Skip to main content
SpringerLink
Log in

Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome

  • Clinical Report
  • Published:
Journal of Anesthesia Aims and scope Submit manuscript

Abstract

Providing anesthesia to patients with Brachmann-de Lange syndrome (BdLS) may be challenging, mainly because of intubation difficulty, gastroesophageal reflux disease, and aspiration complications. The use of spinal anesthesia (SA) in this population has not been reported. We report the uneventful administration of awake SA to a 7-month-old girl with BdLS who was scheduled for rectal biopsy. The current literature is reviewed to discuss the indications for SA in those patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Baum VC. Anesthesia for genetic, metabolic, and dysmorphic syndromes of childhood. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2007. p. 85–6.

    Google Scholar 

  2. Beck B, Fenger K. Mortality, pathological findings and causes of death in the De-Lange syndrome. Acta Paediatr Scand. 1985;74:765–9.

    Article  CAS  PubMed  Google Scholar 

  3. Dorsett D, Krantz ID. On the molecular etiology of Cornelia de Lange syndrome. Ann N Y Acad Sci. 2009;1151:22–37.

    Article  CAS  PubMed  Google Scholar 

  4. Sargent WW. Anesthetic management of a patient with Cornelia de Lange syndrome. Anesthesiology. 1991;74:1162–3.

    Article  CAS  PubMed  Google Scholar 

  5. Moschini V, Ambrosini MT, Sofi G. Anesthesiologic considerations in Cornelia de Lange syndrome. Minerva Anestesiol. 2000;66:799–806.

    CAS  PubMed  Google Scholar 

  6. Yo T, Noguchi I, Kimura T, Sasao M, Amemiya Y, Sekiya H, Kobayashi K. General anesthesia in a patient with Cornelia de Lange syndrome with restricted opening of the mouth. Masui. 1998;47:611–4. (in Japanese with English abstract).

    CAS  PubMed  Google Scholar 

  7. Fernández-García R, Pérez Mencía T, Gutiérrez-Jodra A, López García A. Anesthetic management with laryngeal mask in a child with Brachmann-de Lange syndrome. Paediatr Anaesth. 2006;16:698–700.

    Article  PubMed  Google Scholar 

  8. Tsukazaki Y, Tachibana C, Satoh K, Fukada T, Ohe Y. A patient with Cornelia de Lange syndrome with difficulty in orotracheal intubation. Masui. 1996;45:991–3. (in Japanese with English abstract).

    CAS  PubMed  Google Scholar 

  9. Yokoyama T, Tomoda M, Nishiyama T, Matsuda T, Fujimoto S, Hanaoka K, Manabe M. General anesthesia for a patient with Cornelia de Lange syndrome. Masui. 2000;49:785–7. (in Japanese with English abstract).

    CAS  PubMed  Google Scholar 

  10. Hirai T, Nitahara K, Higa K, Iwakiri S, Shono S, Katori K. Anesthetic management of an infant with Cornelia de Lange syndrome. Masui. 2006;55:454–6. (in Japanese with English abstract).

    PubMed  Google Scholar 

  11. Guzman J. Use of a short flexible fiberoptic endoscope for difficult intubations. Anesthesiology. 1997;87:1563–4.

    Article  CAS  PubMed  Google Scholar 

  12. August DA, Sorhabi S. Is a difficult airway predictable in Cornelia de Lange syndrome? Paediatr Anaesth. 2009;19:707–9.

    Article  PubMed  Google Scholar 

  13. Veall GR. An unusual complication of Cornelia de Lange syndrome. Anaesthesia. 1994;49:409–10.

    Article  CAS  PubMed  Google Scholar 

  14. Papadimos TJ, Marco AP. Lange syndrome, hyperthermia and a difficult airway. Anaesthesia. 2003;58:924–5.

    Article  CAS  PubMed  Google Scholar 

  15. Ozturk E, Bozkirli F, Ozkose Z. Anaesthetic management of a patient with Cornelia de Lange syndrome. Gazi Med J. 2000;11:175–7.

