© 1990 S. Karger AG. Basel 1015-3837/90/0051-0033S2.75/0
Fetal Diagn Ther 1990;5:33-39
Antenatal Diagnosis and Treatment of Fetal Bronchopulmonary Sequestration R.N. Slotnicka , John McGahan'0, L. Milio a , M. Schwartzc, Deborah Ablinh Departments o f aObstetrics and Gynecology, bRadiology, and 'Pediatric Surgery, Division o f Prenatal Diagnosis and Genetics, University o f California, Davis School of Medicine, Sacramento, Calif., USA
Key Words. Ultrasound, thorax • Ultrasound, fetus • Fetal treatment
Introduction Bronchopulmonary sequestration is a rare congenital anomaly that has been diag nosed by prenatal ultrasound [1, 2], The association with large hydrothorax is rare [3-6], We report a case of a prenatally diag nosed hydrothorax, secondary to broncho pulmonary sequestration, successfully man aged by use of prenatal aspiration and drain age of the hydrothorax.
Case Report A 25-year-old gravida 2 para 1001 woman was referred at approximately 31 menstrual weeks o f ges tation for the evaluation o f polyhydramnios and non immune hydrops. Prior family and obstetric history was unremarkable with normal obstetrical ultrasound
at approximately 20 weeks o f gestation to corroborate dates. Upon transfer to our facility, an ultrasound revealed a single live intrauterine pregnancy o f ap proximately 32 weeks with gross polyhydramnios. There was a large left hydrothorax with dramatic mass effect incuding right shift o f the heart, severe compression o f pulmonary structures and downward displacement o f the diaphragm (fig. 1). There was some increased echogenicity o f portions o f the left lung. Mild abdominal ascites and bilateral hydrocele were appreciated. The remainder o f the ultrasound examination was normal. Amniocentesis was per formed upon arrival and revealed a lecithimsphingomyclin ratio of 1.4 with percent saturation o f lecithin o f 58%, negative phosphatidylglycerol and a 53% probalility o f newborn distress based on these val ues. Fetal thoracentesis was chosen as an option be cause of the massive fetal hydrothorax, significant mass effect and resultant fetal hydrops. On hospital day 3, ultrasound-guided thoracic placement of the 22-gauge needle was accomplished without difficulty; the intrathoracic fluid was under marked pressure
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Abstract. Bronchopulmonary sequestration with associated nonimmune hydrops has been previously reported with generally poor prognosis for the neonate. We report a case of bronchopulmonary sequestration and associated pleural effusion successfully managed with a transthoracic catheter placement. The embryology and clinical pathophysiology of bron chopulmonary sequestration are discussed.
34
Slotnick/McGahan/Milio/Schwartz/Ablin
(fig. 2). A total of 126 cm3 o f clear, yellow fluid was removed without difficulty. Postthoracentesis ultra sound showed a largely normal appearance of the fetal thorax except for a small left pleural effusion. Because of increased uterine activity, the patient was placed on prophylactic magnesium sulfate as a tocolytic. The patient was closely observed with daily ultrasound and there was gradual reaccumulation of pleural fluid. By hospital day 5, fluid reaccumulation had progressed to the point where thoracic ultrasound studies could not be distinguished from prethoracen tesis evaluations. Therefore, it was decided to redrain the pleural fluid. On day 7. using aseptic technique and under ultrasound guidance, a 5-French Harrison
double pigtail catheter (Cook Catheter. Bloomington, Ind.) was placed into the fetal thorax (fig. 3). At con clusion of the procedure, the distal tip of the catheter was in the left fetal thorax and the proximal curl within the amniotic cavity (fig. 3). A percutaneous umbilical cord sampling was also performed showing a normal male karyotype, 46,XY. A fetal hematocrit from blood sampling was measured to be 38%, Torch studies proved negative. The procedure was per formed without complications. Over the next few days repeat ultrasound exams showed that the thoracic catheter position was un changed and remarkably minimal thoracic fluid present (fig. 4). The left-to-right mediastinal shift
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 1. Transverse ultrasound (a) and accompanying longitudinal ultrasound (b) of the fetal thorax demonstrating large fluid collec tion (F) which displaces the heart (curved arrow) into the right hemithorax and displaces the diaphragm caudally (open arrow). The left lung is identified (arrows) within the fluid collection.
