Long Gap Discussion: EA & TEF

Blind probing of the distal esophagus in long-gap EA can inadvertently push the diaphragm of an infant all the way up to the upper chest cavity without causing perforation, leading to overestimation of the gap.

The gastroesophageal junction in pure long-gap esophageal atresia is very tiny and requires manipulation of the scope to enter, as it tends to flip out.

Using an endoscope to measure the gap does not allow objective measurement of the boost force applied, unlike using a Hegar dilator with force measurement.

Gap measurement should wait at least 15 days after gastrostomy placement before the first measurement, using a Hegar number 4 dilator after endoscopic confirmation of the cardia location.

Some babies with long-gap EA have no cardia at all.

A contrast study performed at 3 weeks after gastrostomy placement, even in Trendelenburg position, may not fill the distal esophageal segment completely, giving a false sense of the real gap.

Professor Spitz would not perform a cervical esophagostomy immediately in a gasless abdomen case, but would wait a couple of weeks to assess the gap using a Hagar or urethral dilator under fluoroscopic control.

Professor Spitz would opt for esophageal replacement if the gap is more than 5 or 6 vertebrae at 4 to 6 weeks.

Miguel's approach is to place a laparoscopic gastrostomy, measure the gap every 2 weeks until it is less than 2 vertebral bodies, and start thinking about replacement if this is not achieved by 8 weeks.

Professor Bagalo performs gastrostomy at birth and gap measurement every 15 days until 6 weeks, at which point delayed anastomosis is generally possible for inborn babies.

Holger endoscopes all long-gap patients to intubate the lower esophageal sphincter and assess both the length and quality of the lumen.

Holger clips the fistula and leaves it attached, waiting to observe for spontaneous growth, then assesses with endoscope in both upper and lower pouches; if the gap is less than 3 vertebral bodies or centimeters, he proceeds with primary repair.

Jack's institution has interventional radiology place the G-tube and check the gap using a wire threaded through the G-tube into the esophagus, checking every month and allowing up to 3 months for the ends to grow close enough together.

Dr. Folker's institution performs an unstressed gappogram with contrast above and into the lower esophagus to assess the gap, and proceeds with operation once the child is 3 to 3.5 kg and sturdy if the lower esophageal segment is fairly good size.

If after mobilization the tension is judged to be more than desired, Dr. Folker places the esophageal ends on internal traction and waits one week, during which significant growth is achieved and the ends can then be anastomosed.

Even esophageal ends that are 2 to 4 millimeters in size, when placed on traction, will grow into a serviceable esophagus.

Dr. Folker's first patient from Boston was reported to have a gap of 2 centimeters, but this was due to pushing the diaphragm up into the chest; the actual distal esophagus was only a 5 to 6 millimeter nubbin.