JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES Volume 24, Number 11, 2014 ª Mary Ann Liebert, Inc. DOI: 10.1089/lap.2014.0133
Case Report: Pediatrics
Thoracoscopic Removal of Retained Thoracoamniotic Shunt Catheters in Newborns Francesco Macchini, MD, Valerio Gentilino, MD, Anna Morandi, MD, and Ernesto Leva, MD
Abstract
Fetal hydrothorax is associated with significant mortality. However, the development of fetal thoracoamniotic shunting has reduced the mortality rate. Fetal thoracoamniotic shunting can be characterized by significant complications, such as intrathoracic dislodgement of the catheter. The ideal management of dislodged catheters postnatally is not known. We report two newborns with a prenatal diagnosis of fetal hydrothorax who underwent thoracoamniotic shunting complicated by intrathoracic dislodgement of the catheters requiring thoracoscopic removal of the shunts in the neonatal period. Introduction
F
etal pleural effusion is defined as the accumulation of fluid in the pleural space around the fetal lungs. Fetal hydrothorax may be primary or secondary. Multiple pathological conditions, such as congenital malformations, heart defects, cardiac arrhythmias, and viral infections, can cause extravasation of fluid seen in secondary pleural effusions, whereas hydrothorax is defined as primary when the fluid originates from anomalous lymphatic drainage.1 Fetal pleural effusions may spontaneously resolve or progress with subsequent development of nonimmune hydrops fetalis,2 which can be fatal if left untreated. Prenatal interventions have been developed for the management of fetal hydrothorax and may include fetal thoracocentesis3 or thoracoamniotic shunt placement.4,5 Neonatal survival following the insertion of chest tube is reported to be between 47% and 100%.4–7 Complications related to the insertion of fetal chest shunt include shunt obstruction, traumatic hemothorax,8 rupture of the amniotic membranes,9 constriction of fetal limbs by the shunt,10 dislodgement into the amniotic fluid,11 dislodgement into the maternal peritoneal cavity,12 or dislodgement into the fetal chest.13 Intrathoracic displacement has been reported at a rate of 20%.13 However, ideal management of a retained fetal thoracoamniotic shunt is not known. We present our experience with 2 cases of retained fetal thoracoamniotic shunts in the postnatal period. Case Reports Case 1
A gravida 3 para 2 woman was referred to our Center of Perinatal Surgery because of evidence of fetal hydrops in
primary bilateral hydrothorax at 27 weeks of gestation. No associated anomalies were found. At a different institution, bilateral thoracoamniotic shunt pigtail catheters were positioned at 30 weeks of gestation with complete resolution bilaterally of the fetal hydrothoraces. A female newborn, weighing 3805 g, was delivered at 37 weeks by spontaneous vaginal delivery. Apgar scores were 8 and 10 at 1 and 5 minutes, respectively. The left catheter was removed immediately after birth, whereas the right one was not visible. The baby was stable with no need for respiratory support. A chest radiograph revealed the right catheter inside the right hemithorax with extension into the right paracardiac region (Fig. 1a). An ultrasound suggested that the catheter was positioned in the fissure between the right upper and the right middle lobe. Based on the concern about the risk of hilar strangulation, the decision was made to perform thoracoscopy to remove the catheter on Day 5 of life. The site where the shunt had been placed was not visible on the chest wall, and two 3mm ports were inserted: one beneath the tip of the scapula and the other along the posterior axillary line, after induction of pneumothorax at 6 mm Hg. The catheter was identified in the fissure and was easily removed under vision with a grasper (Fig. 1b). No chest tube was left. Operative time was 35 minutes. The postoperative course was uneventful. Case 2
A gravida 1 para 0 woman was referred to our Center of Perinatal Surgery because of suspected primary bilateral hydrothorax and fetal hydrops at 23 weeks of gestation and drained with bilateral thoracoamniotic shunt pigtail catheters that were inserted at a different institution at 27 weeks. Prenatal ultrasound scans revealed the persistence of a left
Neonatal Surgery Unit, Center of Perinatal Surgery, Department of Pediatric Surgery, IRCCS Ca’ Granda Foundation, Maggiore Policlinico Hospital, Milan, Italy.
827
828
MACCHINI ET AL.
