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Laparoscopic treatment of biliary atresia and choledochal cyst
Seminars in Pediatric Surgery (2005) 14, 206-215
Laparoscopic treatment of biliary atresia and choledochal cyst Marcelo Martinez-Ferro, MD,a Edward Esteves, MD,b Pablo Laje, MDa a
From the J.P. Garrahan National Children’s Hospital, and Fundación Hospitalaria Private Children’s Hospital, Buenos Aires, Argentina; and the b Division de Cirurgia Pediatrica, Universidad de Goias, Goiania, Brazil. INDEX WORDS Minimally invasive surgery; Laparoscopy; Biliary atresia; Choledochal cyst
Minimally invasive surgery (MIS) has overcome many technical limitations and has evolved into a safe alternative for the treatment of many complex pediatric surgical procedures. The introduction of this approach for the correction of congenital biliary tract anomalies had to wait until instrumentation and surgeons’ skills improved enough. This happened not so long ago: less than 10 years have elapsed since the first reported case of a minimally invasive operation for choledochal cyst and less than 3 years since the first reported case of a laparoscopic Kasai. This article summarizes the experience gained by the authors in laparoscopic treatment of 41 patients with biliary atresia and 15 patients with choledochal cyst with similar surgical techniques, which are described in detail. Based on the encouraging results, the authors believe that MIS will soon become the gold standard for the correction of congenital biliary tract anomalies. © 2005 Elsevier Inc. All rights reserved.
Striking advances have been accomplished in pediatric minimally invasive surgery (MIS) since the first cases were reported two decades ago. Many of these techniques have evolved in a very short period of time and are safely applied to complex pediatric surgical procedures. Endosurgical procedures are already considered the gold standards for cholecystectomy, splenectomy, or fundoplication, and with rapidly evolving technological improvements and surgeons’ skills, other procedures are rapidly becoming feasible alternatives. However, some MIS procedures are only advisable at the end of the learning curve, and therefore remain in the hands of a few highly trained surgeons (eg, TEF repair, lung lobectomies, colectomies with ileal pouch reconstruction, or repair of duodenal atresia). Advanced biliary tract surgery involving specifically the treatment of congenital cystic
Address reprint requests and correspondence: Marcelo MartinezFerro, MD, Wineberg 3071, Olivos CP 1636, Buenos Aires, Argentina. E-mail: [email protected].
1055-8586/$ -see front matter © 2005 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2005.06.003
dilatations and atresias belonged to that category of intrinsically difficult procedures until recently. Less than 10 years have elapsed since the first reported case of a minimally invasive treatment of a choledochal cyst,1 and less than 3 years since the first reported case of a laparoscopic Kasai procedure.2 Taking into account that conventional biliary surgery in children requires an open large approach that creates dense adhesions that makes recipient hepatectomy extremely difficult when liver transplantation (LT) is ultimately necessary, it is easy to understand why a turn toward MIS may be a desirable goal. The greatest breakthrough in the treatment of patients with biliary atresia (BA) was provided by Kasai in 1959,3 with the introduction of the portoenterostomy (PE). Since then, several modifications have been proposed, but none of them have achieved better results in terms of bile flow and long-term survival. Furthermore, as soon as LT became a therapeutic option for patients with BA, several trials were conducted in an attempt to determine whether PE was better
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than LT alone, and how a previous PE could interfere with a future LT.4,5 It is known that, due to the presence of multiple adhesions, LT is a much more difficult operation when performed after a PE. Different authors identified these difficulties in terms of operative time, blood loss, postoperative complications (eg, higher bowel perforation rate), and overall survival rate, thus suggesting that perhaps the first and only therapy for patients with BA should be LT.6,7 On the other hand, Kasai PE offers a prolonged pretransplant interval, allowing patients to gain weight, grow, and develop.8 Although several clinical and histological scores have been used in an effort to predict the outcome of BA patients to define the need of a PE versus LT, none of these scores gained popularity.9 Endosurgical approach for biliary atresia would make sense if patients could benefit from the proven advantages of MIS10 plus another often ignored attribute: the absence of postoperative adhesions. If this last statement proves to be true and laparoscopic Kasai (LK) proves to be equal or better than open Kasai in terms of long-term bile flow and liver function, then LK might become a new gold standard for the initial surgical treatment for BA. Since Farello’s first report of MIS for choledochal cyst in 1995,1 a few subsequent authors have proposed improvements in technical aspects that have led to the technique presented in this report.11-13 Basically, the use of mechanical sutures has been abandoned and the number of ports reduced to four or even three with the aid of percutaneous liver stay sutures. The age at surgery has been reduced to 2 months old (the youngest in our series). All of our cases belonged to type I of Todani’s classification, but the same surgical technique can be applied to types II and IV. Finally, we believe that as more surgeons gain experience in MIS, the laparoscopic treatment of CC will also become the standard approach.
