
• Goodnight WH, Bahtiyar O, Bennett KA, et al. Subsequent pregnancy outcomes after open maternal-fetal surgery for myelomeningocele. Am J Obstet Gynecol. 2019;220(5):494.e1-494.e7. doi:10.1016/J.AJOG.2019.03.008
• Bennett KA, Carroll MA, Shannon CN, et al. Reducing perinatal complications and preterm delivery for patients undergoing in utero closure of fetal myelomeningocele: further modifications to the multidisciplinary surgical technique. J Neurosurg Pediatr. 2014;14(1):108-114. doi:10.3171/2014.3.PEDS13266
• Iskandar BJ, Finnell RH. Spina Bifida. Ropper AH, ed. N Engl J Med. 2022;387(5):444-450. doi:10.1056/NEJMRA2116032
• McLone DG. Care of the neonate with a myelomeningocele. Neurosurg Clin N Am. 1998;9(1):111-120. doi:10.1016/s1042-3680(18)30284-5
• Charney EB, Weller SC, Sutton LN, Bruce DA, Schut LB. Management of the newborn with myelomeningocele: time for a decision-making process. Pediatrics. 1985;75(1):58-64. doi:10.1542/peds.75.1.58
• Tarcan T, Önol FF, Ilker Y, Alpay H, Şimşěk F, Özek M. The timing of primary neurosurgical repair significantly affects neurogenic bladder prognosis in children with myelomeningocele. J Urol. 2006;176(3):1161-1165. doi:10.1016/J.JURO.2006.04.042
• Crawford AH, Strub WM, Lewis R, et al. Neonatal kyphectomy in the patient with myelomeningocele. Spine (Phila Pa 1976). 2003;28(3):260-266. doi:10.1097/01.BRS.0000042234.98512.BE
• Sharrard WJ. Spinal osteotomy for congenital kyphosis in myelomeningocele. J Bone Joint Surg Br. 1968;50(3):466-471. Accessed July 17, 2023. http://www.ncbi.nlm.nih.gov/pubmed/4882211
• Eckstein HB, Vora RM. Spinal osteotomy for severe kyphosis in children with myelomeningocele. J Bone Joint Surg Br. 1972;54(2):328-333. Accessed July 17, 2023. http://www.ncbi.nlm.nih.gov/pubmed/4556184
• Lindseth RE, Stelzer L. Vertebral excision for kyphosis in children with myelomeningocele. J Bone Joint Surg Am. 1979;61(5):699-704. Accessed July 17, 2023. http://www.ncbi.nlm.nih.gov/pubmed/457713
• Christofersen MR, Brooks AL. Excision and wire fixation of rigid myelomeningocele kyphosis. J Pediatr Orthop. 1985;5(6):691-696. doi:10.1097/01241398-198511000-00012
• Martin J, Kumar SJ, Guille JT, Ger D, Gibbs M. Congenital kyphosis in myelomeningocele: results following operative and nonoperative treatment. J Pediatr Orthop. 1994;14(3):323-328. doi:10.1097/01241398-199405000-00009
• Özdemir N, Özdemir SA, Özer EA. Kyphectomy in neonates with meningomyelocele. Childs Nerv Syst. 2019;35(4):673-681. doi:10.1007/S00381-018-4006-4
• Dias MS, McLone DG. Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am. 1993;4(4):715-726. doi:10.1016/s1042-3680(18)30561-8
• Miller PD, Pollack IF, Pang D, Albright AL. Comparison of simultaneous versus delayed ventriculoperitoneal shunt insertion in children undergoing myelomeningocele repair. J Child Neurol. 1996;11(5):370-372. doi:10.1177/088307389601100504
• Chadduck WM, Reding DL. Experience with simultaneous ventriculo-peritoneal shunt placement and myelomeningocele repair. J Pediatr Surg. 1988;23(10):913-916. doi:10.1016/S0022-3468(88)80383-X
• Parent AD, McMillan T. Contemporaneous shunting with repair of myelomeningocele. Pediatr Neurosurg. 1995;22(3):132-136. doi:10.1159/000120890
• Gamache FW. Treatment of hydrocephalus in patients with meningomyelocele or encephalocele: a recent series. Childs Nerv Syst. 1995;11(8):487-488. doi:10.1007/BF00334972
• Caldarelli M, Di Rocco C, La Marca F. Shunt complications in the first postoperative year in children with meningomyelocele. Childs Nerv Syst. 1996;12(12):748-754. doi:10.1007/BF00261592