Secuestro pulmonar: a propósito de un caso / Pulmonary sequestration: a case report

Resumen Introducción El secuestro pulmonar es un segmento anormal de tejido pulmonar sin comunicación con el árbol traqueobronquial e irrigado por una arteria aberrante de origen sistémico. El secuestro pulmonar puede ser intralobar o extralobar. Caso Clínico Presentamos el caso de un neonato con dificultad respiratoria debido a secuestro pulmonar. El tratamiento quirúrgico consiste en la resección del segmento pulmonar con la ligadura y corte de su arteria. Se describe el manejo realizado y la revisión de la literatura indexada.

Introduction Pulmonary sequestration is an abnormal segment of lung tissue without communication with the tracheobronchial tree and irrigated by an aberrant artery of systemic origin. Can be intralobar or extralobar. Case report We present the case of a neonate with respiratory distress due to pulmonary sequestration. Surgical treatment consists in the resection of the lung segment with the ligature and cut of its artery. Is describe the management carried out and the review of the indexed literature.

Prenatal sonographic markers of the outcome in fetuses with bronchopulmonary sequestration.

PURPOSE: To evaluate the prenatal sonographic predictive markers of the outcome in fetuses with bronchopulmonary sequestration (BPS). METHODS: BPS size and diameter of the feeding artery (FA) were measured prenatally and postnatally. Velocity of the FA and the left ventricular-modified myocardial performance index (LV mod-MPI) were also evaluated prenatally. RESULTS: Forty-seven women were included in the study. Mean gestational age, mass size, diameter and velocity of the FA, and LV mod-MPI at prenatal diagnosis were 23.5 ± 2.2 weeks, 3.6 ± 8.3 cm, 2.3 ± 0.6 mm, 46.6 ± 15.4 cm/s, and 0.46 ± 0.06, respectively. Mean mass diameter and FA diameter measured on postnatal CT examinations were 3.8 ± 1.0 cm and 2.3 ± 0.7 mm, respectively. Five patients had respiratory symptoms after birth. Twenty children (43%) underwent or were scheduled to undergo mass excision, and the remaining 27 (57%) were doing well without any intervention. There was no neonatal death. LV mod-MPI at diagnosis, the FA diameter after birth and the serial change in the FA size were significantly associated with postnatal mass excision. CONCLUSION: The FA diameter and LV mod-MPI may be additional markers for predicting whether fetuses with BPS should undergo mass excision in early childhood or conservative care.

Going With the Flow: An Aid in Detecting and Differentiating Bronchopulmonary Sequestrations and Hybrid Lesions.

OBJECTIVES: To assess the ability of prenatal ultrasound (US) in identifying systemic feeding arteries in bronchopulmonary sequestrations and hybrid lesions and report the ability of US in classifying bronchopulmonary sequestrations as intralobar or extralobar. METHODS: Institutional Review Board-approved radiology and clinical database searches from 2008 to 2015 were performed for prenatal lung lesions with final diagnoses of bronchopulmonary sequestrations or hybrid lesions. All patients had detailed US examinations, and most patients had ultrafast magnetic resonance imaging (MRI). Lesion location, size, and identification of systemic feeding arteries and draining veins were assessed with US. RESULTS: The study consisted of 102 bronchopulmonary sequestrations and 86 hybrid lesions. The median maternal age was 30 years. The median gestational age was 22 weeks 5 days. Of bronchopulmonary sequestrations, 66 had surgical pathologic confirmation, and 100 had postnatal imaging. Bronchopulmonary sequestration locations were intrathoracic (n = 77), intra-abdominal (n = 19), and transdiaphragmatic (n = 6). Of hybrid lesions, 84 had surgical pathologic confirmation, and 83 had postnatal imaging. Hybrid lesion locations were intrathoracic (n = 84) and transdiaphragmatic (n = 2). Ultrasound correctly identified systemic feeding arteries in 86 of 102 bronchopulmonary sequestrations and 79 of 86 hybrid lesions. Of patients who underwent MRI, systemic feeding arteries were reported in 62 of 92 bronchopulmonary sequestrations and 56 of 81 hybrid lesions. Ultrasound identified more systemic feeding arteries than MRI in both bronchopulmonary sequestrations and hybrid lesions (P < .01). Magnetic resonance imaging identified systemic feeding arteries that US did not in only 2 cases. In cases in which both systemic feeding arteries and draining veins were identified, US could correctly predict intrathoracic lesions as intralobar or extralobar in 44 of 49 bronchopulmonary sequestrations and 68 of 73 hybrid lesions. CONCLUSIONS: Ultrasound is most accurate for systemic feeding artery detection in bronchopulmonary sequestrations and hybrid lesions and can also type the lesions as intralobar or extralobar when draining veins are evaluated.

Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations.

PURPOSE: The prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention. METHODS: A retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n=44) or extralobar BPS (n=59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management. RESULTS: Most extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26-28weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth. CONCLUSIONS: BPSs tend to decrease in size after 26-28weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax. LEVEL OF EVIDENCE: IV.

Imaging and differential diagnosis of suprarenal masses in the fetus.

Prenatal sonography and magnetic resonance imaging of suprarenal fetal masses is presented, along with clinical information and follow-up. Imaging pearls and differential considerations for each diagnosis will be discussed. Fetal suprarenal mass diagnoses include neuroblastoma, extralobar pulmonary sequestration, congenital adrenal hyperplasia, partial multicystic dysplastic kidney, renal duplication, urinoma, gastric duplication cyst, and splenic cyst. Recognizing the range of malignant and benign suprarenal fetal masses that can present on prenatal imaging can help guide patient counseling and management.

Fetal and placental vascular tumors: persistent fetal hyperdynamic status predisposes to poorer long-term neurodevelopmental outcome.

OBJECTIVE: To evaluate the association between fetal hemodynamic changes seen in the presence of vascular tumors of fetal or placental origin and risk of adverse pregnancy outcome. METHODS: All cases of placental chorioangioma, sacrococcygeal teratoma and pulmonary sequestration during a 10-year period were included. Ultrasound data and pregnancy and long-term neurodevelopmental outcomes were assessed in this cohort. A survival analysis was performed to assess the relationship between the cardiovascular profile score (CVPS) and adverse pregnancy outcome. RESULTS: There were 56 fetal or placental tumors, including 28 chorioangiomas, 10 sacrococcygeal teratomas and 18 pulmonary sequestrations, diagnosed at a median gestation of 23 + 3 weeks. Abnormal CVPS (≤ 8) was seen in 30% of sacrococcygeal teratomas and in 46% of chorioangiomas, but in none of the pulmonary sequestration cases. Adverse pregnancy outcome occurred in 11 cases (three stillbirths, three neonatal deaths and five cases of developmental delay) and only in those cases in which the tumors were associated with a CVPS of ≤ 8. CONCLUSIONS: Certain fetal and placental vascular tumors are associated with cardiac dysfunction in fetal life. When the CVPS is low (≤ 8), these cases are at increased risk of both fetal/neonatal demise as well as overt long-term neurodevelopmental disability. The long-term neurodevelopmental outcome should be formally and prospectively assessed in cases of fetal and placental vascular tumors.

Management of prenatally diagnosed lung lesions.

Prenatal diagnosis provides insight into the in utero evolution of fetal thoracic lesions such as congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration (BPS), or hybrid lesions. Serial sonographic study of fetuses with thoracic lesions has helped define the natural history of these lesions, determine the pathophysiologic features that affect clinical outcome, and formulate in utero and postnatal management based on prognosis.

The diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: a report of 4 cases.