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Figure 1 Patient is placed across the table and positioned over an elevated platform in order to achieve maximum instrument mobility. Surgeon and both assistants are located at the patient’s feet, facing the monitor.
advanced to the sigmoid colon, to evacuate any residual air or intestinal contents.
Operative technique Preoperative treatment Bowel preparation Bowel preparation deserves special consideration when planning a laparoscopic approach of the biliary tree. Given the complexity of these techniques, the surgeon needs optimal vision of the surgical field and enough room to introduce needles, sutures, and instruments. Therefore, distension of intraabdominal hollow viscera may seriously interfere with the different surgical steps. Bowel cleansing with 100 mL/kg polyethylene glycol 3350 solution (GoLYTELY®, BAREX®) is the procedure of choice; otherwise, three to four saline enemas (30 mL/kg each) are indicated at 6, 3, and 1 hour before surgery. Once in the operating room and under general anesthesia, a soft rubber catheter is inserted through the anus and
Patient positioning Positioning is basically the same for BA and CC, except for older CC patients. Neonates and small infants are placed in a supine position across the table or at the lower end of the table. The surgeon stands at the center of the patient’s feet and both assistants at his left and right. To increase the range of movement of the instruments, patients are raised up by means of a custom-made 10-cm-high platform that we call “the altar” (Figure 1). Older children are placed in a supine position at the lower end of the table in a frog-legged position, with the same set up as for a Nissen fundoplication.
Surgical techniques Laparoscopic approach of the biliary tree is technically challenging and demands fully trained surgeons in mini-
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Figure 3 Liver retraction as described by Esteves. Percutaneous trans-hepatic stay sutures are placed on the left and right lobes. If needed, another percutaneous stitch from just below the xyphoid process can be used to snare the round ligament and retract the liver superiorly.
Figure 2 Positioning of the four trocars and most frequently used instruments passed through them. Notice the Shah-Neto technique for trocar fixation detailed in the upper left quadrant of the picture.
mally invasive techniques. Although BA and CC are different entities, both share many common technical aspects that can basically be divided in five main steps: 1. Port placement Four trocars are placed as shown in Figure 2. Technical specifications and diameters of the cannulae are detailed in Table 1. For the insertion of the first trocar, access to the peritoneal cavity is accomplished by using an open infraumbilical port. A purse string suture is tied around the opening and a 6-mm trocar is introduced. The purse string suture is tightened around the cannula and extra fixation of the trocar is achieved by using the Shah-Neto technique14 by means of a rubber suction
Table 1
tubing that is placed wrapping the cannula. A stay suture is then tied around the tubing and around the insufflation stopcock (Figure 2). This trocar stabilization technique is used for all ports, thus avoiding dislodging of the cannulae and CO2 subcutaneous emphysema. The first trocar is used for the insertion of a short (18 cm) 4-mm, 30-degree-wide angle telescope (Karl Storz, 28731 BWA). We recommend using a wide-angle scope because it provides a better vision in a limited working space. This trocar is also used for starting CO2 insufflation. A pressure of 8 mm Hg with a flow of 4 to 6 L/min provides an excellent surgical field and is well tolerated by patients. A second trocar is placed in the left flank. This is a 6-mm trocar with an extra 3-mm rubber reducer. If possible, we prefer a threaded cannula that enhances the fixation to the abdominal wall. This trocar must have a silicone leaflet valve or a similar device for the introduction of a curved needle. A third 3.5-mm trocar is placed at the right flank and is used for the introduction of grasping and dissection forceps. The fourth 3.5-mm trocar is introduced at the right lower quadrant and is mainly used for the introduction of a 3-mm aspiration/ irrigation device.