Antenatal Diagnosis and Treatment o f Fetal Bronchopulmonary Sequestration
35
Fig. 3. a Three days later, the fluid had nearly completely reaccu mulated and a double ‘J’ stent was placed through the anterior thorax (arrow) into the fluid collection (F). b Part of the catheter is coiled within the fluid collection (arrow) and the other part external to the fetal thorax (arrow). F = Fluid.
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 2. Transverse sonogram of the fetal thorax demonstrating the 20-gauge needle placed using a biopsy guide attachment through the anterior thorax into the fluid collection. The echogenic needle tip is well identified (curved arrow). The fluid was completely aspi rated.
36
Slotnick/McGahan/Milio/Schwartz/Ablin
hematocrit 43%. A venous umbilical blood gas was obtained: pH = 7.32. PCF of 21. PCO: of 20. The intrathoracic catheter was still in place and at the time o f delivery was draining fluid through and around the thoracic tubal aperture (fig. 5). Examina tion o f the tube after delivery revealed the lumen to be completely patent. The neonate required only minimal respiratory' support and was extubated on day 1 after delivery. However, pleural fluid continued to be drained through a chest tube for 1.5 weeks. The fluid re mained clear even after feedings were begun. A CT scan of the chest revealed an inhomogeneous mass of the base of the left lung, consistent with a pulmonary sequestration. The neonate was taken to the operating room on day 18 after delivery and a 3 X 4 X 4 cm pulmonary sequestration was removed without diffi culty. This mass was separate from the left lower lobe o f lung, was lined with pleura and was supplied by a transdiaphragmatic feeding artery and draining vein. Subsequent to this procedure the neonate did well and was discharged on postoperative day 5. The neo nate is now 4 months old and continues to do well and has grown appropriately.
Fig. 4. Follow-up scan performed 3 days later demonstrates the catheter well positioned (arrow), within a small pleural fluid collection and reexpan sion of the left lung (L). The heart now occupies a more normal position (curved arrow). S = Fetal spine. noted previously resolved by postoperative day 2 (hospital day 9), the resultant hydrops and the bilat eral hydrocele resolved by postoperative day 3. On hospital day 12 there was spontaneous rupture o f membranes with vaginal pool phosphatidylglycerol positive; a normal spontaneous vaginal delivery was accomplished. A 2,280-gram male with Apgars of 4 and 8 was delivered without difficulty. A cord arterial blood gas was obtained: pH = 7.23, PCO2 o f 21 with
Bronchopulmonary sequestration is a congenital anomaly where pulmonary paren chyma is separate from normal lung and gen erally not in communication with an airway [7], Its blood supply is from systemic circula tion [8]; an estimated 30-40% have trans diaphragmatic vascular origins [9], Its inci dence is rare and without familial predispo sition [10], A male-to-female predominance has been described [9, 11], The embryologic origin of the broncho pulmonary tree provides an appreciation of the bronchopulmonary sequestration’s vas cular origins. Often arising as an outpouch ing of the foregut separate from and without connection to the normal tracheobronchial tree, the sequestration maintains its systemic circulatory origins without tracheal connec-
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Discussion
Antenatal Diagnosis and Treatment of Fetal Bronchopulmonary Sequestration
37
tion [12, 13]. Variable in size and appear ance, sequestered tissue is more commonly solid than cystic [10]. Bronchopulmonary se questration has been antenatally described a number of times as an echogenic thoracic mass [1-3], As one of the foregut malforma tions, bronchopulmonary sequestration is found more frequently with tracheoesopha geal fistula, esophageal duplication, esopha geal diverticula and esophageal and bron chogenic cysts [14-16], The prognosis for perinatal sequestration is poor. Of those patients with antenatally diagnosed bronchopulmonary sequestration, mortality is high and in a pediatric series, infants with multiple associated anomalies are common [2, 9-12, 14-16]. A recently described case of antenatally diagnosed bronchopulmonary sequestration with pal liative intrauterine treatment is available [6]. Although repeated thoracentesis and place ment of a thoracoamniotic shunt allowed resolution of hydrops, the infant died postnatally of other complications.