FIG. 1. Case 1. (a) Chest x-ray revealing a thoracoamniotic shunt catheter (arrows) inside the thoracic cavity with one of the extremities in the right paracardiac region. (b) Intraoperative image showing the shunt catheter after its mobilization away from the fissure. hydrothorax (24 · 49 mm). A male baby was delivered at 29 weeks through cesarean section due to maternal indications. Birth weight was 1880 g, and the newborn was apnoic, requiring intubation and mechanical ventilation. The right catheter was removed at birth, whereas the left one was not visible on the chest wall. A chest radiograph demonstrated that the catheter was located in the left paravertebral region along the course of the intrathoracic aorta (Fig. 2a). There was a large left pleural effusion requiring a left chest tube that initially drained 120 mL of pleural fluid. After stabilization of the patient and resolution of the hydrothorax, the decision to surgically remove the catheter was made. Thoracoscopy was performed on Day 14 of life. As in the previous case, the site where the shunt had been placed was not visible on the chest wall, and two 3-mm trocars were inserted, one beneath the tip of the scapula and the other one along the posterior axillary line. With insufflations to 5 mm Hg, the catheter was located in the left paravertebral region, where it was enveloped by fibrous tissue (Fig. 2b). Careful removal of the catheter was performed bluntly with a grasper. A chest tube was not left. Operative time was 30 minutes. The postoperative course
was complicated by a left pneumothorax that was successfully drained with a chest tube placed on the first postoperative day. Discussion
The outcome for fetuses with hydrothorax is strongly influenced by different factors: the gestational age at birth, the presence or absence of other congenital anomalies or hydrops, and the degree of pulmonary hypoplasia and hemodynamic impairment. Fetal thoracoamniotic shunting has been shown to be effective and can reverse the hydrops and prevent death.4 Although rare, dislodgement of the shunt into the fetal chest can occur. This may be due to the internalization of the intraamniotic segment due to growth of the chest wall or fetal respiratory movements. It may also be secondary to misplacement of the device at initial placement. Nothing is known about the specific causes of catheter dislodgement as the devices were not positioned in our institution, and the patients were only subsequently transferred to our Center
FIG. 2. Case 2. (a) Chest x-ray showing the presence of a thoracoamniotic shunt catheter (arrows) in the paravertebral region along the aortic course. (b) Intraoperative picture showing the catheter enveloped by fibrous tissue.
RETAINED THORACOAMNIOTIC SHUNT CATHETERS
because of the lack of a pediatric surgery department in the initial center. The decision of placing a thoracoamniotic shunt on both sides for bilateral hydrothoraces can be argued and is not generally part of our practice. In fact, the bilateral effusions can be considered as part of the hydrops spectrum, and treating one side usually leads to the resolution of the contralateral hydrothorax. Placing two shunts would furthermore increase the risk of complications. In our Center we place on average 10 shunts per year, and an additional shunt is added in bilateral effusions only for reaccumulation of fluid. In the last year this happened in 1 out of 5 patients (20%) with bilateral hydrothoraces. Different postnatal strategies in managing thoracoamniotic shunts have been proposed. Some authors13,14 have suggested conservative treatment without surgical removal. Their follow-up (24 and 12 months, respectively) did not revealed significant complications. Infections or respiratory distress was not reported in their series. Other authors have reported significant perinatal morbidity and mortality due to retained shunts. Blanch et al.8 reported a case where abnormal position caused strangulation of the pulmonary hilum, resulting in neonatal death. Other authors have advocated retrieving the shunt15 when a thoracic surgical procedure was required postnatally. Primary reason for us to advocate removal of the catheters was their position and concern for injury to the pulmonary hilum as a result. In fact, in both cases they were located in the mediastinum next to the lung hilum, so that they were considered at high risk of fatal complications. In addition, the limited number of reported cases and the short follow-up do not offer specific validated indications. However, because of the increasing accuracy of prenatal diagnosis and the widespread of fetal thoracoamniotic shunts, the number of thoracoamniotic shunting procedures will certainly rise, leading to the need for specific guidelines and standardized management protocols for a dislodged catheter. We suggest removing intrathoracic dislodged catheters sooner rather than later, once the patient has been stabilized, in order to avoid the risk of fibrous and inflammatory response that may make removal of the shunt more difficult. To the best of our knowledge, this is the first report of totally thoracoscopic removal of retained thoracoamniotic shunts. Thoracoscopy was considered as the procedure of choice thanks to its advantages: clear visualization of the thoracic cavity, removal under direct vision avoiding lesions to organs and vessels, small incisions, and reduced postoperative course. Conclusions
Thoracoscopic removal of dislodged thoracoamniotic shunt catheters in newborns is a safe and effective procedure. We believe that centralization of such rare cases in dedicated perinatal surgical centers with well-established fetal and neonatal surgical skills is mandatory to improve the clinical outcome and reduce mortality. Disclosure Statement
No competing financial interests exist. References
1. Wilson RD, Johnson MP. Prenatal ultrasound guided percutaneous shunts for obstructive uropathy and thoracic disease. Semin Pediatr Surg 2003;12:182–189.
