Open fetal surgery
In 1981, Harrison performed the first open fetal surgical intervention for a case of LUTO, which was not eligible for shunt placement (55). Over the last 30 years, open techniques have been used in the treatment of cystic lung lesions, congenital diaphragmatic hernia, cardiac malformations, SCT, and more recently fetal myelomeningocoele (MMC).
The exposed fetal neural placode in MMC is susceptible to further injury in utero and this mechanism forms the basis of the ‘two-hit’ hypothesis of neurological injury in MMC. Individuals affected by MMC have varying degrees of motor and somatosensory deficits resulting in bladder and bowel dysfunction. Central nervous system complications including hydrocephalus requiring ventriculoperitoneal (VP) shunting arise secondary to hind brain herniation also known as an Arnold-Chiari II malformation. Until recently, postnatal surgical treatment of MMC was the only option. The rationale for in utero repair is to target the second hit of the hypothesis, that is, to limit the exposure of the neural placode to direct trauma and toxins within the amniotic fluid and to reduce ongoing cerebrospinal fluid leak. One of first reports of open fetal MMC repair came from a Vanderbilt University (Nashville, Tennessee, USA) team in 1999 who conducted a nonrandomized observational study over a 9-year period (56). With a study sample size of 29 patients with isolated fetal MMC referred for intrauterine repair, they were able to demonstrate significantly improved hindbrain herniation and decreased need for VP shunting compared with controls. This improvement came at a cost, however, as there were significantly higher levels of preterm delivery in the intervention group (56).Following on from the work by Bruner et al., the clinical outcomes of early studies varied. The risks associated with open MMC surgery were significant and not only included preterm labour but also placental abruption and hysterotomy scar dehiscence.
In order to determine whether the benefits of this surgery outweighed the risks, a multicentre randomized controlled trial comparing safety and efficacy of pre- versus postnatal closure of myelomeningocoele (MOMs trial) was conducted. The primary outcomes were fetal or neonatal death and the need for a VP shunt at less than 12 months of age (57). The need for VP shunting in the prenatal and postnatal surgery groups at 12 months of age were 68% and 98%, respectively (P 31 EXIT procedures undertaken predominantly for fetal neck masses and reversal of tracheal occlusion, five fetuses required a tracheostomy and there was one neonatal death due to inability to secure the airway in a case of extensive lymphangioma. The average maternal blood loss was 848 mL and there were two significant maternal complications (60). The EXIT procedure has demonstrated improvement in the outcomes of neonates with upper airway obstruction; however, it can have implications for the mother. In order to adequately coordinate the management of two patients (i.e. the mother and the fetus), a multidisciplinary team approach is essential.
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