BACKGROUND/PURPOSE: Intradiaphragmatic extralobar pulmonary sequestrations (IDEPSs) are a rare subset of bronchopulmonary sequestrations (BPS). We report the largest series of patients with IDEPS and describe the diagnostic and operative challenges associated with this condition. METHODS: We retrospectively reviewed our experience with fetal and pediatric BPS from 1995 to 2010 to identify patients with IDEPS. RESULTS: We identified 27 patients with BPS and 4 patients in whom the masses were within the diaphragm. In 1 patient, the prenatal ultrasound correctly identified the mass as being within the diaphragm itself, whereas the remaining cases were thought to be intraabdominal or had discordant preoperative imaging findings. The diagnosis of an IDEPS proved challenging to make prospectively using prenatal ultrasound, computed tomography, or magnetic resonance imaging. All patients underwent attempted resection. Two cases required a combined laparoscopic and thoracoscopic approach to accurately localize the mass. The postoperative recovery of these patients was uneventful. CONCLUSIONS: We present the largest reported experience of IDEPS. Because preoperative imaging studies cannot always determine whether a sequestration is intraabdominal, intrathoracic, or intradiaphragmatic, operative planning may pose a challenge. However, the use of minimally invasive approaches can allow exploration of both the thoracic and abdominal cavities with low morbidity.

Intradiaphragmatic pulmonary sequestration: advantages of the thoracoscopic approach.

Pulmonary sequestrations are accessory foregut lesions that are most commonly located within the thorax and occasionally in the abdominal cavity. Sequestrations arising within the diaphragm are exceedingly rare. We describe 2 patients found to have left peridiaphragmatic lesions on prenatal ultrasound and postnatal computed tomography. In the first patient, an initial laparoscopic approach was abandoned in favor of a thoracoscopic approach after no intraabdominal mass was found. The second patient had an uncomplicated thoracoscopic resection of a similar lesion. To our knowledge, these represent the first intradiaphragmatic pulmonary sequestrations to be resected via a minimally invasive approach. The rarity of these lesions makes definitive diagnosis without operative intervention challenging. Thoracoscopy appears to be a reasonable approach for resection of such intradiaphragmatic lesions.

Intradiaphragmatic hybrid lesion in an infant: case report.

Hybrid lesions have elements of both congenital cystic adenomatoid malformation and bronchopulmonary sequestration. We report an unusual case of an infant treated for an intradiaphragmatic hybrid lesion. Although computed tomography remains the criterion standard imaging examination for planning the operation, the exact localization of such lesions may be discovered only at surgical exploration.

Protective effect of fetal pulmonary sequestration in two cases of postnatal manifestation of congenital diaphragmatic hernia.

We describe two cases of postnatally diagnosed congenital diaphragmatic hernia (CDH) combined with pulmonary sequestration, both of which were diagnosed as isolated pulmonary sequestration on prenatal ultrasound. In these cases, prenatal ultrasonography demonstrated only a hyperechoic mass on the left lower lung and the diaphragm seemed intact. In each case both lungs showed otherwise normal development throughout pregnancy. Pulmonary sequestration may serve as a 'protector', preventing herniation of abdominal contents into the thoracic cavity. The co-occurrence of CDH may be obscured by a lung mass, especially on the left lower lung, and therefore it is necessary to deliver these infants at a tertiary center and parents should be counseled about the possibility of postnatal CDH.

Grayscale and Doppler sonographic evaluation of response to in utero treatment of hydrops fetalis caused by extralobar pulmonary sequestration.

Pulmonary sequestration is defined as nonfunctional lung tissue that lacks communication with the bronchial tree and that is supplied by an anomalous systemic vessel. In comparatively rare cases, pulmonary sequestration may lead to hydrothorax or hydrops fetalis, which is nearly universally fatal. In this report, we describe a case of pulmonary sequestration with hydrops fetalis, which was successfully treated by thoracoamniotic shunting. A sonographic Doppler study in this case suggested that the underlying mechanism of the hydropic change in a fetus with extralobar pulmonary sequestration may have differed from that in fetuses with primary hydrothorax not associated with a structural anomaly.