829
2. Smith RP, Illanes S, Denbow ML, Soothill PW. Outcome of fetal pleural effusions treated by thoracoamniotic shunting. Ultrasound Obstet Gynecol 2005;26:63–66. 3. Schmidt W, Harms E, Wolf D. Successful prenatal treatment of non-immune hydrops fetalis due to congenital chylothorax-case report. Br J Obstet Gynaecol 1985;92: 685–687. 4. Nicolaides KH, Azar GB. Thoraco-amniotic shunting. Fetal Diagn Ther 1990;5:153–164. 5. Wilson RD, Baxter JK, Johnson MP, King M, Kasperski S, Crombleholme TM, Flake AW, Hedrick HL, Howell LJ, Adyick NS. Thoracoamniotic shunts: Fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther 2004;19:413–420. 6. Smith RP, Illanes S, Denbow ML, Soothill PW. Outcome of fetal pleural effusions treated by thoracoamniotic shunting. Ultrasound Obstet Gynecol 2005;26:63–66. 7. Thompson PJ, Greenough A, Nicolaides KH. Respiratory function in infancy following pleuroamniotic shunting. Fetal Diagn Ther 1993;8:79–83. 8. Blanch G, Walkinshaw SA, Hawdon JM, Weindling AM, van Velzen D, Rodeck CH. Internalization of pleuroamniotic shunt causing neonatal demise. Fetal Diagn Ther 1996;11:32–36. 9. Ronderos-Dumit D, Nicolini U, Vaughan J, Fisk NM, Chamberlain PF, Rodeck CH. Uterine-peritoneal amniotic fluid leakage: An unusual complication of intrauterine shunting. Obstet Gynecol 1991;78:913–915. 10. Brown R, Nicolaides K. Constriction band of the arm following insertion of a pleuroamniotic shunt. Ultrasound Obstet Gynecol 2000;15:439–440. 11. Rodeck CH, Fisk NM, Fraser DI, Nicolini U. Long-term in utero drainage of fetal hydrothorax. N Engl J Med 1988;319:1135–1138. 12. Bernaschek G, Deutinger J, Hansmann M, Bald R, Holzgreve W, Bollmann R. Feto-amniotic shunting—Report of the experience of four European centres. Prenat Diagn 1994;14:821–833. 13. Sepulveda W, Galindo A, Sosa A, Diaz L, Flores X, de la Fuente P. Intrathoracic dislodgement of pleuroamniotic shunt. Three case reports with long-term follow-up. Fetal Diagn Ther 2005;20:102–105. 14. Alkazaleh F, Saleem M, Badran E. Intrathoracic displacement of pleuroamniotic shunt successful in utero treatment of fetal hydrops secondary to hydrothorax. Fetal Diagn Ther 2009;25:40–43. 15. Inoue S, Odaka A, Baba K, Kunikata T, Sobajima H, Tamura M. Thoracoscopy-assisted removal of a thoracoamniotic shunt double-basket catheter dislodged into the fetal thoracic cavity: Report of three cases. Surg Today 2014;44:761–766.
