Case Report: Fetoscopic Laparoschisis (FETO-LAP)-A New Therapeutic Route to Explore for Fetuses with Severe Diaphragmatic Hernias

Abstract

Background: The purpose of this report is to describe the seminal case of a near-term human fetus with a life-threatening left diaphragmatic hernia that underwent fetoscopic tracheal occlusion (FETO) combined with fetoscopic partial removal of herniated bowel from the fetal chest by fetoscopic laparoschisis (FETO-LAP).

Case summary: A life-threatening left diaphragmatic hernia (liver-up; o/e LHR of ≤25%; MRI lung volume ≤ 20%) was observed in a human fetus at 34 weeks of gestation. After counselling the mother about the high risks of postnatal demise if left untreated, the expected limitations of fetoscopic tracheal occlusion (FETO), and the previously untested option of combining FETO with fetoscopic laparoschisis, i.e., partial removal of the herniated bowel from the fetal chest (FETO-LAP), she consented to the latter novel treatment approach. FETO-LAP was performed at 36 + 5 weeks of gestation under general maternofetal anesthesia. Mother and fetus tolerated the procedure well. The neonate was delivered and the balloon removed on placental support at 37 + 2 weeks of gestation. On ECMO, a rapid increase in tidal volume was seen over the next eight days. Unfortunately, after this period, blood clots obstructed the ECMO circuit and the neonate passed away.

Discussion: This seminal case shows that in a fetus with severe left diaphragmatic hernia, partial removal of the herniated organs from the fetal chest is not only possible by minimally invasive fetoscopic techniques but also well tolerated. As the effect of FETO alone is limited in saving severely affected fetuses, combining FETO with fetoscopic laparoschisis (FETO-LAP) offers a new therapeutic route with multiple, potentially life-saving implications.

Keywords: FETO; diaphragmatic hernia; experimental surgery; fetal intervention; fetal surgery; fetoscopy; laparoschisis; new techniques; tracheal balloon occlusion.

Figure 1

Cross-section through the chest in a human fetus with severe left-sided diaphragmatic hernia at 36 + 3 weeks + days of gestation. (Top)—Note the small area left for the fetal heart and right lung as a result of the large volume of herniated abdominal organs. (Bottom)—Oblique horizontal view of the upper fetal abdomen. This view was taken in order to follow the course of the intraabdominal umbilical vein. Note the close proximity of the left kidney, the leftwardly displaced coursing umbilical vein and the incision site (x). The left lateral vertical incision avoids the risk of injury to the larger abdominal wall vessels.

Figure 2

External aspect of the fully percutaneous, minimally invasive fetoscopic setup that required four short skin incisions and four percutaneous–transabdominal–transuterine–paraplacental punctures with 18-gauge needles (external diameter 1.2 mm) for over-the-wire insertion of four 11-F-canulas (external diameter 5 mm). Bottom left—Fetoscopic view during fetoscopic tracheal balloon occlusion, moments before detaching the latex balloon.

Figure 3

Employing monopolar cautery (top), scissors (middle) and various graspers, a vertical laparotomy was performed in the anterior axillary line, thus transsecting the oblique and transverse abdominal muscles. Through the opening, it was easily possible to introduce the fetoscope via the defect into the fetal chest and even insufflate the fetal thoracic cavity (bottom), gaining superior visibility of the herniated small and large bowel (bottom).

Figure 4

Fetoscopic view during FETO-LAP at the moment of eventeration of one bowel loop before the fetal abdomen (top). Neonatal aspect of the herniated bowel loops in the delivery suite (bottom).