This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Luce, V. Articles by Benhamou, D. Search for Related Content PubMed PubMed Citation Articles by Luce, V. Articles by Benhamou, D.

---

LETTERS TO THE EDITOR

Anesthetic Management for a Parturient Affected by the VACTERL Association

Département d’Anesthésie-Réanimation, Hôpital Antoine Béclère, Clamart, France

To the Editor:

VACTERL is an acronym for vertebral, anal, cardiovascular, tracheo-esophageal, renal, and limb defects. Two or three of these criteria at least are needed for this diagnosis (1–5). A Cesarean delivery was scheduled at 37 weeks gestation for a 27-year-old, gravida 1, para 0 affected by this association. Indeed, this parturient had an imperforated anus and abnormalities of upper limbs, surgically treated during early childhood, and segmentation defects of the lumbar spine. Cardiac or renal abnormalities had never been noticed. Clinical examination showed a modified Mallampati class 3 score, a limited cervical extension, and a major dorsal scoliosis. Functional respiratory tests showed a forced vital capacity = 1.46 L (45% of predicted) and a FEV1/FVC ratio = 90%. A lumbar MRI had been already performed when the patient was 13 years old and had shown a medulla ending at L1 and no intramedullary abnormalities. Echocardiography was found to be normal. As this patient had a VACTERL association without cardiac and neurological component, plus an anticipated difficult endotracheal intubation with a respiratory insufficiency, regional anesthesia seemed the most appropriate. As expected, vertebral marks were difficult to identify because of the fixed lumbar scoliosis. However, the epidural space was found on first attempt in the L4–5 interspace. An adequate sensory block was obtained 20 minutes after the fractionated injection of 20 mL of 2% lidocaine with 1/200,000 epinephrine. Had failure occurred, general anesthesia with fiberoptic tracheal intubation was planned. Cesarean delivery and postoperative period were uneventful. This is the first description of the anesthetic management of a pregnant woman affected by the VACTERL association. Because of the diversity of the VACTERL abnormalities, it seems advisable to make a complete and careful assessment for each patient to help choosing the optimal anesthetic technique.

References

1. Rittler M, Paz JE, Castilla EE. VACTERL association, epidemiologic definition and delineation. Am J Med Genet 1996; 63: 529–36.[Web of Science][Medline]
2. Czeizel A, Ludanyi I. VACTERL-Association. Acta Morphol Hung 1984; 32: 75–96.[Web of Science][Medline]
3. Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, TE fistula with oesophagal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr 1973; 82: 104–7.[Web of Science][Medline]
4. Khoury MJ, Cordero JF, Greenberg F, et al. A population study of the VACTERL association: evidence for its etiologic heterogeneity. Pediatrics 1983; 71: 815–20.[Abstract/Free Full Text]
5. Martinez-Frias ML, Frias JL, Opitz JM. Errors of morphogenesis and developmental field theory. Am J Med Genet 1998; 76: 291–6.[Web of Science][Medline]

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Luce, V. Articles by Benhamou, D. Search for Related Content PubMed PubMed Citation Articles by Luce, V. Articles by Benhamou, D.