Address correspondence to: Francesco Macchini, MD Neonatal Surgery Unit Center of Perinatal Surgery Department of Pediatric Surgery IRCCS Ca’ Granda Foundation Ospedale Maggiore Policlinico Via Commenda 10 20122 Milan Italy E-mail: [email protected]
Case Report: Pediatrics
Thoracoscopic Removal of Retained Thoracoamniotic Shunt Catheters in Newborns Francesco Macchini, MD, Valerio Gentilino, MD, Anna Morandi, MD, and Ernesto Leva, MD
Abstract
Fetal hydrothorax is associated with significant mortality. However, the development of fetal thoracoamniotic shunting has reduced the mortality rate. Fetal thoracoamniotic shunting can be characterized by significant complications, such as intrathoracic dislodgement of the catheter. The ideal management of dislodged catheters postnatally is not known. We report two newborns with a prenatal diagnosis of fetal hydrothorax who underwent thoracoamniotic shunting complicated by intrathoracic dislodgement of the catheters requiring thoracoscopic removal of the shunts in the neonatal period. Introduction
F
etal pleural effusion is defined as the accumulation of fluid in the pleural space around the fetal lungs. Fetal hydrothorax may be primary or secondary. Multiple pathological conditions, such as congenital malformations, heart defects, cardiac arrhythmias, and viral infections, can cause extravasation of fluid seen in secondary pleural effusions, whereas hydrothorax is defined as primary when the fluid originates from anomalous lymphatic drainage.1 Fetal pleural effusions may spontaneously resolve or progress with subsequent development of nonimmune hydrops fetalis,2 which can be fatal if left untreated. Prenatal interventions have been developed for the management of fetal hydrothorax and may include fetal thoracocentesis3 or thoracoamniotic shunt placement.4,5 Neonatal survival following the insertion of chest tube is reported to be between 47% and 100%.4–7 Complications related to the insertion of fetal chest shunt include shunt obstruction, traumatic hemothorax,8 rupture of the amniotic membranes,9 constriction of fetal limbs by the shunt,10 dislodgement into the amniotic fluid,11 dislodgement into the maternal peritoneal cavity,12 or dislodgement into the fetal chest.13 Intrathoracic displacement has been reported at a rate of 20%.13 However, ideal management of a retained fetal thoracoamniotic shunt is not known. We present our experience with 2 cases of retained fetal thoracoamniotic shunts in the postnatal period. Case Reports Case 1
A gravida 3 para 2 woman was referred to our Center of Perinatal Surgery because of evidence of fetal hydrops in
primary bilateral hydrothorax at 27 weeks of gestation. No associated anomalies were found. At a different institution, bilateral thoracoamniotic shunt pigtail catheters were positioned at 30 weeks of gestation with complete resolution bilaterally of the fetal hydrothoraces. A female newborn, weighing 3805 g, was delivered at 37 weeks by spontaneous vaginal delivery. Apgar scores were 8 and 10 at 1 and 5 minutes, respectively. The left catheter was removed immediately after birth, whereas the right one was not visible. The baby was stable with no need for respiratory support. A chest radiograph revealed the right catheter inside the right hemithorax with extension into the right paracardiac region (Fig. 1a). An ultrasound suggested that the catheter was positioned in the fissure between the right upper and the right middle lobe. Based on the concern about the risk of hilar strangulation, the decision was made to perform thoracoscopy to remove the catheter on Day 5 of life. The site where the shunt had been placed was not visible on the chest wall, and two 3mm ports were inserted: one beneath the tip of the scapula and the other along the posterior axillary line, after induction of pneumothorax at 6 mm Hg. The catheter was identified in the fissure and was easily removed under vision with a grasper (Fig. 1b). No chest tube was left. Operative time was 35 minutes. The postoperative course was uneventful. Case 2
A gravida 1 para 0 woman was referred to our Center of Perinatal Surgery because of suspected primary bilateral hydrothorax and fetal hydrops at 23 weeks of gestation and drained with bilateral thoracoamniotic shunt pigtail catheters that were inserted at a different institution at 27 weeks. Prenatal ultrasound scans revealed the persistence of a left
Neonatal Surgery Unit, Center of Perinatal Surgery, Department of Pediatric Surgery, IRCCS Ca’ Granda Foundation, Maggiore Policlinico Hospital, Milan, Italy.
827
828
MACCHINI ET AL.