Position, diameters, and specifications of the trocars
Trocars
ø mm
Length (cm)
Device
Accesories (extra)
Position
1 2 3 and 4
6 6 3.9
8.5 6 5
Storz 30160WX Storz 30120EKX Storz 30117KPK
3-mm rubber sealing cap. (reducer) 3-mm rubber sealing cap. (reducer)
Umbilicus Left flank Right flank Right lower quadrant
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Figure 4 Technique for biliary atresia. (A) Most of the dissection and resection of the atretic biliary tract is performed using a 3-mm monopolar hook instrument. (B) The portal plate is carefully sectioned and excised with 3-mm scissors.
2. Liver retraction and exposure Liver stay sutures are very convenient for an adequate exposure of the biliary tree. As originally described by one of the authors,2 two percutaneous trans-hepatic stitches are placed (Figure 3; also see Figure 6A) entering the abdominal cavity near the border of the left and right costal margins, passing through the liver parenchyma and exiting the abdominal cavity 1 cm away from its entrance point. For additional retraction, another percutaneous stitch from just below the xyphoid process can be used to snare the round ligament and retract the liver superiorly. In our experience, none of the patients bled after this maneuver regardless of the diagnosis or the degree of liver fibrosis.
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using a 3-mm hook. The atretic gallbladder and cystic duct are dissected free from the liver and the dissection carried toward the fibrous remnant of the common bile duct and hepatic duct (Figure 4A). The dissection is then progressed to the duodenum and pancreas distally following the choledochal remnants, which are transected using the monopolar hook. Proximally, the atretic biliary tree leads directly to the portal plate. At this point, careful resection of enlarged hilar lymph nodes is needed to access the hilar vessels. Once both hepatic arteries and the portal vein are recognized, the portal plate has to be gently dissected using a 3-mm Kelly dissector. Special attention must be given the small portal vessels that emerge vertically from the portal plate to the portal vein; it is advised to coagulate them with the hook to increase the portal plate surface. Main hepatic arteries (left and right) and the portal vein are the anatomical landmarks that establish the boundaries of the portal plate. Finally, the fibrous remnant of the portal plate is excised sharply with a 3-mm curved endoscopic scissors (Figure 4B). In the majority of cases, we expect to observe under magnification bile flowing from small bile ducts still patent at the portal plate (Figure 5). Most times, profuse bleeding will appear after cutting the plate. It is advised to avoid the use of monopolar cautery as still patent microscopic bile ducts can be destroyed. We recommend packing the plate site with Surgicel® (Ethicon, Inc., Somerville, NJ) and waiting while the Roux-en-Y is performed. The hemostatic pack must be retrieved later before starting the anastomosis. (b) Technique for choledochal cyst. Before starting the dissection, the need for a cholangiography must be considered. We do not routinely perform contrast studies in these cases, but, if needed, a procedure similar to that used for biliary atresia can be performed at any time during surgery. The gallbladder is dissected free from the liver and the cystic
3. Dissection and resection of the abnormal biliary tree (a) Technique for biliary atresia. Initial inspection of the atretic biliary tree is important to determine whether there is a real need for performing cholangiography. In most cases, no study is needed as experienced surgeons can precisely determine whether the biliary tree is patent or atretic. If cholangiography is needed, a 22-gauge angiocath is used to percutaneously access the gallbladder and to perform a contrast study using diatrizoate meglumine contrast and regular fluoroscopy. Once the presence of an atretic biliary tree is confirmed, dissection is started by the gallbladder
Figure 5 Great magnification provided by the method enhances the surgeon’s view of the portal plate as seen in this picture, taken after the resection of the fibrous remnants. The Roux-en-Y limb will be placed at the encircled area. RHA, right hepatic artery; PV, portal vein; LHA, left hepatic artery.
