weekly_question 22/11/2020

[Admin]

please write the answer and your justification, correct answer will be submitted after 48 hrs

You are asked to see a prenatal consult for a 26-week fetus with a thoracic mass found on screening ultrasound. The lesion is on the left and in the region of the lower lobe. The lesion is solid without obvious cysts. There is a feeding vessel from the aorta. Mediastinal shift is noted. There is no evidence of pleural effusion and the CVR is 1.2.

What is the next appropriate step in the management of this mother?

A placement of thoracoamniotic shunt

B serial fetal ultrasounds

C administer maternal steroids

D immediate fetal surgery for resection of the mass

E arrange for urgent EXIT procedure

[Admin]

Correct answer
B serial fetal ultrasounds

The terminology regarding congenital pulmonary airway malformations (CPAM), cystic adenomatoid malformation (CCAM) and pulmonary sequestration is in evolution. Historically, sequestrations were classified as those lesions with a systemic blood supply although there may be overlap with CCAM. Bronchopulmonary sequestrations are classified as intralobar or extralobar. Both consist of nonfunctional pulmonary tissue that does not directly communicate with the bronchial tree. CPAMs can achieve large size and cause mediastinal shift as well as nonimmune hydrops. With impressive mass effect, there may be pulmonary hypoplasia and hypertension resulting in acute respiratory failure upon birth.

Intralobar sequestrations are incorporated into the surrounding lung tissue. These infants usually do not have associated anomalies. They can often present later in life with pneumonia. Systemic arterial blood supply is the rule, but venous return may occur either systemically or through the pulmonary veins. These systemic vessels may be quite large, extend below the diaphragm and are characteristically thin walled. Unrecognized division of the vessel can result in retraction of a bleeding vessel below the diaphragm with a fatal result.

Extralobar sequestrations usually are covered with pleura and separated from the remaining lung. They are often found incidentally when repairing a diaphragmatic hernia or during another gastrointestinal procedure. They may be encountered above or below the diaphragm. Again, the arterial blood supply is systemic. More than 30% of these lesions will have associated anomalies including cardiac, hindgut and gastrointestinal abnormalities. Postnatally, an echocardiogram should be obtained.

Monitoring with serial fetal ultrasounds is appropriate to follow for ongoing growth. Some lesions will shrink during late gestation. Maternal steroids are considered first line therapy in a fetus with a large microcystic lesions (cyst volume ratio, CVR greater than 1.6) and hydrops or impending hydrops. Steroids appear to be most effective when administered in fetuses less than 26 weeks of gestation, but may be repeated.

Thoracoamniotic shunts are indicated for a large CPAM containing a predominant macrocyst. Fetal lung resection is reserved for pre-viable or borderline viable fetuses with hydrops who have large solid lesions unresponsive to steroids. EXIT (ex-utero intrapartum treatment) procedure is typically performed at 35 weeks’ gestation or later. However, the indications for the EXIT procedure remain controversial because of the potential maternal morbidity. The EXIT procedure has not been properly studied in comparison to standard delivery followed by emergency thoracotomy.

Most authorities recommend that the child with a fetal lung mass undergo postnatal computerized tomography (CT) or magnetic resonance imaging (MRI) to determine if the abnormal lung tissue is still present. Elective resection should be considered, even for asymptomatic lesions, due to concerns about infection or lesions that harbor or develop primary lung tumors. Both infection and primary lung malignancy have been described as early as the second year of life.

When the lung abnormality occurs in the right lower lobe, the Scimitar syndrome including anomalous pulmonary venous return to the heart should be considered.

[Shehanah]

B

[Dr Ammar]

Pulmonary sequestration
B

[matsyuk]

B

[Ben Salah Radhouene]

B and C

[Bashar]

PS

[Aorajkhan]

B

[Admin]

Correct answer
B serial fetal ultrasounds

The terminology regarding congenital pulmonary airway malformations (CPAM), cystic adenomatoid malformation (CCAM) and pulmonary sequestration is in evolution. Historically, sequestrations were classified as those lesions with a systemic blood supply although there may be overlap with CCAM. Bronchopulmonary sequestrations are classified as intralobar or extralobar. Both consist of nonfunctional pulmonary tissue that does not directly communicate with the bronchial tree. CPAMs can achieve large size and cause mediastinal shift as well as nonimmune hydrops. With impressive mass effect, there may be pulmonary hypoplasia and hypertension resulting in acute respiratory failure upon birth.

Intralobar sequestrations are incorporated into the surrounding lung tissue. These infants usually do not have associated anomalies. They can often present later in life with pneumonia. Systemic arterial blood supply is the rule, but venous return may occur either systemically or through the pulmonary veins. These systemic vessels may be quite large, extend below the diaphragm and are characteristically thin walled. Unrecognized division of the vessel can result in retraction of a bleeding vessel below the diaphragm with a fatal result.

Extralobar sequestrations usually are covered with pleura and separated from the remaining lung. They are often found incidentally when repairing a diaphragmatic hernia or during another gastrointestinal procedure. They may be encountered above or below the diaphragm. Again, the arterial blood supply is systemic. More than 30% of these lesions will have associated anomalies including cardiac, hindgut and gastrointestinal abnormalities. Postnatally, an echocardiogram should be obtained.

Monitoring with serial fetal ultrasounds is appropriate to follow for ongoing growth. Some lesions will shrink during late gestation. Maternal steroids are considered first line therapy in a fetus with a large microcystic lesions (cyst volume ratio, CVR greater than 1.6) and hydrops or impending hydrops. Steroids appear to be most effective when administered in fetuses less than 26 weeks of gestation, but may be repeated.

Thoracoamniotic shunts are indicated for a large CPAM containing a predominant macrocyst. Fetal lung resection is reserved for pre-viable or borderline viable fetuses with hydrops who have large solid lesions unresponsive to steroids. EXIT (ex-utero intrapartum treatment) procedure is typically performed at 35 weeks’ gestation or later. However, the indications for the EXIT procedure remain controversial because of the potential maternal morbidity. The EXIT procedure has not been properly studied in comparison to standard delivery followed by emergency thoracotomy.

Most authorities recommend that the child with a fetal lung mass undergo postnatal computerized tomography (CT) or magnetic resonance imaging (MRI) to determine if the abnormal lung tissue is still present. Elective resection should be considered, even for asymptomatic lesions, due to concerns about infection or lesions that harbor or develop primary lung tumors. Both infection and primary lung malignancy have been described as early as the second year of life.

When the lung abnormality occurs in the right lower lobe, the Scimitar syndrome including anomalous pulmonary venous return to the heart should be considered.

[rokia]

B