FIG. 1. Case 1. (a) Chest x-ray revealing a thoracoamniotic shunt catheter (arrows) inside the thoracic cavity with one of the extremities in the right paracardiac region. (b) Intraoperative image showing the shunt catheter after its mobilization away from the fissure. hydrothorax (24 · 49 mm). A male baby was delivered at 29 weeks through cesarean section due to maternal indications. Birth weight was 1880 g, and the newborn was apnoic, requiring intubation and mechanical ventilation. The right catheter was removed at birth, whereas the left one was not visible on the chest wall. A chest radiograph demonstrated that the catheter was located in the left paravertebral region along the course of the intrathoracic aorta (Fig. 2a). There was a large left pleural effusion requiring a left chest tube that initially drained 120 mL of pleural fluid. After stabilization of the patient and resolution of the hydrothorax, the decision to surgically remove the catheter was made. Thoracoscopy was performed on Day 14 of life. As in the previous case, the site where the shunt had been placed was not visible on the chest wall, and two 3-mm trocars were inserted, one beneath the tip of the scapula and the other one along the posterior axillary line. With insufflations to 5 mm Hg, the catheter was located in the left paravertebral region, where it was enveloped by fibrous tissue (Fig. 2b). Careful removal of the catheter was performed bluntly with a grasper. A chest tube was not left. Operative time was 30 minutes. The postoperative course
was complicated by a left pneumothorax that was successfully drained with a chest tube placed on the first postoperative day. Discussion
The outcome for fetuses with hydrothorax is strongly influenced by different factors: the gestational age at birth, the presence or absence of other congenital anomalies or hydrops, and the degree of pulmonary hypoplasia and hemodynamic impairment. Fetal thoracoamniotic shunting has been shown to be effective and can reverse the hydrops and prevent death.4 Although rare, dislodgement of the shunt into the fetal chest can occur. This may be due to the internalization of the intraamniotic segment due to growth of the chest wall or fetal respiratory movements. It may also be secondary to misplacement of the device at initial placement. Nothing is known about the specific causes of catheter dislodgement as the devices were not positioned in our institution, and the patients were only subsequently transferred to our Center
FIG. 2. Case 2. (a) Chest x-ray showing the presence of a thoracoamniotic shunt catheter (arrows) in the paravertebral region along the aortic course. (b) Intraoperative picture showing the catheter enveloped by fibrous tissue.
RETAINED THORACOAMNIOTIC SHUNT CATHETERS
because of the lack of a pediatric surgery department in the initial center. The decision of placing a thoracoamniotic shunt on both sides for bilateral hydrothoraces can be argued and is not generally part of our practice. In fact, the bilateral effusions can be considered as part of the hydrops spectrum, and treating one side usually leads to the resolution of the contralateral hydrothorax. Placing two shunts would furthermore increase the risk of complications. In our Center we place on average 10 shunts per year, and an additional shunt is added in bilateral effusions only for reaccumulation of fluid. In the last year this happened in 1 out of 5 patients (20%) with bilateral hydrothoraces. Different postnatal strategies in managing thoracoamniotic shunts have been proposed. Some authors13,14 have suggested conservative treatment without surgical removal. Their follow-up (24 and 12 months, respectively) did not revealed significant complications. Infections or respiratory distress was not reported in their series. Other authors have reported significant perinatal morbidity and mortality due to retained shunts. Blanch et al.8 reported a case where abnormal position caused strangulation of the pulmonary hilum, resulting in neonatal death. Other authors have advocated retrieving the shunt15 when a thoracic surgical procedure was required postnatally. Primary reason for us to advocate removal of the catheters was their position and concern for injury to the pulmonary hilum as a result. In fact, in both cases they were located in the mediastinum next to the lung hilum, so that they were considered at high risk of fatal complications. In addition, the limited number of reported cases and the short follow-up do not offer specific validated indications. However, because of the increasing accuracy of prenatal diagnosis and the widespread of fetal thoracoamniotic shunts, the number of thoracoamniotic shunting procedures will certainly rise, leading to the need for specific guidelines and standardized management protocols for a dislodged catheter. We suggest removing intrathoracic dislodged catheters sooner rather than later, once the patient has been stabilized, in order to avoid the risk of fibrous and inflammatory response that may make removal of the shunt more difficult. To the best of our knowledge, this is the first report of totally thoracoscopic removal of retained thoracoamniotic shunts. Thoracoscopy was considered as the procedure of choice thanks to its advantages: clear visualization of the thoracic cavity, removal under direct vision avoiding lesions to organs and vessels, small incisions, and reduced postoperative course. Conclusions
Thoracoscopic removal of dislodged thoracoamniotic shunt catheters in newborns is a safe and effective procedure. We believe that centralization of such rare cases in dedicated perinatal surgical centers with well-established fetal and neonatal surgical skills is mandatory to improve the clinical outcome and reduce mortality. Disclosure Statement
No competing financial interests exist. References
1. Wilson RD, Johnson MP. Prenatal ultrasound guided percutaneous shunts for obstructive uropathy and thoracic disease. Semin Pediatr Surg 2003;12:182–189.
