**6.6 Sacrococcygeal teratoma (SCT)**

Although the mortality rate is 5% for SCT diagnosed in the newborn, the mortality rate is about 50% for fetal SCT. Rupture of the tumor, or hemorrhage inside, or high output heart failure, and premature labor, form the main causes of fetal loss. Every attempt at interventions, to prevent this high prenatal mortality are the target of study of several fetal centers. Resection of the tumor (in utero) should be consider for treatment of pre-mature patients with early signs of heart failure or placentomegaly.


It is important to note the related morbidity of all mentioned procedures, like risk of preterm delivery, beside procedure failure rate. The related morbidity can be reduced with minimally invasive procedures, however, the fear from decreased efficacy in local control, still a significant problem. Moreover, these less invasive

**15**

*Principles of Fetal Surgery*

**Figure 4.**

and fetal hydrops [78].

**6.7 Cystic pulmonary airway malformations (CPAM)**

non-surgical options have been reported.

Most prenatally detected lung lesions are cystic pulmonary airway malformations (CPAM), broncho-pulmonar sequestrations or so called 'hybrid' lesions, containing features of both. The outcome of most lesions are favorable even without pre-natal intervention, despite often impressive appearance at mid-gestation. During pregnancy many lesions may regress, or disappear completely. Therefore, non-operative treatment (watchful waiting) is preferred by most fetal surgeons. Surprisingly, pressure effect or hemo-dynamic changes may cause sudden physiologic derangements, which may end with progressive heart failure and intrauterine demise. Therefore, pre-natal intervention may be warranted to improve outcome. Pre-natal interventions for fetal lung lesions aim to alleviate the pressure effect of the mass by partial or complete removal of the lesion. Many surgical and

• In macrocystic lesions, needle thoraco-centesis or thoraco-amniotic shunt drain-

age under ultrasound guided may be used for decompression.

*DOI: http://dx.doi.org/10.5772/intechopen.85883*

procedures should be used as early as possible, preferably before early signs of heart failure, to prevent IUFD. Certainly the best option is complete tumor resection, if possible. A systematic review on 34 cases of SCT from 1980 to 2013, using minimally invasive fetal procedures, they reported 44% (14/32) overall survival with 29.7 ± 4.0 weeks, as mean gestational delivery age. They considered heart failure as a bad prognostic indicator, (30% survival rate (6/20)). Another review compares two minimally invasive procedures, direct tumor control, and vascular occlusion of the tumor feeding vessels. Study included 33 cases, 11 cases submitted to vascular occlusion (group A) and 22 submitted to direct tumor control (group B). They reported 63.6% (7/11) survival in vascular ablation (group A) compared to 40.9% (9/22) in direct tumor control (group B). They claimed that reduction of the tumor blood supply slowly appears safer than rapid tumor necrosis which may lead to hemorrhage inside the tumor. Regarding outcomes of fetuses with large SCTs and fetal hydrops before viability, due to rarity of the tumor the available small case series suggest that fetal intervention does confer a survival advantage. However, randomized trials are needed to make a valuable conclusion, also long-term outcomes data are needed. Because these procedures are associated with significant risks, so to get the best benefit these patients must be performed only in specialized centers, and must be limited to cases presented with both high-output heart failure

*Some amniotic bands may constrict fetal limbs, and may be serious to cause limb amputation.*

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

Amniotic band syndrome can lead to fetal death from umbilical cord strangulation and/or congenital limb deformity or loss, presumed to result from ischemia caused by constriction bands that interfere with vascular perfusion. There is increasing experience with intrauterine release of congenital constrictions and evidence is mounting that this therapy may help save/restore some limb function and morphology. The location of the bands and timing of fetal damage will affect the presentation, severity, and outcome of the condition. For example, pseudosyndactyly or limb amputation can be the results of constriction bands at the extremities, whereas more midline bands can result in craniofacial, thoracic, or abdominal defects, and may be fatal. The etiology of this syndrome is unknown, and theories range from a genetic basis or early disruption of the germinal disc to traumatic disruption of the membranes later in fetal development. Fetoscopic release of amniotic bands using minimally invasive surgery, can help in preservation of life and or limb saving in cases of ABS. The present acceptable functional outcome in 50% of cases is promising, although, clear selection criteria are needed to justify the risk of this in-utero invasive procedure, through increased experience and larger studies on this

In cases of extremity involvement by amniotic band syndrome, the band must be released using fetoscope to save the normal development of the limb and allow for normal limb function. Ultrasound imaging can easily diagnose the problem, showing; distal limb edema and interrupted blood flow by Doppler, with or without visualization of the constricting band. Although, the available reports include small case series, its results suggest that fetuses must have distal arterial limb flow detected by Doppler in order to benefit from intervention. Moreover, data from recent studies reported that fetuses with single limb involvement tend to do better than those with multiple involved limbs. Surprising, the incidence of PROM with this procedure seem to be higher than for other fetoscopic procedures, (reported rates up to 78%). Although, small number of cases were reported in all available studies, and considering the learning curve in this studies, it could also be related to

Although the mortality rate is 5% for SCT diagnosed in the newborn, the mortality rate is about 50% for fetal SCT. Rupture of the tumor, or hemorrhage inside, or high output heart failure, and premature labor, form the main causes of fetal loss. Every attempt at interventions, to prevent this high prenatal mortality are the target of study of several fetal centers. Resection of the tumor (in utero) should be consider for treatment of pre-mature patients with early signs of heart failure or

• Removal of the external part of the tumor is usually preferred, followed by

• Alternatively, radio-frequency, or thermal ablation can be used to occlude

It is important to note the related morbidity of all mentioned procedures, like risk of preterm delivery, beside procedure failure rate. The related morbidity can be reduced with minimally invasive procedures, however, the fear from decreased efficacy in local control, still a significant problem. Moreover, these less invasive

**6.5 Amniotic band syndrome (ABS)**

type of therapy for ABS (**Figure 4**).

**6.6 Sacrococcygeal teratoma (SCT)**

later removal of its pelvic extension.

supplying arteries to the tumor.

inherent membrane problems in these fetuses [76, 77].

**14**

placentomegaly.

**Figure 4.** *Some amniotic bands may constrict fetal limbs, and may be serious to cause limb amputation.*

procedures should be used as early as possible, preferably before early signs of heart failure, to prevent IUFD. Certainly the best option is complete tumor resection, if possible. A systematic review on 34 cases of SCT from 1980 to 2013, using minimally invasive fetal procedures, they reported 44% (14/32) overall survival with 29.7 ± 4.0 weeks, as mean gestational delivery age. They considered heart failure as a bad prognostic indicator, (30% survival rate (6/20)). Another review compares two minimally invasive procedures, direct tumor control, and vascular occlusion of the tumor feeding vessels. Study included 33 cases, 11 cases submitted to vascular occlusion (group A) and 22 submitted to direct tumor control (group B). They reported 63.6% (7/11) survival in vascular ablation (group A) compared to 40.9% (9/22) in direct tumor control (group B). They claimed that reduction of the tumor blood supply slowly appears safer than rapid tumor necrosis which may lead to hemorrhage inside the tumor. Regarding outcomes of fetuses with large SCTs and fetal hydrops before viability, due to rarity of the tumor the available small case series suggest that fetal intervention does confer a survival advantage. However, randomized trials are needed to make a valuable conclusion, also long-term outcomes data are needed. Because these procedures are associated with significant risks, so to get the best benefit these patients must be performed only in specialized centers, and must be limited to cases presented with both high-output heart failure and fetal hydrops [78].
