Anaesthesia for a Child with Down syndrome UndergoingCleft Palate Repair and Review of Anaesthesia Management of Other Associated Congenital Syndromes

Abstract

Cleft lip/palate are the most common craniofacial anomalies in children, with an incidence of 1:800 live births. Cleft palate alone occurs in 1:2000 live births 1 .It occurs due to the failure of fusion or break in fusion of nasal and maxillary processes with the palatine shelves, which form during 8 th week of the embryonic period. About 150 syndromes may be associated with cleft deformities. The most well-known are the Pierre Robin's, TreacherCollins and Goldenhaar syndrome. Congenital heart disease (CHD) occurs in 5-10% of these patients 1 .Surgical repair of cleft lip is usually done at 1-3 months of age for cosmetic purpose and cleft palate at 6 months to 1 year of age to promote facial growth and the speech. The successful outcome following cleft repair depends on the age of the patient, associated morbidities, anaesthetic expertise and post-operative care 1 . Infants with facial deformities are usually associated with abnormal dentition/hearing defect, recurrent ear/upper respiratory tract infection (URTI), pulmonary aspiration and poor nutrition. Until recently criteria for cleft repair in infants was 10 pounds of weight, 10 weeks of age and haemoglobin of 10gm% 2 . Recent concepts of early repair in neonates are based on improvements in parent-infant bonding, feeding, growth and speech development 2 . Anaesthesia for cleft surgery in infant and children carries a higher risk with general anaesthesia and airway complications due to associated respiratory problems. Review of literature mentions higher incidence of perioperative respiratory complications when associated with the common cold symptoms in children for cleft repairs 3,4 . Morbidity during general anaesthesia is associated with the difficult airway, endotracheal (ET) tube compression/disconnection and post-operative airway obstruction 1,4 . Down syndrome or Trisomy 21 is the most common chromosomal abnormality, and similar to KFS it also results in anatomical changes of the airways, such as: cleft lip and palate, narrow nasopharynx, and relatively large and protuberating tongue. The larynx and the cricoid ring tend to be small predisposing to acquired subglottic stenosis. Patients might have atlantoaxial subluxation, making neck extension risky 1 . Here we are presenting a 5 yr old male child with weight of 12kg for cleft palate repair with Down syndrome done under general anaesthesia.He was having complete cleft palate was seen with a bifid uvula. The cardiovascular,respiratory and per abdominal examinations had no positive findings.However,CNS examination showed hypotonia in all four limbs with power 2/5 in upper limbs and lower limbs.

Cleft Palate in a Newborn With Trisomy 21: A Case Report

“…Congenital airway abnormalities may include a narrow nasopharynx, a large and protuberant tongue, a smaller larynx size, and a cricoid ring predisposing to acquired subglottic stenosis and cleft lip and palate [ 8 ]. While craniofacial defects are common, orofacial clefts in DS are quite rare, highlighting the atypical presentation that our case represents.…”

Anaesthetic Management in a Congenital Catharact Patient with Down Syndrome

“…Down's syndrome is also referred to as Trisomy 21 due to the presence ofan extra copy of chromosome number 21and the commonest of congenital anomalies occurring 1 in 800 live births 1,2 . The children are disabled by mental retardation (100% have IQ's below 65), seizure disorder (2-9%) .…”