Combined Pre- and Postnatal Minimally Invasive Approach to a Complex Symptomatic Congenital Pulmonary Airway Malformation.

Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that usually remains asymptomatic during the fetal and neonatal period. However, it can occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms at birth (such as respiratory distress) and may require urgent surgical intervention. Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of gestation, a male neonate was born (weight 2,850 g). The TAS spontaneously displaced during delivery, causing an open pneumothorax (PNX), initially treated with a drainage. His condition gradually worsened, requiring ventilatory support. Computed tomography (CT) scan showed different giant cysts in the context of the right lower lobe, left mediastinal shift, and compression of the rest of the lung. An urgent surgical management was required. A thoracoscopic right lower lobectomy was performed at 10 days of life (weight 2,840 g). The postoperative course was uneventful; the child remained totally asymptomatic and showed a good recovery. To the best of our knowledge, this is the first reported case of open iatrogenic PNX following TAS positioning and the second of neonatal thoracoscopic lobectomy in a newborn weighting less than 3 kg. The purpose of this report is to indicate that minimally invasive surgery is feasible, safe, and effective for the resection of CPAM, even in small newborns.

The long-term outcome following thoraco-amniotic shunting for congenital lung malformations.

AIM OF THE STUDY: Insertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center's long-term experience. METHODS: Retrospective single center review of the period (Jan 2000-Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range). MAIN RESULTS: Thirty one fetuses underwent 37 TAS insertions at a 25 (20-30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2-10) weeks following the 1st insertion. Twenty-eight survived to be born. Sixteen (9 male) infants were delivered in our center at 39 (36-41) weeks gestational age and birth weight of 3.1 (2.6-4.2) kg. All infants underwent surgery at 2 (0-535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1-70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4-20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC - 70% predicted). CONCLUSIONS: TAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity.

Management Options for Fetal Bronchopulmonary Sequestration.

To evaluate the prenatal course and perinatal outcome of fetuses with bronchopulmonary sequestration (BPS) managed expectantly or using minimally invasive methods. This was a retrospective study of 29 fetuses with suspected BPS managed between 2010 and 2021 in three fetal medicine centers in Poland. Medline was searched to identify cases of BPS managed expectantly or through minimally-invasive methods. In 16 fetuses with BPS, there was no evidence of cardiac compromise. These fetuses were managed expectantly. Thirteen hydropic fetuses with BPS qualified for intrauterine intervention: a thoraco-amniotic shunt (TAS) was inserted in five fetuses, laser coagulation of the feeding vessel was performed in seven cases, and one fetus had combined treatment. In the combined data from the previous and the current study of various percutaneous interventions for BPS associated with hydrops, the survival rate was 91.2% (31/34) for TAS, 98.1% (53/54) for laser coagulation, and 75% (3/4) for intratumor injection of sclerosant. After taking into account cases with available data, the rate of preterm birth before 37 weeks in the group treated with laser coagulation was 14.3% (7/49) compared to 84.6% (22/26) in the group treated with TAS. The need for postnatal sequestrectomy was lower in the group of fetuses treated with laser coagulation 23.5% (12/51) in comparison to fetuses treated with TAS 84% (21/26). In fetuses with BPS without hydrops, progression of the lesion's volume, leading to cardiac compromise, is unlikely. In hydropic fetuses with BPS, intrauterine therapy using minimally invasive methods prevents fetal demise. Both, the rate of preterm birth and the need for postnatal surgery is significantly lower in the group treated with laser coagulation compared to the group treated with TAS.

In-utero treatment of prenatal thoracic abnormalities by thoraco-amniotic shunts, short and long term neuro developmental outcome: A single center experience.

BACKGROUND: Severe primary fetal hydrothorax (PFH) and fetal lung lesions (FLL) such as congenital pulmonary airway malformation (CPAM) and Bronchopulmonary sequestration (BPS) are often treated by thoraco-amniotic shunt (TAS). OBJECTIVES: To compare short and long-term outcome of fetuses treated by TAS due to FLL to those treated due to PFH. METHOD: A retrospective analysis was performed for all fetuses treated by TAS, between the years 2004-2015, evaluating the short and long term neurodevelopmental outcome. Long term neurodevelopment was additionally analyzed prospectively by Vineland adaptive behavioral scale (VABS) standardized questionnaires. RESULTS: 38 fetuses were treated by 52 TAS insertions; of which 13 (35%) due to FLL and 25 due to PFH. Perinatal survival was high (87.9%) with 3 neonatal death and one termination of pregnancy (TOP). High survival rate persisted even in cases requiring recurrent shunt insertion (80% survival). There was no significant difference in short or long term outcome including perinatal survival (84% Vs 90%, P = 0.64) and hydrops resolution (91% Vs 63%, p = 0.19). Long term outcome, including rate of neurodevelopmental abnormalities (23.5% Vs 20%) and VABS score (91.3 ± 13.3 Vs 96.4 ± 14.7), were similar for both groups. CONCLUSION: TAS insertion is effective and resulting in high perinatal survival even in cases when sequential insertion is needed. Short and long- term outcome of neonates with FLL treated by TAS are comparable to neonates treated due to PFH.

