Anaesthetic Management for Cataract Surgery in VACTERL Syndrome Case Report

Lecturer, Department of Anaesthesiology, Dr. D. Y. Patil Medical Collage & Hospital, Nerul, Navi-Mumbai 400706.
Indian journal of anaesthesia 02/2009; 53(1):94-7.
Source: PubMed

ABSTRACT SUMMARY: Eight year old girl, weighing 14 kg with VACTERL syndrome V: Vertebral anomalies, A: Anal malformation, C: Cardiovascular defect, TE: Tracheal and esophageal malformation, R: Renal agenesis, L: Limb anomalies. underwent cataract surgery under general anaesthesia. She had multiple congenital anomalies like esophageal atresia, imperforate anus (corrected), single kidney & radial aplasia. Anticipating problems of gastro-esophageal reflux & chronic renal failure, successful management was done.

3 Reads
  • [Show abstract] [Hide abstract]
    ABSTRACT: Duplications of the bladder is uncommon and rarely occurs as isolate anomaly. Two cases of complex malformations from the VACTERL-association are reported. The study of urogenital duplications in cases from the literature are described.
    Der Urologe 04/1998; 37(2):203-5. · 0.44 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Field theory provides a rational basis for birth defects terminology. During blastogenesis in higher metazoa, pattern formation in the primary field leads to the establishment of upstream expression domains of growth and transcription factors, which, in various permutations and at specific sites and times, lay down the pattern of progenitor fields. Further spatially coordinated, temporally synchronized, and epimorphically hierarchical morphogenetic events, mostly during organogenesis, lead to the attainment of final form in the secondary, epimorphic fields. Because of shared molecular determinants, spatial contiguity, and close timing of morphogenetic events during blastogenesis, most malformations arising during blastogenesis are polytopic, i.e., involving two or more progenitor fields, e.g., acrorenal, cardiomelic, gastromelic, or splenomelic anomalies. Defects of organogenesis tend to be monotopic malformations, e.g., cleft palate or postaxial polydactyly. We suggest that what were called "associations" (e.g., VATER, schisis) be designated primary polytopic developmental field defects, or simply polytopic field defects, and that the term "association" be reserved for the original definition of a statistical combination of anomalies (mostly of organogenesis) [Spranger et al. (1982): J Pediatr 100:160-165]. If genetically caused or predisposed, all structures involved in a polytopic or monotopic malformation are genetically abnormal, whereas the parts secondarily affected as a consequence of a malformation sequence (e.g., spina bifida) are genetically normal. Polytopic field anomalies, per se, must be distinguished from pleiotropy, although such anomalies may constitute a part of pleiotropy (e.g., in trisomy 18). Because they are downstream from pattern-forming events in the primary field, multiple anomalies of organogenesis more likely represent syndromal pleiotropy.
    American Journal of Medical Genetics 04/1998; 76(4):291-6. DOI:10.1002/(SICI)1096-8628(19980401)76:43.0.CO;2-T · 3.23 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Vater association is a polytopic disorder including several malformations of which radial hypoplasia is the most common observed at birth. This report aimed at describing a case of Vater association in a child submitted to renal transplantation. Male patient, 11 years old, 23 kg, Hbs positive, submitted to esophagostomy at 30 hours of life. Since 7 years of age patient is submitted to hemodialysis, currently via atrial catheter for the lack of other access ways. Renal transplantation with cadaver donor was performed without intercurrences with adequate diuresis after vascular anastomoses. Right iliac fossa hematoma was drained in the first postoperative day. Patient was discharged 21 days after transplantation with normal renal function. Vater association is an extremely rare and complex event and this report aimed at describing for the first time a renal transplantation with cadaver donor in a child with this congenital defect, the result of which has been fully satisfactory.
    Revista brasileira de anestesiologia 07/2003; 53(3):388-91. · 0.51 Impact Factor