There have been several reports regarding fetal thoracentesis and transthoracic drain age of fetal hydrothorax [4, 5, 17-19]. Fetal hydrothorax has a perinatal mortality of 53100%. These effusions may be either pri mary or secondary. Primary effusions are generally chylous in nature, where secondary effusions may be part of a generalized fluid retention associated with nonimmune hy drops. Pleural effusions may also be associ ated with trisomy 21 [19], Intrathoracic compression of the develop ing lung produces pulmonary hypoplasia, which is the main cause of perinatal death in the fetus with hydrothorax [5, 19]. Large pleural effusions may cause hydroamnios by interfering with fetal swallowing and hy drops by vena cava obstruction and cardiac compression. In our case, it is interesting that the fetal ascites and the scrotal hydro celes resolved after transthoracic drainage. In cases where thoracentesis has been per formed, there has been rapid reaccumulation of pulmonary effusions, with most of these
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 5. Postnatal photo demon strating the distal tip of the shunt tube external to the left thorax (ar row).
fetuses dying from pulmonary insufficiency [4]. In fact, all 4 of the cases treated by Longaker et al. [4] with thoracentesis died be cause of pulmonary insufficiency. Only a fe tus which was treated by a thoracoamniotic shunt had permanent decompression of the pleural effusion with resolution of the hy drops. Similarly, in our case, after the initial thoracentesis there was reaccumulation of fluid and it was only with in utero shunting that there was a resolution of the hydrops and eventual delivery of a normal viable fetus. In review of the literature by Longaker et al. [4], they found that the larger the pleural effu sions. the greater the resultant hydrops, pul monary hypoplasia and the poorer the prog nosis. In case in which hydrops developed, there was a 52% survival rate, compared with 100% of fetuses surviving in cases in which hydrops did not develop. In our case, as the majority of fetal lung developments should have already occurred by the time of shunt ing, improved survival may be secondary to treatment of the fetal hydrops rather than protection of lung development. However, the age at delivery and age at diagnosis was important in determining survival. The age of diagnosis seems to be an important prog nostic indicator. If the age of diagnosis was less than 33 weeks, then only 43% of these fetuses survived, while if the age of diagnosis was greater than 33 weeks, 80% survival oc curred. Therefore, our case was in the time frame in which fetal prognosis is poor and fetal intervention is important. In summary, fetal hydrothorax is usually secondary to chylous effusion or nonimmune hydrops. Only rarely is pulmonary se questration the cause. If pleural effusions are large they may result in pulmonary hypopla sia. but may be successfully managed by in utero thoracoamniotic shunting.
Slotnick/McGahan/Milio/Schwartz/Ablin
References 1 Mariona. F. McAlpin G. Zador I, et al: Sono graphic detection of fetal extrathoracic pulmonary sequestration. JCU 1986:5:283. 2 Romero R, Chervenak FA, Kotzen J. et al: Ante natal sonographic findings of extralobar pulmo nary sequestration. J Ultrasound Med 1982; 1: 131. 3 Jouppila P, Kirkinen P, Herva T. et al: Prenatal diagnosis o f pleural effusions by ultrasound. JCU 1983:11:516. 4 Longaker MT. Laberge MJ. Dansereau J, Langer JC, et al: Primary fetal hydrothorax: Natural his tory and management. J Pediatr Surg 1989:24: 573. 5 Roberts AB. Clarkson NS. Pattison MG. Mok PM: Fetal hydrothorax in the second trimester of pregnancy: Successful intra-uterine treatment at 24 weeks gestation. Fetal Ther 1986:1:203. 6 Weiner C, Varner M, Pringle K, et al: Antenatal diagnosis and palliative treatment o f nonimmune hydrops fetalis secondary to pulmonary extralo bar sequestration. Obstet Gynecol 1986:68:275. 7 Iwai K, Shindo G, Hajikano H. et al: Intralobar pulmonary sequetration, with special reference to developmental pathology. Am Rev Respir Dis 1973:107:91 1. 8 Canty TG: Extralobar pulmonary sequestration. Unusual presentation and systemic vascular com munication in association with a right-sided dia phragmatic hernia. J Thorac Cardiovasc Surg 1981:81:96. 9 Carter R: Pulmonary sequestration. Ann Thorac Surg I969;7:68. 10 Savic B. Birtel FJ. Tholen W. et al: Lung seques tration: Report of seven cases and review o f 540 published cases. Thorax 1979;34:96. 11 Stocker JT, Kagan-Hallet K: Extralobar pulmo nary sequestration. Analysis o f 15 cases. Am J Clin Pathol 1979:72:917. 12 Sade RM. Clouse M, Ellis FH Jr: The spectrum of pulmonary sequestration. Ann Thorac Surg 1974; 18:644. 13 Wimbish KJ. Agha FP, Brady TM: Bilateral pul monary sequestration: Computed tomographic appearance. AJR 1983:140:689. 14 Bunta:n WL, Woolley MM. Mahour GIL et al: Pulmonary sequestration in children: A twentyfive year experience. Surgery 1977;81:413.
