Treatment of lumbar and intrathoracic meningocele: bioethical implications

Cristina Tornali; Andrea Pratico'; Flavio Vecchio; Agata Polizzi; Martino Ruggieri; Ignazio Vecchio

doi:10.23750/abm.v92i3.11600

Treatment of lumbar and intrathoracic meningocele: bioethical implications

Authors

Cristina Tornali Department of Clinical and Experimental Medicine, University of Catania, Italy
Andrea Pratico' a:1:{s:5:"en_US";s:21:"University of Catania";}
Flavio Vecchio Department of Clinical and Experimental Medicine, University of Catania, Italy
Agata Polizzi Chair of Pediatrics, Department of Educational Sciences, University of Catania, Italy
Martino Ruggieri Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Italy
Ignazio Vecchio Department of Clinical and Experimental Medicine, University of Catania, Italy

DOI: https://doi.org/10.23750/abm.v92i3.11600

Keywords:

MENINGOCELE, LUMBAR, INTRATHORACIC, BIOETHICS, IN-UTERO SURGERY

Abstract

Myelomeningocele is a congenital malformation caused by a developmental defect of the spinal cord structures. The exactcause is unknown, but different factors have been involved includingradiation, malnutrition, drugs. Myelomeningocele can develop at any point in the spine, but the lumbosacral region is affected in over 75% of cases. Chest X-raysand computed tomography study are mandatory to reveal tracheal malformations or associatedanomaliesof the ribs. Treatment of myelomeningocele must be multidisciplinary and involve at the same time neurologists, radiologists, neurosurgeons, thoracic surgeons, bioethical experts and take care of the childand also of the family. Some experiences concern the possibility of a in-utero correction of myelomeningocele, in order to avoiding serious and progressive damages to the nervoussystem. Given the improvement of myelomeningocele management, the quality of life is nowadays more acceptable than in the past; however, some severe forms of myelomeningocele cannot still be corrected: in this cases, a “non-interventional” approach may require a form of passive euthanasia that should be discussed and approved with and by parents and Any dissent of the parents must be respected and considered reasonable. The choice of a “non-intervention”, which should be guaranteed to all the people capable of self-determination, is not however so immediate and direct in the case of the minor: the dissent expressed on his behalf by the parents or legal representative may be ethically difficult to be accepted.In this case, the best interest of the child must prevail as the goal of any therapeutic choice.

References

Haslam RHA, The nervous system. Nelson Textbook of pediatric, W.B. Saunders, Philadelphia, 1996 pp.1793.

Kancherla V, Ibnehasan M, Hamid R, Paul L, Selhub J, Oakley G, et al. Prenatal folic acid use associated with decreased risk of myelomeningocele: a case control study offers further support for folic acid fortification in Bangladesh. Plos One. 2017 Nov.30; 12(11).

Adzick NS, Walsh DS. Myelomeningocele: prenatal diagnosis, pathophysiology and management. Seminars in Pediatric Surgery 2003; 12:168-174

Galli M, Albertini G, Romei M, Santambrogio GC, Tenore N, Crivellini M. Gait analysis in children affected by myelomeningocele: comparison of the various levels of lesion. Functional neurology. 2002;17:203-10.

Müller T, Arbeiter K, Aufricht C. Renal function in meningomyelocele: risk factors, chronic renal failure, renal replacement therapy and transplantation. Current opinion in urology. 2002; 12:479-84.

Wells TR, Jacobs RA, Senac MO, Landing BH. Incidence of short trachea in patients with myelomeningocele. Pediatric neurology. 1990;6:109-11.

Maiuri F, Corriero G, Giampaglia F, Simonetti L. Lateral thoracic meningocele. Surgical neurology. 1986; 26:409-12.

Das P, Goyal T, Hunt MA. Intrathoracic meningocele associated with neurofibromatosis Type 1 and a novel technique for surgical repair: case report. Journal of Neurosurgery: Spine. 2017; 27:291-4.

Duwe BV, Sterman DH, Musani AI. Tumors of the mediastinum. Chest. 2005;128:2893-909.

Costanzo M, Marziani A, Condorelli F, Migliore M, Cannizzaro MA. Post-thyroidectomy hypocalcemic syndrome: predictive value of early PTH. Preliminary results. Ann ital chir. 2010;81:301.

Migliore M, Costanzo M, Cannizzaro MA. Cervico-mediastinal goiter: is telescopic exploration of the mediastinum (video mediastinoscopy) useful? Interactive CardioVascular and Thoracic Surgery. 2010;10:439-40.

Migliore M, Vecchio I, Rampello L, Borrata FR, Astuto M, Rampello LI. Multisciplinary approach of non-thymomatous myasthenia gravis. Acta Med Medit. 2012; 28:211-3.

Migliore M. Efficacy and safety of single-trocar technique for minimally invasive surgery of the chest in the treatment of noncomplex pleural disease. J Thor cardiovasc surg. 2003; 126:1618.

Migliore M, Calvo D, Criscione A, Borrata F. Uniportal video assisted thoracic surgery: summary of experience, mini-review and perspectives. J Thor dises. 2015; 7(9):E378.

Rintoul NE, Sutton LN, Hubbard AM, Cohen B, Melchionni J, Pasquariello PS, Adzick NS. A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics. 2002; 109:409-13.

Jobe AH Fetal surgery for myelomeningocele. NEJM 2002; 347:230-1.

Moldenhauer JS, Adzick NS. Fetal surgery of myelomeningocele Fetal surgery for myelomeningocele: After the Management of Myelomeningocele study (MOMS). Semin Fetal Neonatal Med. 2017; 22:360-366.

Karmur BS, Kulkarni AV. Medical and socioeconomic predictors of quality of life in myelomeningocele patients with shunted hydrocephalus. Child's Nervous System. 2018;34:741-7.

Ramírez N, Valentín P, Mayer OH, et al. The Spinal Structure and Lung Function Relationship in an Untreated Nonambulatory Myelomeningocele Group of Patients. J Am Acad Orthop Surg. 2019;27:327–334.

Doers T, Walker JL, van den Brink K, Stevens DB, Heavilon J. The progression of untreated lumbar kyphosis and the compensatory thoracic lordosis in myelomeningocele. Dev Med Child Neurol. 1997;39:326–330.

McCarthy DJ, Sheinberg DL, Luther E, McCrea HJ. Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review. Neurosurg Focus. 2019;47:E5.

McHaffie HE, Laing IA, Parker M, McMillan J. Deciding for imperiled newborns: medical authority or parental autonomy?. Journal of Medical Ethics. 2001; 27:104-9.

Bacone F. Della dignità e del progresso delle scienze. Opere filosofiche. 1965;2:1-524.

Burgio GR, Marseglia GL, Cattorini P. Bioetica per il neonato. La problematica delle decisioni. AA. VV., Bioetica per il bambino, Pavia, Ed. Mediche Italiane. 1989:125-38.

Dworkin R. Il dominio della vita: aborto, eutanasia e libertà individuale; Edizioni di Comunità, Milano. 1994.

Kipnis K, Williamson G Nontretment decisions for severely compromised newborns, Ethics, 1984, 95:90-111.

Truog RD Futility in pediatrics: from case to policy. Journal of clinical ethics, 2000;11:136-141.

McHaffie HE, Cuttini M, Brölz-Voit G, Randag L, Mousty R, Duguet AM, et al. . Withholding/withdrawing treatment from neonates: legislation and official guidelines across Europe. Journal of Medical Ethics. 1999; 25:440-6.

Feinberg J. The moral limits of the criminal law: volume 3: harm to self. Oxford University Press on Demand; 1989 Aug 17.