Perinatal Outcome in Fetuses with Dislodged Thoraco-Amniotic Shunts.

OBJECTIVE: Fetal thoraco-amniotic shunts (TASs) can dislodge in utero, migrating internally into the fetal thorax or externally into the amniotic cavity. Our objective was to evaluate the perinatal and long-term outcome of fetuses with TAS dislodgement and conduct a review of the literature. METHODS: This is a retrospective review of all TAS inserted for primary pleural effusions and macrocystic congenital pulmonary airway malformations (CPAMs) in a tertiary fetal medicine center (1991-2020). Antenatal history, procedural factors, and perinatal and long-term outcomes were reviewed in all fetuses with dislodged shunts and compared to fetuses with shunts that did not dislodge. RESULTS: Of 211 TAS inserted at a mean gestational age of 27.8 weeks ± 5.47 (17.4-38.1 weeks), 187 (89%) were inserted for pleural effusions and 24 (11%) for macrocystic CPAMs. Shunts dislodged in 18 fetuses (8.5%), 17 (94%) of which were for pleural effusions. Shunts migrated into the chest wall/amniotic cavity or into the thorax among 7/18 (39%) and 11/18 (61%) fetuses, respectively. Eleven (61%) fetuses were initially hydropic, which resolved in 8 (72%) cases. Effusions were bilateral in 9 (50%), amnioreduction was required in 6 (33%), and fetal rotation in 8 cases (44%). Four (22%) fetuses underwent repeat shunting, 12 (67%) neonates required ventilatory support, and 2 (11%) neonates required chest tubes. There was no significant difference in technical factors or outcomes between infants with shunts that dislodged and those that did not. Among 11 intrathoracic shunts, 2 (18%) were removed postnatally and the remainder are in situ without any shunt-related or respiratory complications over a follow-up period of 9 months to 22 years. CONCLUSION: TAS dislodged antenatally in 8.5% of fetuses, with 2/3 of shunts migrating into the thorax, and nearly 25% requiring re-shunting. Retained intrathoracic shunts were well tolerated and may not necessarily require surgical removal after birth.

Prenatal diagnosis of mediastinal neurenteric cyst: a case report and review of the literature.

Neurenteric cyst is a very rare developmental anomaly. Prenatal diagnosis of mediastinal neurenteric cysts has been reported rarely. We present a case of neurenteric cyst associated with vertebral anomalies diagnosed by prenatal ultrasonography at 31 weeks of gestation, which was treated successfully in the early neonatal period. In addition, we searched the English literature for all cases of mediastinal neurenteric cyst diagnosed in the prenatal period reported to date. We found that only 17 cases were reported previously. We reviewed the reports of these 17 patients along with our case, and we investigated the prenatal and postnatal diagnosis and treatment approaches and the factors influencing the prognosis. Fetuses with mediastinal neurenteric cysts should be monitored regularly by ultrasonography. Fetuses with no signs of hydrops are more likely to survive with proper neonatal center transfer, regular follow-up, and appropriate postnatal approach. Fetuses with hydrops findings have a high risk of fetal and neonatal death.

Successful in utero Percutaneous Fetoscopic Release of a Wrapped Pleuro-Amniotic Shunt around the Fetal Arm: Case Report and Review of the Literature.

BACKGROUND AND OBJECTIVES: Fetal pleural effusions are either primary or secondary to chromosomal or structural fetal abnormalities. In both cases, thoraco-amniotic shunts may be indicated to prevent life-threatening consequences. In this report, we describe a rare complication of shunts and successful in utero management of this complication. We also summarize the prior reports of shunt wrapping around fetal limbs and successful prenatal release of the shunts. DATA SOURCE: Search including PubMed, MEDLINE and EMBASE databases from inception till November 2015 was conducted for cases with shunt entrapment around fetal limbs with or without prenatal management. RESULTS: Four cases including our case have been reported in the literature with wrapping of shunts around fetal limbs. Three of these cases were successfully managed prenatally using different interventional and anesthetic techniques. CONCLUSIONS: Prenatal shunts could be rarely complicated by wrapping of the shunt around fetal limbs with possible serious vascular and neurological consequences. Different techniques have been described for prenatal management of this condition. We describe a less invasive approach that could be done under local anesthesia in suitable cases.