Cephaloskeletal dysplasia (Taybi-Linder syndrome): Case report and anesthetic considerations

  • Jesús Acosta-Martínez Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
  • Rosana Guerrero-Domínguez Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
  • Daniel López-Herrera-Rodríguez Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
  • Marta García-Santigosa Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
  • Francisco Sánchez-Carrillo Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
  • María Luisa Marenco de la Fuente Anesthesiology and Resuscitation, Hospitales Universitarios Virgen del Rocío, Sevilla, Spain
Keywords: Dwarfism, Anesthesia, Child, Airway management, Osteochondrodysplasias

Abstract

Introduction: Microcephalic osteodysplastic primordial dwarfism (or Taybi-Linder syndrome) is a rare disease characterized by bone and central nervous system malformations, in addition to intrauterine retardation.

Case presentation: 20-year-old patient operated on for adhesiolysis and enteropexy due to bowel obstruction from post surgical adhesions.

Conclusion: The anesthetic considerations in these patients include the potential airway impairment secondary to facial malformations and neurological complications, primarily seizures.

References

1. Majewski F, Ranke M, Schinzel A. Studies of microcephalic primordial dwarfism II: the osteodysplastic type II of primordial dwarfism. Am J Med Genet. 1982;12:23-35.
2. Majewski F, Stoeckenius M, Kemperdick H. Studies of microcephalic primordial dwarfism III: an intrauterine dwarf with platyspondyly and anomalies of pelvis and clavicles osteodysplastic primordial dwarfism type III. Am J Med Genet. 1982;12:37-42.
3. Hall JG, Flora C, Scott CI Jr, Pauli RM, Tanaka KI. Majewski osteodysplastic primordial dwarfism type II (MOPDII): natural history and clinical findings. Am J Med Genet. 2004;130A:55-72.
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8. Pierce MJ, Morse RP. The neurologic findings in Taybi-Linder syndrome (MOPD I/III): case report and review of the literature. Am J Med A Genet. 2012;158A:606-10.
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10. Vichi GF, Currarino G, Wasserman RL, Duvina PL, Filippi L. Cephaloskeletal dysplasia (Taybi-Linder syndrome; osteodysplastic primordial dwarfism type III): report of two cases and review of the literature. Pediatr Radiol. 2000;30:644-52.
How to Cite
1.
Acosta-Martínez J, Guerrero-Domínguez R, López-Herrera-Rodríguez D, García-Santigosa M, Sánchez-Carrillo F, Marenco de la Fuente ML. Cephaloskeletal dysplasia (Taybi-Linder syndrome): Case report and anesthetic considerations. Colomb. J. Anesthesiol. [Internet]. 2016 Jan. 1 [cited 2024 Mar. 29];44(1):40-3. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/66

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Published
2016-01-01
How to Cite
1.
Acosta-Martínez J, Guerrero-Domínguez R, López-Herrera-Rodríguez D, García-Santigosa M, Sánchez-Carrillo F, Marenco de la Fuente ML. Cephaloskeletal dysplasia (Taybi-Linder syndrome): Case report and anesthetic considerations. Colomb. J. Anesthesiol. [Internet]. 2016 Jan. 1 [cited 2024 Mar. 29];44(1):40-3. Available from: https://www.revcolanest.com.co/index.php/rca/article/view/66
Section
Case Report / Case Series

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