Twisha is born with pure isolated Long Gap Oesophageal Atresia –LGOA with the gap of 4vbs/6cms, type B as shown in this diagram. It is one type of Tracheoesophageal Fistula/Oesophageal Atresia -TOF/OA. This is very rare congenital birth defect which occurs 1 in 4000 babies. This birth defect prevents her eating/swallowing anything by mouth as her Oesophagus (food pipe) is not connected to her stomach. With TOF/OA some baby may have other birth defects “VACTRAL” where there is defect in the heart, kidney, air way, limbs, anus, etc. Fortunately Twisha was lucky to not to have any other birth defect related to TOF/OA. She is born healthy apart from her birth defect. She looks like a normal kid with dressing on. At present Twisha is given a spit fistula on the right side of her chest. It is the opening of her upper Oesophagus to drain her secretions and to help her learn sucking and oral fake feeding practising. She is actually fed by a Gastrostomy button on her belly, which delivers the food directly into her stomach. She is growing on it.
There are several methods (other than the Foker Method) to make a connection between the mouth to stomach. They are Gastric Pull UP, Gastric Tubes, Colonic Interposition, Jejunum Transposition, etc. But all these technique gives replacement to baby’s oesophagus not the repair. Here is the normal anatomy of the human body.
Here is the picture of Gastric Pull Up, where surgeon Pull baby’s whole stomach into the chest to complete the gap between Oesophageal ends. In our body, stomach always belongs to lower diaphragm. If it comes into the chest, it will be over crowded in Chest as there are our lungs and heart also functions. It is not the ideal place for stomach to be in chest. Our lungs need space to inflate and deflate. When stomach comes into chest, it affects lung capacity. Sometimes lungs collapse and stop working. Baby will have problem in digesting food, tolerating food, and so baby will have nutrition deficiency for lifelong. The stomach acid/content may go into her lungs (because of closeness of stomach’s opening and air pipe opening) and causes lung decease. It poses risk to chronic aspiration and recurrent pneumonia. Baby will have dumping syndrome (ie. diarrhea and low blood sugar) and chronic malnutrition. All these problems are very difficult to manage afterwards. It will cause lifelong medical complications.
Another technique is Gastric tube, where surgeon cuts baby’s stomach into half and makes a tube from ½ part of the stomach to make a connection between mouth and stomach. Even more that tube made from stomach doesn’t have peristaltic movement (the movement of esophageal muscles which moves the food down to stomach). So baby will have problem in swallowing. Food can only go into stomach by gravity. It works as a passage between mouth and stomach. . Baby will never be able to eat a full meal. In some baby’s stomach stops working. Baby keeps throwing up their food endlessly. And sometimes doesn’t survive with that. And baby will also lead to those problems related to digesting food and so malnutrition for rest of life.
In colonic interposition surgeon makes a graft from the colon and use it to make a link from mouth to stomach. In many patients this technique also hadn’t worked. That graft doesn’t allow the peristaltic movement as colon works differently than Oesophagus. Colon grows faster than Oesophagus. It will be a very different shape of colonic-oesophagus than normal oesophagus and it requires various revisions. There would be delay in food emptying to stomach. There will be also risk of Malnutrition. And baby is put at a higher risk of cancer.
All techniques other than Foker Technique give replacement to the baby. And Baby will have other lifelong medical complications. There is only the Foker Technique, which allows True Primary Repair and gives baby a normal quality of life. This technique is also called “growth procedure”. It is a reconstructive procedure of the Oesophagus, where baby’s Oesophagus grows under traction sutures. Baby is kept sedated & paralysed for couple of weeks/months during the whole procedure. And when the Oesophagus grows completely it’s been joined up. Here is the illustration of Foker Procedure.
Twisha has got very good working little tummy and She is already born with defect in her Oesophagus; by doing anything other than Foker procedure we will cause damage to the other organ of her body, where there is still possibility of Foker Procedure on her. She deserves this chance to have Foker Procedure at Boston to have a normal life in future. For more information on TOF/OA please follow the links below.
http://www.rch.org.au/oara/what/index.cfm?doc_id=1602[Page does not exist anymore]
Her birth defects
DISCLAIMER: The information and reference materials contained here are intended solely for the general information of the reader. This medical information is merely information – not an expert advice.Help To Give Twisha A Normal Life