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
38
Antenatal Diagnosis and Treatment o f Fetal Bronchopulmonary Sequestration
19
Rodeck CH, Fisk NM, Fraser DI. Nicolini U: Long-term in utero drainage o f fetal hydrothorax. N Engl J Med 1988;319:1135.
Received: December 11, 1989 Accepted: October 14, 1990 R. Nathan Slotnick. MD Department o f Obstetrics and Gynecology University o f California Davis School o f Medicine 1621 Alhambra Boulevard, Suite 2500 Sacramento, CA 95616 (USA)
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
15 Demos NJ, Teresi A: Congenital lung malforma tions. A unified concept and a case report. J Thorac Cardiovasc Surg 1975:70:260. 16 Gerle RD, Jaretzki A, Ashley CA, et al: Congenital bronchopulmonary-foregut malformation: Pul monary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968;278: 1413. 17 Benaccrraf BR, Frigolctto FD Jr: Mid-trimester fetal thoracentesis. JCU 1985:13:202. 18 Benacerraf BR. Frigolctto FD Jr. Wilson M: Suc cessful midtrimester thoracentesis with analysis of the lymphocyte population in the pleural effusion. Am J Obstet Gynecol 1986:155:398-399.
39
Fetal Diagn Ther 1990;5:33-39
Antenatal Diagnosis and Treatment of Fetal Bronchopulmonary Sequestration R.N. Slotnicka , John McGahan'0, L. Milio a , M. Schwartzc, Deborah Ablinh Departments o f aObstetrics and Gynecology, bRadiology, and 'Pediatric Surgery, Division o f Prenatal Diagnosis and Genetics, University o f California, Davis School of Medicine, Sacramento, Calif., USA
Key Words. Ultrasound, thorax • Ultrasound, fetus • Fetal treatment
Introduction Bronchopulmonary sequestration is a rare congenital anomaly that has been diag nosed by prenatal ultrasound [1, 2], The association with large hydrothorax is rare [3-6], We report a case of a prenatally diag nosed hydrothorax, secondary to broncho pulmonary sequestration, successfully man aged by use of prenatal aspiration and drain age of the hydrothorax.
Case Report A 25-year-old gravida 2 para 1001 woman was referred at approximately 31 menstrual weeks o f ges tation for the evaluation o f polyhydramnios and non immune hydrops. Prior family and obstetric history was unremarkable with normal obstetrical ultrasound
at approximately 20 weeks o f gestation to corroborate dates. Upon transfer to our facility, an ultrasound revealed a single live intrauterine pregnancy o f ap proximately 32 weeks with gross polyhydramnios. There was a large left hydrothorax with dramatic mass effect incuding right shift o f the heart, severe compression o f pulmonary structures and downward displacement o f the diaphragm (fig. 1). There was some increased echogenicity o f portions o f the left lung. Mild abdominal ascites and bilateral hydrocele were appreciated. The remainder o f the ultrasound examination was normal. Amniocentesis was per formed upon arrival and revealed a lecithimsphingomyclin ratio of 1.4 with percent saturation o f lecithin o f 58%, negative phosphatidylglycerol and a 53% probalility o f newborn distress based on these val ues. Fetal thoracentesis was chosen as an option be cause of the massive fetal hydrothorax, significant mass effect and resultant fetal hydrops. On hospital day 3, ultrasound-guided thoracic placement of the 22-gauge needle was accomplished without difficulty; the intrathoracic fluid was under marked pressure
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Abstract. Bronchopulmonary sequestration with associated nonimmune hydrops has been previously reported with generally poor prognosis for the neonate. We report a case of bronchopulmonary sequestration and associated pleural effusion successfully managed with a transthoracic catheter placement. The embryology and clinical pathophysiology of bron chopulmonary sequestration are discussed.
