[MIM 158 170]

(Alfi syndrome, monosomy 9 p, 9 p syndrome)

Rare. Partial deletion of the short arm of chromosome 9. The severity of the clinical presentation depends on the number of genes lost.

Clinical signs:

-         psychomotor and developmental retardation

-         trigonocephaly

-         micrognathia, small mouth with an arched or cleft palate, choanal stenosis or atresia

-         narrow upslanting palpebral fissures, hypertelorism. low set and improperly hemmed ears; flattened bridge of the nose

-          long fingers, occasional polydactyly

-         heart defects (ASD, VSD, patent ductus arteriosus, coarctation of the aorta) or renal malformation

-         occasional: omphalocele, diaphragmatic hernia, scoliosis

Anesthetic implications: 

cardiac ultrasound, risk of difficult intubation, hypotonia, craniosynostosis

References : 

-          Cakmakaya OA, Bakan M, Altintas F, Kaya G. 
Anesthetic management in a child with deletion 9p syndrome. 
Pediatr Anesth 2007; 17:88-9.

-         Sirisena ND, Wijetunge UKS, de Silva R, Dissanayake VHW. 
Child with deletion 9p syndrome presenting with craniofacial dysmorphism, developmental delay, and multiple congenital malformations. 
Case Reports in Genetics 2013; ID 785830, 4 p

-         Sanket B, Ravindra M, Ramavakoda CY. 
Anaesthetic considerations in an infant with a rare genetic condition: chromosome 9p22 deletion syndrome.
Indian J Anaesth 2015; 59:516-7 

Updated: August 2019