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Seminars in Pediatric Surgery, Vol 14, No 4, November 2005 needed to seal the distal end of the cyst. If needed, interrupted stitches or titanium clips may be applied at this point. The proximal common hepatic duct is left closed until the Roux-en-Y loop is performed. 4. Construction of the Roux-en-Y The ligament of Treitz is easily identified by laparoscopy, and the proximal jejunum at 20 to 40 cm distal to the ligament is grasped, marked, and subsequently exteriorized. Marking the bowel correctly is crucial at this point to identify precisely the proximal and distal ends of the loop after exteriorization. For this purpose, we mark the proximal end with one dot and the distal end with two dots. Marks are placed by gently applying the tip of the monopolar hook to the seromuscular surface of the jejunum. The marked jejunum is exteriorized through the umbilical port wound and divided. The previously marked area (1 to 2 cm long) must be completely resected to avoid complications. It is wise to vertically enlarge the aponeurosis at the umbilical port site up to 15 mm so that the anastomosis can be performed smoothly (Figure 8). A 30-cm Roux-en-Y limb is created, and its proximal end is treated differently according to the
Figure 6 Technique for choledochal cyst. (A) Percutaneous transhepatic Esteves’s retraction sutures as used for BA are placed to expose the choledochal cyst. (B) Using the gall bladder as a grip for traction, the abnormally dilated biliary structures are dissected free from the liver parenchyma.
artery is divided by using a monopolar hook (Figure 6B). In older patients, cystic artery division required the use of Ligasure or Autosonix. Using the gallbladder as a grip for traction, the dilated common bile duct (cyst) is rotated to permit its posterior dissection from both sides. In younger patients, this is an easy maneuver and the posterior wall of the cyst is freely dissected from the hepatic artery and the portal vein without the need for opening the anterior cystic wall. In older patients, due to chronic cholangitis, it is safer to open the cyst transversely and evacuate its contents to separate the posterior wall from the surrounding tissues. In most of our cases, the dissection was completed without the need for opening the cyst wall. Dissection must be carried up to the proximal narrowing of the cyst, just before it divides into the left and right hepatic ducts. At this point, two 2 to 0 Ethibond threads are passed and temporarily tied around the common hepatic duct just at the proximal end of the cyst using an external Roeder knot to avoid continuous bile leaking from the proximal biliary tree. Then, the common hepatic duct is sectioned between both ties and dissection is carried on toward the pancreas (Figure 7A). In most cases, the cyst can be dissected free from the duodenum and pancreas using the hook (Figure 7B) and no sutures are
Figure 7 Technique for choledochal cyst. (A) The common hepatic duct is temporarily ligated in order to avoid continuous bile leakage into the operative field. Then it is completely transected between both ties. (B) In most cases, the cyst can be dissected free from the duodenum and pancreas, using the hook instrument.
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Figure 8 The Roux-en-Y limb is performed outside the peritoneal cavity using the previously dilated umbilical port wound.
diagnosis. In patients with BA, the end is left open and returned to the peritoneal cavity for an end-to-end anastomosis. In CC cases, the end is closed manually or by means of mechanical suture, so that an end-to-side anastomosis can be performed. A new purse string suture using a thicker thread (0 or 1 nylon) must be placed around the umbilical site to avoid CO2 leaking. Finally, the Roux-en-Y limb is passed either antecolic or retrocolic up to the porta hepatis. 5. Bilio-intestinal anastomosis
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Figure 10 Technique for choledochal cyst. An end-to-side anastomosis is preferred for choledochal cyst cases. A lateral intestinal incision can be performed in situ, thus permitting an exact diameter correlation between the intestinal and the biliary tract ends.
both posterior corners of the anastomosis (Figure 9). This maneuver will facilitate the precise placement of the posterior central stitches that must enter the portal plate near its posterior border and exit very close to the portal vein. For the anastomosis, we use extracorporeal Roeder knot tying. The anterior face of the porto-jejunostomy is easily performed by means of interrupted stitches.
(a) Technique for biliary atresia (porto-enterostomy). The anastomosis to the portal plate is performed with 5/0 PDS® (Ethicon, Inc.) with a C1 needle. Because it is featured with a silicone leaflet valve, all the sutures are passed in and out of the peritoneal cavity through the 6-mm left flank trocar. Although the anastomosis can be performed directly, we recommend passing two initial percutaneous stay sutures at
(b) Technique for choledochal cyst (hepato-jejunostomy). Because the diameter of the transected common bile duct may vary from patient to patient and most of the times that of the jejunum twice or three times the diameter of the biliary tree, we prefer to perform an end-to-side anastomosis. The advantage of this technique is that the lateral intestinal incision can be performed in situ, thus allowing an exact diameter correlation between the intestinal and biliary tract ends
Figure 9 Technique for biliary atresia. Percutaneous stay sutures are placed at each corner of the posterior wall of the anastomosis. This maneuver facilitates the correct placement of the rest of the posterior wall stitches as they pass close to the portal vein margin.