34
Slotnick/McGahan/Milio/Schwartz/Ablin
(fig. 2). A total of 126 cm3 o f clear, yellow fluid was removed without difficulty. Postthoracentesis ultra sound showed a largely normal appearance of the fetal thorax except for a small left pleural effusion. Because of increased uterine activity, the patient was placed on prophylactic magnesium sulfate as a tocolytic. The patient was closely observed with daily ultrasound and there was gradual reaccumulation of pleural fluid. By hospital day 5, fluid reaccumulation had progressed to the point where thoracic ultrasound studies could not be distinguished from prethoracen tesis evaluations. Therefore, it was decided to redrain the pleural fluid. On day 7. using aseptic technique and under ultrasound guidance, a 5-French Harrison
double pigtail catheter (Cook Catheter. Bloomington, Ind.) was placed into the fetal thorax (fig. 3). At con clusion of the procedure, the distal tip of the catheter was in the left fetal thorax and the proximal curl within the amniotic cavity (fig. 3). A percutaneous umbilical cord sampling was also performed showing a normal male karyotype, 46,XY. A fetal hematocrit from blood sampling was measured to be 38%, Torch studies proved negative. The procedure was per formed without complications. Over the next few days repeat ultrasound exams showed that the thoracic catheter position was un changed and remarkably minimal thoracic fluid present (fig. 4). The left-to-right mediastinal shift
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 1. Transverse ultrasound (a) and accompanying longitudinal ultrasound (b) of the fetal thorax demonstrating large fluid collec tion (F) which displaces the heart (curved arrow) into the right hemithorax and displaces the diaphragm caudally (open arrow). The left lung is identified (arrows) within the fluid collection.
Antenatal Diagnosis and Treatment o f Fetal Bronchopulmonary Sequestration
35
Fig. 3. a Three days later, the fluid had nearly completely reaccu mulated and a double ‘J’ stent was placed through the anterior thorax (arrow) into the fluid collection (F). b Part of the catheter is coiled within the fluid collection (arrow) and the other part external to the fetal thorax (arrow). F = Fluid.
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 2. Transverse sonogram of the fetal thorax demonstrating the 20-gauge needle placed using a biopsy guide attachment through the anterior thorax into the fluid collection. The echogenic needle tip is well identified (curved arrow). The fluid was completely aspi rated.
36
Slotnick/McGahan/Milio/Schwartz/Ablin
hematocrit 43%. A venous umbilical blood gas was obtained: pH = 7.32. PCF of 21. PCO: of 20. The intrathoracic catheter was still in place and at the time o f delivery was draining fluid through and around the thoracic tubal aperture (fig. 5). Examina tion o f the tube after delivery revealed the lumen to be completely patent. The neonate required only minimal respiratory' support and was extubated on day 1 after delivery. However, pleural fluid continued to be drained through a chest tube for 1.5 weeks. The fluid re mained clear even after feedings were begun. A CT scan of the chest revealed an inhomogeneous mass of the base of the left lung, consistent with a pulmonary sequestration. The neonate was taken to the operating room on day 18 after delivery and a 3 X 4 X 4 cm pulmonary sequestration was removed without diffi culty. This mass was separate from the left lower lobe o f lung, was lined with pleura and was supplied by a transdiaphragmatic feeding artery and draining vein. Subsequent to this procedure the neonate did well and was discharged on postoperative day 5. The neo nate is now 4 months old and continues to do well and has grown appropriately.
