Case 4 - Little Pui Pui

Little Pui Pui had already undergone three major surgeries with multiple medical problems at the age of 4. She suffered from Sotos syndrome which is a genetic condition causing physical overgrowth during the first years of life. She had related disorders such as hydrocephalus (“water’’ in the brain), congenital heart disease, laryngomalacia (congenital softening of the tissues of the larynx) and also scoliosis (curvature of the spine). Poor Little Pui Pui had to undergo complicated surgeries in her brain, heart and gastrostomy for feeding respectively in order to survive. Unfortunately her previous epilepsy and stroke causing muscle weaknesses on her right body which is known as hemiparesis.


With a referral from Pamela Youde Nethersole Eastern Hospital when she was 13 months, Little Pui Pui came to DKCH for the first time on 19 July 2010. Though she was given braces to help her spine, her two curves progressed and reached 54° and 39° on the consultation in Jul 2013.


Conventionally, internal bracing required using growing rods. However, this requires Little Pui Pui to undergo distraction surgery under general anesthesia every 6 months or so. Due to her young age, she may require changing the set of rods after 3-4 years because she may outgrow the distractible length available. Therefore inserting magnetic controlled growing rods was a better option over conventional rods. The non-invasive lengthening procedure eliminates the need for repeated lengthening surgeries. The surgery of growing rod was completed on 14 November 2013. Little Pui Pui continued to visit Scoliosis Clinic at DKCH to have the rod extraction regularly for further elongation. The total length of extraction was 1.5mm on the left and 2.8mm on the right. Her thoraic spine improved from 36° to 16° while her lumbar spine from 55° to 28°. 

 

Last update: June 2014