Figure 11 Technique for choledochal cyst. Percutaneous stay sutures are placed and the posterior wall of the anastomosis is performed with a running suture of 5/0 PDS and the anterior aspect with interrupted sutures of the same material.
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Figure 12 External appearance at 7th postoperative day after a laparoscopic Kasai PE for BA, before removal of the skin sutures (arrows).
(Figure 10). Once the antimesenteric wall of the jejunum is opened, two percutaneous stay sutures are placed on each corner of the posterior wall of the anastomosis (Figure 11). Subsequently, the posterior wall is sutured with running 5/0 PDS® (Ethicon, Inc.) and the anterior aspect with interrupted stitches of the same material. In contrast to BA cases, this is a high-flow anastomosis and special attention must been taken to avoid bile leakage.
Results Biliary atresia The first patient with BA that underwent a laparoscopic Kasai was operated on in February 2001 by one of the
Table 2
EE MMF
authors (E.E.). Since then, 41 cases have been treated by both first authors. Long-term results were assessed following the classification proposed by van Heurn and coworkers15: bile flow was defined as “good” if total bilirubin (TBil) levels returned to 3 mg/dL or less within 3 months after surgery, “partial” if TBil levels decreased after the operation but did not return to normal within 3 months after surgery, and “poor” if bilirubin levels remained high with acolic stools. The Brazilian patients (n ⫽ 19) were operated on at the Hospital of the University of Goias, Goiania, Brazil and other 4 centers by one of the authors (E.E.). The mean age at surgery was 43 days (range 24 to 150). One patient needed conversion due to profuse bleeding from an anomalous portal vein branch that was accidentally sectioned. Mean operative time was 2.5 hours (range 2 to 4.2 hours). After surgery, mean time for oral feedings was 18 hours, and mean hospital stay was 3.9 days. No other important operative complications were observed. At this institution, 35% of the patients received 0.2 mg/kg of prednisolone up to 4 to 6 months postoperatively. Following Van Heurn’s classification, 16/19 (84.2%) patients had good bile flow, 2/19 had partial bile flow (10.5%), and 1/19 (5.3%) had poor flow. Patients with good and partial bile flow represent 94.7%. From this series, 6/19 patients received a liver transplantation at a mean age of 14.1 months. As all patients were transplanted elsewhere, no data regarding the presence and nature of adhesions were available. The Argentinian patients (n ⫽ 22) were operated on at two institutions: The “J.P.Garrahan” National Children’s Hospital (JPGNCH) and the Fundacion Hospitalaria Children’s Hospital, Buenos Aires. Only patients operated on or supervised by one of the authors (M.M-F.) were included in this analysis. The mean age at surgery was 79.1 days (range 20 to 135 days). No conversion was needed. Two intraoperative complications were observed: an accidental section of the left hepatic artery without clinical consequences and an intraoperative pneumothorax not related to the surgical procedure but to airway anesthetic instrumentation. Mean length of surgery was 3.5 hours (range 2.5 to 4.5 hours). After surgery, mean time to oral feedings was 48 hours (range 20 to 600 hours). Mean hospital stay was 5 days (range 2 to 40 days). Only two patients received postoperative prednisolone. Immediate bile flow recovery was defined by the presence of colored stools up to 1 month after surgery, and this was observed in 89% of the cases. On long-term assessment, 7/22 (32%) had good bile flow, 9/22 (41%) had partial flow, and 6/22 (27%) had poor flow.