    Google Scholar 

  16. Lumb AB, Carli F. Respiratory arrest after a caudal injection of bupivacaine. Anaesthesia. 1989;44:324–5.

    Article  CAS  PubMed  Google Scholar 

  17. Fortuna A. Respiratory arrest after a caudal injection of bupivacaine. Anaesthesia. 1989;44:1007.

    Article  CAS  PubMed  Google Scholar 

  18. Lee E, Collins G. Respiratory arrest after caudal bupivacaine. Anaesthesia. 1990;45:63–4.

    Article  CAS  PubMed  Google Scholar 

  19. del Rio ER, Cordero Luna D, Vásquez Montes MT, Chávez Ramírez MA, Rodríguez Pérez MV, Cruz-Villaseñor JA, Rodríguez Aranda E, Olvera Durán JA. Caudal block and sedation for bilateral herniorrhaphy in an infant with Cornelia de Lange syndrome. Rev Esp Anestesiol Reanim. 2004;51:403–4.

    PubMed  Google Scholar 

  20. Williams RK, Adams DC, Aladjem EV, Kreutz JM, Sartorelli KH, Vane DW, Abajian JC. The safety and efficacy of spinal anesthesia for surgery in infants: the Vermont Infant Spinal Registry. Anesth Analg. 2006;102:67–71.

    Article  PubMed  Google Scholar 

  21. Kachko L, Simhi E, Tzeitlin E, Efrat R, Tarabikin E, Peled E, Metzner I, Katz J. Spinal anesthesia in neonates and infants. A single-center experience of 505 cases. Paediatr Anaesth. 2007;17:647–53.

    Article  PubMed  Google Scholar 

  22. Katznelson R, Mishaly D, Hegesh T, Perel A, Keidan I. Spinal anesthesia for diagnostic cardiac catheterization in high-risk infants. Paediatr Anaesth. 2005;15:50–3.

    Article  PubMed  Google Scholar 

  23. Peterson KL, DeCampli WM, Pike NA, Robbins RC, Reitz BA. A report of two hundred twenty cases of regional anesthesia in pediatric cardiac surgery. Anesth Analg. 2000;90:1014–9.

    Article  CAS  PubMed  Google Scholar 

  24. Chaney MA. Intrathecal and epidural anesthesia and analgesia for cardiac surgery. Anesth Anal. 2006;102:45–64.

    Article  CAS  Google Scholar 

  25. Roposch A, Bhaskar AR, Lee F, Adedapo S, Mousny M, Alman BA. Orthopaedic manifestations of Brachmann-de Lange syndrome: a report of 34 patients. J Pediatr Orthop B. 2004;13:118–22.

    Article  PubMed  Google Scholar 

  26. Mutlu M, Dilber B, Aslan Y. Cornelia de Lange syndrome associated with thoracic meningocele. Clin Dysmorphol. 2010;19:161–3.

    Article  PubMed  Google Scholar 

  27. Viscomi CM, Abajian JC, Wald SL, Rathmell JP, Wilson JT. Spinal anesthesia for repair of meningomyelocele in neonates. Anesth Analg. 1995;81:492–5.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Anesthesia, Schneider Children’s Medical Center of Israel (SCMCI) (Affiliated to Sackler Faculty of Medicine, Tel Aviv University), 14 Kaplan Street, 49202, Petah Tiqwa, Israel

    Ludmyla Kachko, Elena Sanko & Jacob Katz

  2. Department of Pediatric and Adolescent Surgery, Schneider Children’s Medical Center of Israel (SCMCI) (Affiliated to Sackler Faculty of Medicine, Tel Aviv University), Petah Tiqwa, Israel

    Enrique Freud

Authors
  1. Ludmyla Kachko
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elena Sanko
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Enrique Freud
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jacob Katz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ludmyla Kachko.

About this article

Cite this article

Kachko, L., Sanko, E., Freud, E. et al. Spinal anesthesia in a child with Brachmann-de Lange (Cornelia de Lange) syndrome. J Anesth 24, 942–944 (2010). https://doi.org/10.1007/s00540-010-1026-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00540-010-1026-y

Keywords

Search

Navigation