Fig. 4. Follow-up scan performed 3 days later demonstrates the catheter well positioned (arrow), within a small pleural fluid collection and reexpan sion of the left lung (L). The heart now occupies a more normal position (curved arrow). S = Fetal spine. noted previously resolved by postoperative day 2 (hospital day 9), the resultant hydrops and the bilat eral hydrocele resolved by postoperative day 3. On hospital day 12 there was spontaneous rupture o f membranes with vaginal pool phosphatidylglycerol positive; a normal spontaneous vaginal delivery was accomplished. A 2,280-gram male with Apgars of 4 and 8 was delivered without difficulty. A cord arterial blood gas was obtained: pH = 7.23, PCO2 o f 21 with
Bronchopulmonary sequestration is a congenital anomaly where pulmonary paren chyma is separate from normal lung and gen erally not in communication with an airway [7], Its blood supply is from systemic circula tion [8]; an estimated 30-40% have trans diaphragmatic vascular origins [9], Its inci dence is rare and without familial predispo sition [10], A male-to-female predominance has been described [9, 11], The embryologic origin of the broncho pulmonary tree provides an appreciation of the bronchopulmonary sequestration’s vas cular origins. Often arising as an outpouch ing of the foregut separate from and without connection to the normal tracheobronchial tree, the sequestration maintains its systemic circulatory origins without tracheal connec-
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Discussion
Antenatal Diagnosis and Treatment of Fetal Bronchopulmonary Sequestration
37
tion [12, 13]. Variable in size and appear ance, sequestered tissue is more commonly solid than cystic [10]. Bronchopulmonary se questration has been antenatally described a number of times as an echogenic thoracic mass [1-3], As one of the foregut malforma tions, bronchopulmonary sequestration is found more frequently with tracheoesopha geal fistula, esophageal duplication, esopha geal diverticula and esophageal and bron chogenic cysts [14-16], The prognosis for perinatal sequestration is poor. Of those patients with antenatally diagnosed bronchopulmonary sequestration, mortality is high and in a pediatric series, infants with multiple associated anomalies are common [2, 9-12, 14-16]. A recently described case of antenatally diagnosed bronchopulmonary sequestration with pal liative intrauterine treatment is available [6]. Although repeated thoracentesis and place ment of a thoracoamniotic shunt allowed resolution of hydrops, the infant died postnatally of other complications.
There have been several reports regarding fetal thoracentesis and transthoracic drain age of fetal hydrothorax [4, 5, 17-19]. Fetal hydrothorax has a perinatal mortality of 53100%. These effusions may be either pri mary or secondary. Primary effusions are generally chylous in nature, where secondary effusions may be part of a generalized fluid retention associated with nonimmune hy drops. Pleural effusions may also be associ ated with trisomy 21 [19], Intrathoracic compression of the develop ing lung produces pulmonary hypoplasia, which is the main cause of perinatal death in the fetus with hydrothorax [5, 19]. Large pleural effusions may cause hydroamnios by interfering with fetal swallowing and hy drops by vena cava obstruction and cardiac compression. In our case, it is interesting that the fetal ascites and the scrotal hydro celes resolved after transthoracic drainage. In cases where thoracentesis has been per formed, there has been rapid reaccumulation of pulmonary effusions, with most of these
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
Fig. 5. Postnatal photo demon strating the distal tip of the shunt tube external to the left thorax (ar row).
fetuses dying from pulmonary insufficiency [4]. In fact, all 4 of the cases treated by Longaker et al. [4] with thoracentesis died be cause of pulmonary insufficiency. Only a fe tus which was treated by a thoracoamniotic shunt had permanent decompression of the pleural effusion with resolution of the hy drops. Similarly, in our case, after the initial thoracentesis there was reaccumulation of fluid and it was only with in utero shunting that there was a resolution of the hydrops and eventual delivery of a normal viable fetus. In review of the literature by Longaker et al. [4], they found that the larger the pleural effu sions. the greater the resultant hydrops, pul monary hypoplasia and the poorer the prog nosis. In case in which hydrops developed, there was a 52% survival rate, compared with 100% of fetuses surviving in cases in which hydrops did not develop. In our case, as the majority of fetal lung developments should have already occurred by the time of shunt ing, improved survival may be secondary to treatment of the fetal hydrops rather than protection of lung development. However, the age at delivery and age at diagnosis was important in determining survival. The age of diagnosis seems to be an important prog nostic indicator. If the age of diagnosis was less than 33 weeks, then only 43% of these fetuses survived, while if the age of diagnosis was greater than 33 weeks, 80% survival oc curred. Therefore, our case was in the time frame in which fetal prognosis is poor and fetal intervention is important. In summary, fetal hydrothorax is usually secondary to chylous effusion or nonimmune hydrops. Only rarely is pulmonary se questration the cause. If pleural effusions are large they may result in pulmonary hypopla sia. but may be successfully managed by in utero thoracoamniotic shunting.