Embriología del desarrollo de los bronquios y el parénquima pulmonar / Embryology of development of the bronchi and lung parenchyma

Las malformaciones congénitas de aparato respiratorio son un extenso número de patologías que oscilan en gravedad dependiendo del momento embriológico en que se dio el fallo y cuales mecanismos fueron afectados. En la revisión se profundiza en patologías congénitas como el secuestro broncopulmonar y la malformación adenomatoide quística...

Prenatal findings and differential diagnosis of scimitar syndrome and pulmonary sequestration.

OBJECTIVES: Scimitar syndrome and pulmonary sequestration (PS) have overlapping features. This accounts for postnatal descriptions of a 'sequestrated lung' in scimitar-syndrome patients. We review the similarities and differences in these two conditions, and the antenatal ultrasound findings that allow correct prenatal identification and differential diagnosis. METHODS: This was a retrospective analysis of prenatally diagnosed cases of scimitar syndrome or PS that underwent fetal echocardiography between January 1995 and November 2004. RESULTS: There were five cases of PS and six of scimitar syndrome. Right-sided mediastinal shift (ipsilateral relative to the affected lung) was the commonest indication for referral in scimitar syndrome whereas in sequestration, referral was because of hyperechogenic lung and contralateral mediastinal shift. Lung echogenicity was normal in scimitar syndrome but focally increased in PS. Abnormal, unobstructed pulmonary venous drainage was identified prenatally in four cases of scimitar syndrome. It was normal in three fetuses with sequestration but abnormal and obstructed in the other two cases complicated by hydrothorax. Abnormal systemic arterial supply to the affected lung was easily demonstrated in all fetuses with PS, but could not be shown prenatally in scimitar-syndrome fetuses. Postnatal embolization was warranted in one case with PS and four with scimitar syndrome, one each in the neonatal period. CONCLUSION: Prenatally, scimitar syndrome and PS are clearly distinct entities based on lung echogenicity and laterality of mediastinal shift. Hyperechogenic lung and dextrocardia indicate the possibility of PS and scimitar syndrome, respectively. Complete delineation of the vascular pattern should be attempted in all, but is more challenging in scimitar syndrome. Obstructed venous return in PS may identify fetuses at risk of developing hydrothorax.

Single-needle laser treatment with drainage of hydrothorax in fetal bronchopulmonary sequestration with hydrops.

Bronchopulmonary sequestration (BPS) is sometimes associated with hydrothorax and hydrops in utero. In the absence of fetal hydrops, perinatal outcome is favorable and justifies expectant management. In the presence of fetal hydrops, perinatal outcome is reported to be extremely poor and intervention should be considered. Therapeutic options include open fetal surgery, minimally invasive coagulation of the blood supply and thoracoamniotic shunting. We present the first case of fetal hydrops and a large hydrothorax due to BPS treated successfully with one ultrasound-guided thin needle insertion, through which both laser coagulation of the feeding artery and drainage of the hydrothorax were performed. Following the procedure the hydrothorax and hydrops gradually disappeared and the BPS diminished in size. A healthy neonate was delivered uneventfully at term. We describe the case and discuss the different therapeutic options.

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls.

BACKGROUND: Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. METHODS: Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors' institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. RESULTS: In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. CONCLUSIONS: Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.

Fetal pleuroamniotic shunting for bronchopulmonary sequestration with hydrops.

Bronchopulmonary sequestration (BPS), a non-functional pulmonary tissue mass, when complicated by fetal hydrops, carries a high risk of perinatal mortality. However, a limited number of cases of BPS complicated by fetal hydrops with an informative clinical course have been reported. We report here on three cases of BPS complicated by fetal hydrothorax and hydrops that were successfully treated by pleuroamniotic shunting, which should be considered as a treatment option for fetal hydrothorax and hydrops associated with BPS.