Vol 15, No 1 (2021)
Case report
Published online: 2021-01-11

open access

Page views 570
Article views/downloads 558
Get Citation

Connect on Social Media

Connect on Social Media

Palliative surgery in a patient diagnosed with trisomy 18 and rare neurological comorbidities

Paula Szenejko1, Stefan Anzelewicz2, Piotr Czauderna2
Palliat Med Pract 2021;15(1):76-79.

Abstract

Introduction: The authors describe a case of a patient with a prenatal diagnosis of Trisomy 18 with atypical
manifestation, rare anomalies of the central nervous system and subsequent surgical treatment.
Case presentation: The infant was diagnosed with holoprosencephalia, schizencephalia, corpus callosum
agenesis and severe hydrocephalus. No significant heart, gastrointestinal or kidney defects were found.
Being in otherwise good condition, the patient was qualified for a ventriculi-peritoneal shunt surgery due
to rapidly increasing head circumference. Following the uneventful operation patient’s parents reported
a general improvement in their child condition.
Conclusions: Bearing in mind the possibility of complications and the child’s poor prognosis, the reasons
for surgical intervention were evaluated, as well as the possible outcomes of withholding invasive treatment.
Discussion over the benefits of palliative surgery and the risk of ‘palliative harm’ illustrate how selected
children benefit from an interventionist approach.

Article available in PDF format

View PDF Download PDF file

References

  1. Rekate HL. The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Cerebrospinal Fluid Res. 2008; 5: 2.
  2. Munch T, Rostgaard K, Rasmussen ML, et al. Familial aggregation of congenital hydrocephalus in a nationwide cohort. Brain. 2012; 135(8): 2409–2415.
  3. Tully HM, Dobyns WB. Infantile hydrocephalus: a review of epidemiology, classification and causes. Eur J Med Genet. 2014; 57(8): 359–368.
  4. Metwalley KA, Farghalley HS, Abd-Elsayed AA. Congenital hydrocephalus in an Egyptian baby with trisomy 18: a case report. J Med Case Rep. 2009; 3: 114.
  5. Wilkinson D. Ethical Dilemmas in Postnatal Treatment of Severe Congenital Hydrocephalus. Camb Q Healthc Ethics. 2016; 25(1): 84–92.
  6. Graham E. Niemowlęta z Trisomią 18 i złożonymi wrodzonymi wadami serca nie powinny poddawać się otwartej operacji serca. J Law Medicine Ethics. 2016; 44(2): 286–291.
  7. Cereda A, Carey J. Zespół trisomii 18. Orphanet J Rare Dis. 2012; 7(1): 81.
  8. Rege SV, Patil H. Bilateral giant open-lip schizencephaly: A rare case report. J Pediatr Neurosci. 2016; 11(2): 128–130.
  9. Silberberg A, Robetto J, Grimaux G, et al. Ethical issues about the paradigm shift in the treatment of children with trisomy 18. Eur J Pediatr. 2020; 179(3): 493–497.
  10. Kattwinkel J, Perlman JM, Aziz K, et al. American Heart Association. Neonatal resuscitation: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Pediatrics. 2010; 126(5): e1400–e1413.
  11. Spierson H, Masood Y, Craigie RJ, et al. Trisomy 18: palliative surgical intervention. Arch Dis Child. 2018; 103(10): 1001.
  12. Dyer C. Parents fail to overturn ruling not to resuscitate baby. BMJ. 2005; 330(7498): 985.
  13. Jaykar R, Patil S. Indications of ventriculoperitoneal shunt: a prospective study. International Surgery Journal. 2017; 4(4): 1319.