Slotnick/McGahan/Milio/Schwartz/Ablin
References 1 Mariona. F. McAlpin G. Zador I, et al: Sono graphic detection of fetal extrathoracic pulmonary sequestration. JCU 1986:5:283. 2 Romero R, Chervenak FA, Kotzen J. et al: Ante natal sonographic findings of extralobar pulmo nary sequestration. J Ultrasound Med 1982; 1: 131. 3 Jouppila P, Kirkinen P, Herva T. et al: Prenatal diagnosis o f pleural effusions by ultrasound. JCU 1983:11:516. 4 Longaker MT. Laberge MJ. Dansereau J, Langer JC, et al: Primary fetal hydrothorax: Natural his tory and management. J Pediatr Surg 1989:24: 573. 5 Roberts AB. Clarkson NS. Pattison MG. Mok PM: Fetal hydrothorax in the second trimester of pregnancy: Successful intra-uterine treatment at 24 weeks gestation. Fetal Ther 1986:1:203. 6 Weiner C, Varner M, Pringle K, et al: Antenatal diagnosis and palliative treatment o f nonimmune hydrops fetalis secondary to pulmonary extralo bar sequestration. Obstet Gynecol 1986:68:275. 7 Iwai K, Shindo G, Hajikano H. et al: Intralobar pulmonary sequetration, with special reference to developmental pathology. Am Rev Respir Dis 1973:107:91 1. 8 Canty TG: Extralobar pulmonary sequestration. Unusual presentation and systemic vascular com munication in association with a right-sided dia phragmatic hernia. J Thorac Cardiovasc Surg 1981:81:96. 9 Carter R: Pulmonary sequestration. Ann Thorac Surg I969;7:68. 10 Savic B. Birtel FJ. Tholen W. et al: Lung seques tration: Report of seven cases and review o f 540 published cases. Thorax 1979;34:96. 11 Stocker JT, Kagan-Hallet K: Extralobar pulmo nary sequestration. Analysis o f 15 cases. Am J Clin Pathol 1979:72:917. 12 Sade RM. Clouse M, Ellis FH Jr: The spectrum of pulmonary sequestration. Ann Thorac Surg 1974; 18:644. 13 Wimbish KJ. Agha FP, Brady TM: Bilateral pul monary sequestration: Computed tomographic appearance. AJR 1983:140:689. 14 Bunta:n WL, Woolley MM. Mahour GIL et al: Pulmonary sequestration in children: A twentyfive year experience. Surgery 1977;81:413.
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
38
Antenatal Diagnosis and Treatment o f Fetal Bronchopulmonary Sequestration
19
Rodeck CH, Fisk NM, Fraser DI. Nicolini U: Long-term in utero drainage o f fetal hydrothorax. N Engl J Med 1988;319:1135.
Received: December 11, 1989 Accepted: October 14, 1990 R. Nathan Slotnick. MD Department o f Obstetrics and Gynecology University o f California Davis School o f Medicine 1621 Alhambra Boulevard, Suite 2500 Sacramento, CA 95616 (USA)
Downloaded by: Lund University Libraries 130.235.66.10 - 7/24/2017 7:24:19 PM
15 Demos NJ, Teresi A: Congenital lung malforma tions. A unified concept and a case report. J Thorac Cardiovasc Surg 1975:70:260. 16 Gerle RD, Jaretzki A, Ashley CA, et al: Congenital bronchopulmonary-foregut malformation: Pul monary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968;278: 1413. 17 Benaccrraf BR, Frigolctto FD Jr: Mid-trimester fetal thoracentesis. JCU 1985:13:202. 18 Benacerraf BR. Frigolctto FD Jr. Wilson M: Suc cessful midtrimester thoracentesis with analysis of the lymphocyte population in the pleural effusion. Am J Obstet Gynecol 1986:155:398-399.
39