Comparative results of laparoscopic Kasai
Patients (n)
Age at Kasai (days)
Conversions
Op. time (hours)
Good ⫹ partial bile flow (%)
Steroids
Liver transplant
Age at transplant (months)
19 22
43 79.1
1 0
2.5 3.5
94.7 73
19/19 (100%) 2/22 (9%)
6/19 (31.6%) 10/22 (45.4%)
14.1 13
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Table 3 Comparison between historic series of conventional Kasai (1997 to 2001) and the laparoscopic cases from 2001 at the JPGNCH Conventional Kasai (n ⫽ 29)
Laparoscopic Kasai (n ⫽ 22)
Age at Kasai (days) Good ⫹ partial flow Poor flow Liver transplant
82.4 52% 48% 15/29 (51.7%)
Age at liver transplant (months)
14
79.15 73% 27% 10/22 (45.45%) 13
Patients with good and partial bile flow represent 73% of the series. From these patients, 10/22 patients received a liver transplantation at a mean age of 13 months. Excellent cosmetic results were observed in all patients (Figure 12); four patients presented with an umbilical hernia and two required further surgical repair. Table 2 summarizes the experience of both first authors. The Argentinian series was compared in a retrospective analysis with 29 patients that received a Conventional Kasai at the JPGNCH from 1997 to 2001. No difference in age at surgery was observed. As seen in Table 3, overall bile flow was better for the laparoscopic group. A similar number of patients required liver transplantation in both groups and at a similar age. As a second stage of analysis, further data were collected in a prospective study regarding the presence or absence of adhesions at the moment of laparotomy for liver resection. Adhesions were classified as unique or multiple. Multiple adhesions were subclassified in nine groups depending on the compromised organs. In this study, six patients received a Laparoscopic Kasai and were transplanted by the same transplant team at the JPGNCH; they were compared with six patients who received a conventional Kasai elsewhere and were transplanted in the same period at the JPGNCH. Both groups had similar characteristics regarding age, weight, and type of transplant. As seen in Table 4, although surgical time and blood loss were better for the laparoscopic group, the statistical analysis revealed no significant differ-
Table 4 Prospective analysis of complications encountered at the time of liver transplantation in patients with BA who previously received conventional versus laparoscopic Kasai
Blood loss Operative time Multiple adhesions Unique adhesion
Conventional Kasai
Laparoscopic Kasai
370 ml 180 min 6/6 (100%) 0
170 ml 120 min 0 1/6 (16.6%)
*Mann Whitney U test. † Fisher test (P ⬎ 0.05).
P 0.07* 0.1* 0.0015†
Figure 13 Intraoperative view of the peritoneal cavity at the time of liver transplantation of a patient with BA, and a previous laparoscopic Kasai PE. Absolute absence of adhesions is characteristic in these patients. Notice the tortuous appearance of the re-vascularized umbilical vein due to portal hypertension.
ence. However, when analyzing the presence of peritoneal adhesions, most patients in the laparoscopic group revealed an absolute absence of intraabdominal adhesions (Figure 13). When compared with the open group, a highly significant difference was observed (Table 4).
Choledochal cyst Fourteen patients with choledochal cyst were treated between both first authors at the above-mentioned institutions. Only one patient needed conversion due to severe adhesions secondary to recurrent cholangitis; even after conversion, the surgery remained extremely difficult to perform. Patients operated in Brazil received an end-toend anastomosis. One patient of the Argentinian Series presented a partial leak at the posterior wall of the hepato-intestinal anastomosis that needed open reoperation with a good final outcome. All patients are doing well, no patients presented complications at long-term follow up, and all remain free of complications. Overall results are detailed in Table 5.