829
2. Smith RP, Illanes S, Denbow ML, Soothill PW. Outcome of fetal pleural effusions treated by thoracoamniotic shunting. Ultrasound Obstet Gynecol 2005;26:63–66. 3. Schmidt W, Harms E, Wolf D. Successful prenatal treatment of non-immune hydrops fetalis due to congenital chylothorax-case report. Br J Obstet Gynaecol 1985;92: 685–687. 4. Nicolaides KH, Azar GB. Thoraco-amniotic shunting. Fetal Diagn Ther 1990;5:153–164. 5. Wilson RD, Baxter JK, Johnson MP, King M, Kasperski S, Crombleholme TM, Flake AW, Hedrick HL, Howell LJ, Adyick NS. Thoracoamniotic shunts: Fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther 2004;19:413–420. 6. Smith RP, Illanes S, Denbow ML, Soothill PW. Outcome of fetal pleural effusions treated by thoracoamniotic shunting. Ultrasound Obstet Gynecol 2005;26:63–66. 7. Thompson PJ, Greenough A, Nicolaides KH. Respiratory function in infancy following pleuroamniotic shunting. Fetal Diagn Ther 1993;8:79–83. 8. Blanch G, Walkinshaw SA, Hawdon JM, Weindling AM, van Velzen D, Rodeck CH. Internalization of pleuroamniotic shunt causing neonatal demise. Fetal Diagn Ther 1996;11:32–36. 9. Ronderos-Dumit D, Nicolini U, Vaughan J, Fisk NM, Chamberlain PF, Rodeck CH. Uterine-peritoneal amniotic fluid leakage: An unusual complication of intrauterine shunting. Obstet Gynecol 1991;78:913–915. 10. Brown R, Nicolaides K. Constriction band of the arm following insertion of a pleuroamniotic shunt. Ultrasound Obstet Gynecol 2000;15:439–440. 11. Rodeck CH, Fisk NM, Fraser DI, Nicolini U. Long-term in utero drainage of fetal hydrothorax. N Engl J Med 1988;319:1135–1138. 12. Bernaschek G, Deutinger J, Hansmann M, Bald R, Holzgreve W, Bollmann R. Feto-amniotic shunting—Report of the experience of four European centres. Prenat Diagn 1994;14:821–833. 13. Sepulveda W, Galindo A, Sosa A, Diaz L, Flores X, de la Fuente P. Intrathoracic dislodgement of pleuroamniotic shunt. Three case reports with long-term follow-up. Fetal Diagn Ther 2005;20:102–105. 14. Alkazaleh F, Saleem M, Badran E. Intrathoracic displacement of pleuroamniotic shunt successful in utero treatment of fetal hydrops secondary to hydrothorax. Fetal Diagn Ther 2009;25:40–43. 15. Inoue S, Odaka A, Baba K, Kunikata T, Sobajima H, Tamura M. Thoracoscopy-assisted removal of a thoracoamniotic shunt double-basket catheter dislodged into the fetal thoracic cavity: Report of three cases. Surg Today 2014;44:761–766.
Address correspondence to: Francesco Macchini, MD Neonatal Surgery Unit Center of Perinatal Surgery Department of Pediatric Surgery IRCCS Ca’ Granda Foundation Ospedale Maggiore Policlinico Via Commenda 10 20122 Milan Italy E-mail: [email protected]
