Left lung cystadenoma

Our lungs are very important respiratory organs in the body. Whether the lungs develop normally directly affects whether we can survive. The development of the lungs is also an important diagnostic indicator for the newborn. Once a lung disease occurs, it will have more serious consequences. The most typical one is pulmonary cystadenoma. The lungs exist in pairs. This disease can appear on both sides or on one side. Left lung cystadenoma is one of these cases and is very harmful.

Indications for hormone therapy for CCAM: 1. CCAM cases that are of high-risk microcystic type; 2. Fetal edema; 3. CVR>1.6. The possible mechanism of hormone therapy is to solve the problem of immature lungs in CCAM cases: in the study of CCAM cases, it was found that the expression of Hox b5 gene was similar to that of early lung tissue; CD34 staining examination of lung tissue was also similar to that of early lung tissue, indicating that CCAM has congenital deficiencies in lung development. Curran PF et al. applied antenatal hormone therapy to 16 cases of microcystic CCAM. Among them, 13 cases were born alive, and 11/13 (84.6%) survived until discharge. During the treatment, the CVR of all cases was greater than 1.6, and 9 cases (69.2%) also had non-immune edema. Among the cases treated with hormones, the CVR decreased in 8 cases (61.5%), the edema was relieved in 7 cases (77.8%), and the edema was not relieved in 2 cases. Conclusions: Hormonal therapy is an effective approach in high-risk microcystic CCAM cases.

Shunt puncture The drainage of cysts (shunt puncture) first requires a visual system that can keep track of the specific situation of the mass and puncture at any time. Using a fetoscope and under the guidance of a visual system, the drainage tube is placed between the thoracic cyst and the amniotic cavity to achieve the treatment goal. Assessment of shunt status: Use a visual system to understand the lung's postoperative re-expansion status and possibly discover previously undiagnosed pulmonary sequestration to prevent further progression of edema. The drainage fluid can be used for relevant laboratory examinations: 1. Cytological examination to determine whether there is lymphatic exudation; 2. Infection indicators; 3. Fetal karyotype examination. The average gestational age for prenatal diagnosis of CCAM by Wilson et al. was 20 weeks, and the average gestational age for catheterization was 23+1 weeks. The average volume of the CCAM mass before surgery was 50.5-25.7 cm3; the average volume decreased by 51% after surgery. The average gestational age at birth of CCAM patients after this treatment was 33+3 weeks, and the average catheterization time was 10+2 weeks. The survival rate after birth is 70%. Indications for treatment include fetal hydrops and signs of pulmonary hypoplasia. The successful catheterization significantly prolonged the baby's gestational age at birth. Clinical summary by Schott S et al. suggests that thoracic shunt surgery can achieve a survival rate of 70% in cases of giant alveolar type. Mark et al. [22] performed shunt puncture treatment for 40 cases of CCAM from 1998 to 2009, of which 75% (30 cases) survived. (III) EXIT surgery (ex-utero intrapartum therapy, EXIT) EXIT surgery is a procedure in which the fetus is delivered by cesarean section without cutting the umbilicus. The CCAM tumor is removed first, and then the umbilicus is cut to allow the newborn to start breathing, in order to reduce the pressure of the tumor on the chest cavity and relieve respiratory distress. Principles of EXIT surgery: 1. Severe mediastinal shift; 2. Continuously increased CVR value (>1.6) combined with significant compression of normal lung tissue; 3. Combined with fetal edema. EXIT surgery requires careful planning and full teamwork, including anesthesia, cardiopulmonary bypass specialists, neonatology, nursing, obstetrics, pediatric surgery, and extracorporeal membrane oxygenation (ECMO) support. The survival rate of EXIT surgery for lung tumor resection is 86-90%, and the operation time is 27-100 minutes, with an average of 64 minutes. Possible risks after birth include: recurrence, airway fistula, bleeding, chylothorax, sepsis, gastroesophageal reflux, etc. The necessary factors for the success of EXIT surgery are to ensure uteroplacental gas exchange and fetal hemodynamic stability. In addition, the family must be informed of potential risks, including bleeding in the mother, the need for further lung tissue resection after birth, longer NICU monitoring and treatment, and the corresponding increased costs. Risks to the mother include polyhydramnios, preterm birth, chorioamnionitis, and hemorrhage. The EXIT procedure allows for rapid removal of lung masses after birth, eliminating acute respiratory failure caused by mediastinal shift, air trapping, and compression of normal lung tissue.

Open fetal surgery The principles or goals of fetal surgery are: 1. Restore normal anatomy; 2. Restore normal physiology; 3. Allow the lungs to grow and develop before birth. There is no clear indication for open fetal surgery during the fetal period. For CCAM with no symptoms or edema, CVR1.6, obvious compression or obvious mediastinal shift, tendency to edema or existing edema, and polyhydramnios, intervention during the fetal period is often required, including open fetal surgery. Mark counted the outcomes of 13 fetal surgeries with gestational ages ranging from 21 to 29 weeks, of which 8 survived (62%) and 5 died. The outcomes for the surviving cases were summarized as follows: edema resolved within 1-2 weeks; mediastinal position restored within 3 weeks; lung growth accelerated; and delivery occurred after an average of 8 weeks. Postnatal surgical options CCAM is less likely to be associated with other congenital malformations. Most cases can be delivered vaginally and treated surgically after birth. Early surgical resection has become a generally accepted view. However, there is also a view that no further treatment is needed after birth (accounting for 18% of the total prenatal diagnoses). Lllanes S et al. summarized that in cases where CCAM masses were discovered prenatally by ultrasound and disappeared before delivery, postpartum CT examination revealed that 64% of them were still abnormal, and 67% were consistent with the postoperative pathological histological diagnosis; this suggests that relying solely on ultrasound imaging for diagnosis and surgical treatment planning is clearly insufficient. Therefore, if CCAM is diagnosed in the fetus before birth, another CT scan is required after birth to confirm the diagnosis. Those with clear symptoms after birth require emergency surgical treatment; there is no clear standard for when to perform surgery on asymptomatic patients. Adzick NS pointed out that surgery should be performed at least one month after birth because the risk of anesthesia begins to gradually decrease when the baby is 4 weeks old. Factors related to the need for surgical resection include: the appearance of obvious respiratory symptoms, repeated infections and the risk of malignant transformation of the tumor; other clinical manifestations such as hemoptysis, hemothorax, etc. However, some people believe that it is best not to wait until symptoms appear before undergoing surgery, as this may affect the overall development of the lungs. However, if the surgery is performed before complications occur, there is no obvious correlation between the long-term effects of the surgery and the timing of the surgery.

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