Have you heard about the rare genetic disorder called 'Prader Willi Syndrome'? It is characterised by physical, mental and behavioural issues. This disorder can cause massive weight gain in children, due to constant sense of hunger, which mostly begins around 2 years of age. Onlymyhealth editorial team covered the case study of a 12 year old morbidly obese child suffering from Prader Willi Syndrome and other complications. A team of doctors at Max Super Speciality Hospital, Vaishali and Dr. Vivek Bindal Associate Director & Head– Max Institute of Minimal Access, Bariatric & Robotic Surgery, Max Super Speciality Hospital, Vaishali conducted bariatric surgery on her which was successful and the child has now lost 18 kgs, after two months of the surgery. Read this article further to know more about the true story.
Story of a 12-year-old girl Prachi
The female child Prachi, suffering from Prader Willi Syndrome was bed-ridden since the past 1.5 years. Along with the severe weight gain, the child’s condition was even more complicated because of obstructive sleep apnea and diabetes. The child was not being able to get up from the bed to play, walk or perform any other activities. In fact, the constant sitting had led to prolonged pressure on the skin, causing bed sores.
Talking about the condition of the child and the diagnosis, Dr. Vivek Bindal said, “The 12-year-old female child was experiencing excessive weight gain since birth. The child had been extensively evaluated at multiple hospitals by paediatric endocrinology and genetic departments with a full genetic and hormonal evaluation. She had been diagnosed with Prader-Willi Syndrome, a rare syndrome characterized by weight gain. She also had severe obstructive sleep apnea (a condition in which the soft tissues in the throat temporarily relax causing the airway to be narrowed or closed, and breathing is cut off momentarily) and her oxygen saturation used to dip below 70% during sleep. Thus, Growth Hormone therapy (a treatment for PraderWili Syndrome) could not be instituted for her."
"Her condition was so poor that she could not even sit in a wheel-chair. To add to all these conditions, she was additionally diagnosed with diabetes due to insulin resistance because of morbid obesity. The challenges this case presented to us were monumental and the parents were desperate to get her treated. Upon complete evaluation by experts from Pediatric endocrinology, pediatric intensivists, anesthesiologists and a bariatric surgery team, the child was diagnosed with Morbid Obesity with Metabolic Syndrome," he further added.
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Bariatric surgery: Best possible treatment option
The child's father was facing a lot of difficulties as most of the hospitals were not able to find the right choice of treatment for this disorder. Bariatric surgery as a treatment option was a challenging surgery due to high intra-abdominal fat and short height. At Max Hospital, Vaishali, the procedure was performed successfully and safely saving the 12-year-old child's quality of life.
Managing morbidly obese kids are very different from adults, and it can be extremely difficult for the caregivers as well. A large abdominal surface with small coelomic cavity (the space where the internal organs are suspended) can lead to operation difficulty due to high intra-abdominal fat and short height.
Details about the performed procedure
Dr. Bindal shared, “We performed Laparoscopic Sleeve Gastrectomy on the child. Gastric sleeve surgery or Sleeve gastrectomy is a procedure which requires creating a small stomach sleeve by using a stapling device, while the rest of the stomach is removed. This procedure gives a feeling of fullness to patients and reduces their hunger, causing weight loss and significant improvements in health. It is performed by removing approximately 80% of the stomach. The remaining stomach resembles a banana and hence is called ‘Sleeve’. The new stomach pouch holds smaller volume than normal stomach and reduces the amount of food intake."
He further added, "In this particular case, the child had a huge left lobe of liver reaching up to the umbilicus or the navel, making it very difficult to get space for operating on her stomach. The entire upper part of the stomach (gastric fundus) was mobilized to be removed. A gastric sleeve was created over gastric calibration tube using Ethicon powered staplers. The staple line was overlapped with a V loc for double security to prevent any post operative complications. All precautions were undertaken to ensure success of the surgery inkling an intra operative air insufflation test was also done, and it revealed no active bleeding and no leak.”
Also read: Weight Loss Diet For Obese Children Between 10-15 Years
Post bariatric surgery results
After getting the bariatric surgery, the child came out of anesthesia and was taken to the ward. She was even discharged after a couple of days without any pains. Prachi has now lost 18 kilograms in 2 months, and she is now able to stand with support. Both the child and her family are extremely happy and positive about her being able to walk again soon on her own. The patient got the surgery on September 14 and has lost around 18 kgs of body weight already.
What is Prader Willi Syndrome?
Prader-Willi syndrome is a rare genetic disorder that causes obesity, intellectual disability and short height. Prader-Willi syndrome is a syndrome which is mostly caused by the deletion of a part of chromosome 15. Patients suffering from this disorder mostly experience symptoms such as:
- Behavioural problems
- Intellectual disability
- Short height
- Increased sense of hunger which leads to obesity
Although, there is no treatment option for Prader-Willi syndrome, but a lot of patients can benefit by taking a customised diet plan. All possible measures were tried but failed to deal with the increasing weight of child. Thus, bariatric surgery was planned and conducted on the child at Max Hospital, Vaishali, after complete check-up and fitness for anaesthesia.
Also read: Home based weight management beneficial for both kids and parents
Speaking about the various options available for treatment and the final choice, Dr. Vivek Bindal said that bariatric surgery was the best treatment option to reduce weight for this case. Prader-Willi Syndrome can cause weight regain in long run even after bariatric surgery, but there was no other option to get the child out of the morbid obesity, diabetes, and severe OSA. However, the complication rate of Bariatric surgery in PraderWili Syndrome patients can be higher due to less healing ability of tissues in them.