Advertisement

Journal of Anesthesia

, Volume 25, Issue 2, pp 298–300 | Cite as

A novel association of alveolar capillary dysplasia, atypical duodenal atresia, and subglottic stenosis

  • Takeru Shimizu
  • Taeko Fukuda
  • Shinichi Inomata
  • Tsuyoshi Satsumae
  • Makoto Tanaka
Clinical Report

Abstract

Alveolar capillary dysplasia (ACD), which is a rare and lethal congenital pulmonary anomaly found in newborns, begins its onset or causes deterioration of the infant's condition some time after birth. Various congenital anomalies in combination with ACD have been reported, except for subglottic stenosis. Therefore, we aim to report a novel association in a case of ACD with the combination of atypical duodenal atresia and subglottic stenosis. The male infant was scheduled for duodeno-duodenostomy because a double-bubble sign was observed on a chest radiograph. He arrived at the operating theater without any symptoms. After induction of general anesthesia, although mask ventilation was performed without difficulties throughout the entire procedure, oxygen saturation values of the upper and lower extremities dissociated after several attempts of intubation. Surgery was canceled because of instability of the respiratory condition. Respiratory insufficiency worsened progressively, and the infant died at 5 days of age. An autopsy confirmed ACD and revealed cartilaginous subglottic stenosis, which had made intubation difficult. This report highlights the hazards of the onset and worsening of ACD, and the importance of thorough echocardiography before surgery when atypical duodenal atresia is suspected. Anesthesiologists should also be prepared for the difficulty of intubation.

Keywords

Alveolar capillary dysplasia Atypical duodenal atresia Subglottic stenosis 

Notes

Conflict of interest

This case was supported solely by departmental resources.

References

  1. 1.
    Khorsand J, Tennant R, Gillies C, Phillipps AF. Congenital alveolar capillary dysplasia: a developmental vascular anomaly causing persistent pulmonary hypertension of the newborn. Pediatr Pathol. 1985;3:299–306.PubMedCrossRefGoogle Scholar
  2. 2.
    Janney CG, Askin FB, Kuhn C III. Congenital alveolar capillary dysplasia—an unusual cause of respiratory distress in the newborn. Am J Clin Pathol. 1981;76:722–7.PubMedGoogle Scholar
  3. 3.
    Kitayama Y, Kamata S, Okuyama H, Usui N, Sawai T, Kobayashi T, Fukui Y, Okada A. Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia. J Pediatr Surg. 1997;32:99–100.PubMedCrossRefGoogle Scholar
  4. 4.
    Kinugasa H, Horigome H, Sugiura M, Saito T, Iijima T, Matsui A. Intravenous prostacyclin combined with inhaled nitric oxide therapy for an infant with alveolar capillary dysplasia. Pediatr Int. 2002;44:525–7.PubMedGoogle Scholar
  5. 5.
    Usui N, Kamiyama M, Kamata S, Yoneda A, Tazuke Y, Fukuzawa M. A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum. J Pediatr Surg. 2004;39:1808–11.PubMedCrossRefGoogle Scholar
  6. 6.
    Garola RE, Thibeault DW. Alveolar capillary dysplasia, with and without misalignment of pulmonary veins: an association of congenital anomalies. Am J Perinatol. 1998;15:103–7.PubMedCrossRefGoogle Scholar
  7. 7.
    Kane TD, Greenberg JM, Bove KE, Warner BW. Alveolar capillary dysplasia with misalignment of the pulmonary veins: a rare but fatal cause of neonatal respiratory failure. Pediatr Surg Int. 1998;14:89–91.PubMedCrossRefGoogle Scholar
  8. 8.
    Sears BE, Carlin J, Tunell WP. Severe congenital subglottic stenosis in association with congenital duodenal obstruction. Anesthesiology. 1978;49:214–5.PubMedCrossRefGoogle Scholar
  9. 9.
    Al-Hathlol K, Phillips S, Seshia MK, Casiro O, Alvaro RE, Rigatto H. Alveolar capillary dysplasia. Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature. Early Hum Dev. 2000;57:85–94.PubMedCrossRefGoogle Scholar
  10. 10.
    Michalsky MP, Arca MI, Groenman F, Hammond S, Tibboel D, Caniano DA. Alveolar capillary dysplasia: a logical approach to a fatal disease. J Pediar Surg. 2005;40:1100–5.CrossRefGoogle Scholar
  11. 11.
    Parker TA, Ivy DD, Kinsella JP, Torielli F, Ruyle SZ, Thilo EH, Abman SH. Combined therapy with inhaled nitric oxide and intravenous prostacyclin in an infant with alveolar-capillary dysplasia. Am J Respir Crit Care Med. 1997;155:743–6.PubMedGoogle Scholar
  12. 12.
    Starnes VA, Oyer PE, Bernstein D, Baum D, Gamberg P, Miller J, Shumway NE. Heart, heart–lung, and lung transplantation in the first year of life. Ann Thorac Surg. 1992;53:306–10.PubMedCrossRefGoogle Scholar
  13. 13.
    Haraida S, Lochbühler H, Heger A, Nerlich A, Diebold J, Wiest I, Müller-Höcker J, Löhrs U. Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension. Pediatr Pathol Lab Med. 1997;17:959–75.PubMedCrossRefGoogle Scholar
  14. 14.
    Moore KL, Persaud TVN, editors. The developing human, clinically oriented embryology. 7th ed, chaps 11, 12. Philadelphia: Saunders; 2003. p. 242–64.Google Scholar
  15. 15.
    Jacobs IN, Teague WG, Bland JW Jr. Pulmonary vascular complications of chronic airway obstruction in children. Arch Otolaryngol Head Neck Surg. 1997;123:700–4.PubMedGoogle Scholar
  16. 16.
    Dhara SS, Liu EH, Tan KH. Monitored transtracheal jet ventilation using a triple lumen central venous catheter. Anaesthesia. 2002;57:578–81.PubMedCrossRefGoogle Scholar

Copyright information

© Japanese Society of Anesthesiologists 2010

Authors and Affiliations

  • Takeru Shimizu
    • 1
  • Taeko Fukuda
    • 1
  • Shinichi Inomata
    • 1
  • Tsuyoshi Satsumae
    • 1
  • Makoto Tanaka
    • 1
  1. 1.Department of Anesthesiology and Critical Care Medicine, Graduate School of Comprehensive Human SciencesUniversity of TsukubaTsukubaJapan

Personalised recommendations