Conference Lectures

SYNDROMES IN CONGENITAL CATARACT: ANAESTHETIC IMPLICATIONS
DrPushpa Susan Isaac
Giridhar Eye Institute,Cochin

Congenital cataracts are one of the most common treatable causes of visual impairment and blindness during infancy, with an estimated prevalence of 1 to 6 cases per 10,000 live births.It may be bilateral or unilateral.The bilateral cataracts maybe associated with syndromes.Some of the syndromes that are associated with cataract are :
1. Chromosomal abnormality---          Trisomy-21(Down),Trisomy-18(Edward), Trisomy-13(Patau )  .       
2.Craniofacial Syndromes—Hallerman-Streiff,Rubenstein –Taybi,Smith-Lemli-Opitz
3.Musculoskeletal Syndromes--Conradi,Albright,Myotonic Dystrophy
4.Renal--Lowe,Alport
5.Metabolic---   Galactosemia,Fabry,Wilson,mannisidosis,diabetes
6.Maternal Infection(TORCH diseases)
Rubella
Cytomegalo virus
Varicella
Syphillis
Toxoplasmosis
What are the problems that we are likely to encounter in children having syndromes coming for cataract surgery?
1.Pulmonary/ airway—mask ventilation---abnormal shape of the face, receding mandible,asymmetry of the face,macroglossia,microcephaly,macrocephaly,restrictive airway disease,sleepapnoea
Difficult Intubation
Maintaining saturation during and after surgery---some children may require post op ventilation
2.Cardiac ---cyanotic and acyanotic heart disease
Cardiac arrhythmias
Hypertension
Pulmonary hypertension
3.Musculoskeletal----spine deformities
Deformities of extremities
Susceptibility to fractures
Instabilitiy of spine-espatlanto –occipital instability
4.Fluid andElectrolyte imbalances—,Dehydration,K+,Ca++,Na+,Cl-,HCO3-
5.Metabolic disturbances—Blood sugar,urea, creatinine
6.Endocrine disturbances—Thyroid,parathyroid,pituitary,adrenal
7.Gastro intestinal disturbances---reflux disease—vomiting
Gaseous distension of abdomen
Imperforate anus
Trachea-oesophageal fistula
Oesophageal atresia
Omphalocoele
Diarrhea-leading to dehydration and electrolyte imbalance
8.Hepatic failure
9Renal failure
10.Nervous system anomalies:
Developmental abnormalities of brain and spinal cord
Mental retardation
Seizure disorder

  • Laboratory Tests : In contrast to unilateral cataracts, bilateral congenital cataracts may be associated with many systemic and metabolic diseases. A basic laboratory evaluation for bilateral cataracts of unknown etiology in apparently healthy children includes:
    • Blood Routine examination
    • Arterial blood gases
    •  Urine test for sugars, reducing substance, galactose 1-phosphate uridyltransferase, galactokinase, amino acids

- Infectious diseases: TORCH and varicella titers, VDRL
- Serum calcium, phosphorus, glucose and ferritin
-thyroid hormone levels
-electrolytes
-renal function tests
-liver function tests.
Cardiac Assessment---ECG,ECHO,Angiogram,etc
Respiratory System—CXR,PFT,SPO2,MRI,ETC
Nervous System---EEG,CTScan,MRI,etc
After assessing the child thoroughly,with the help of the paediatrician, ophthalmologist and the concerned specialists, the anaesthesia and surgery has to planned and executed.The parents have to be counselled thoroughly and informed well in advance about the risk factors involved in treating such cases.If the child has too many risk factors,it is advisable to do the procedure at a centre that is equipped to handle such high risk cases.A high risk consent has to be obtained prior to the surgery.Always have a high index of suspicion when doing congenital cataracts.