Discussion Laparoscopic approach to CC and BA is no longer an idealistic and impracticable issue in children. Although
214 Table 5
EE MMF
Seminars in Pediatric Surgery, Vol 14, No 4, November 2005 Laparoscopic treatment of patients with choledochal cyst, comparative results Patients (n)
Age at surgery (years)
Conversions
Op. time (hours)
Operative complications
Postop. complications
First feeding (hours)
9 6
2.5 4.8
0 1
3.5 3.5
0 0
0 1(leak)
48 36
BA and CC share some technical aspects, they have very different physiopathology, natural history, pre- and postoperative management, and long-term prognosis. There is a clear rationale for the laparoscopic approach of CC, as it is well known that, once the abnormal dilated ducts are excised, the majority of these patients will enjoy a good quality of life. Under these circumstances, the excellent cosmetic results observed after MIS are obviously welcomed in comparison with the large incisions usually required for conventional operation. All the other known benefits of MIS (less pain, prompt oral feedings, and rapid hospital discharge) were also observed in all of our patients. There is a worldwide increasing experience with the laparoscopic approach of CC,16 and most series show good results with low conversion rates and outcomes similar to those observed with open surgery. Our series reveal similar results as those of other authors.12,13 In our experience, the only limitation for this approach was the presence of severe adhesions secondary to chronic and recurrent episodes of cholangitis in an older child. Considering the above-mentioned evidence, the laparoscopic treatment of CC has become the procedure of choice at our Institutions. Is there a rationale for laparoscopic approach to BA if almost 90% of the patients will end up with a LT, and what would be the real benefit of offering these patients a temporary good cosmetic result? We believe that this approach is beneficial: magnification provided by the telescope undoubtedly helps to optimize the dissection and identification of the anatomy of the portal plate, and the other benefits of MIS, like fast recovery, prompt oral feedings, less pain, and rapid hospital discharge, are also applicable to these patients. Nevertheless, all these arguments are still weak considering the poor prognosis of liver function in most patients. Although the Kasai portoenterostomy remains the first definitive surgical procedure for infants with BA, it invariably leads to the formation of intraabdominal adhesions, particularly in the supracolic compartment. In the frequent eventuality of liver transplantation months or years later, the presence of firm adhesions with profuse vascularization due to portal hypertension, together with coagulation defects associated with liver dysfunction in end-stage liver disease, add considerable technical difficulties and risks to the already complex transplant procedure. We showed in our series that patients treated by laparoscopic Kasai had almost no adhesions at the time of liver transplantation. Reduction of postoperative adhesion formation is a frequently ignored
attribute of MIS and has been analyzed by few authors.17,18 This feature now acquires maximum relevance in the surgical treatment of BA. A practical example that illustrates this situation is the fact that liver transplant surgeons at our institution encourage us now to operate all the BA cases by MIS techniques. The differences in bile flow and proportion of transplanted patients between the Brazilian and the Argentinian series may be explained by the different ages at surgery (43 days versus 79 days) and the use of postoperative steroids (35% versus 9%). The comparison of laparoscopic versus open procedures between two similar groups of patients from the same institution (Table 3) showed a better postoperative bile flow rate for the laparoscopic group and a similar transplantation rate and age at transplantation. Laparoscopic Kasai can be performed safely in infants. Our initial results reveal similar or even better postoperative results than those observed with the open conventional approach as well as the enormous advantage of preventing postoperative adhesions, thus facilitating subsequent liver transplantation. If all of these statements remain accurate, laparoscopic Kasai may become the gold standard procedure for initial treatment of BA.
References 1. Farello GA, Cerofolini A, Rebonato M, et al. Congenital choledochal cyst: video-guided laparoscopic treatment. Surg Laparosc Endosc 1995;5:354-8. 2. Esteves E, Neto EC, Neto MO, et al. Laparoscopic Kasai portoenterostomy for biliary atresia. Pediatr Surg Int 2002;18:737-40. 3. Kasai M, Suzuki S. A new operation for “non-correctable” biliary atresia: hepatic portoenterostomy. Shujutsu 1959;13:733-9. 4. Cuervas-Mons V, Rimola A, van Thiel DA, et al. Does previous abdominal surgery alter the outcome of pediatric patients subjected to orthotopic liver transplantation? Gastroenterology 1986;90:853-7. 5. Lilly JR, Altman RP. Liver transplantation and Kasai operation in the first year of life: therapeutic dilemma in biliary atresia. J Pediatr 1987;110:561-2. 6. Wood RP, Langnas A, Stratta R, et al. Optimal therapy for patients with biliary atresia: portoenterostomy (“Kasai” procedures) versus primary liver transplantation. J Pediatr Surg 1990;25:153-62. 7. Ryckman F, Fisher R, Pedersen S, et al. Improved survival in biliary atresia patients in the era of liver transplantation. J Pediatr Surg 1993;28:382-6. 8. Sandler AD, Azarow KS, Superina RA. The impact of a previous Kasai procedure on liver transplantation for biliary atresia. J Pediatr Surg 1997;32:416-9.
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9. Azarow KS, Phillips J, Sandler AD, et al. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg 1997;32:168-74. 10. Martínez Ferro M, Questa H, Gutierrez V. Operación de Kasai laparoscópica. Detalles técnicos y resultados iniciales de una técnica promisoria. Cir Pediatr 2004;17:36-9. 11. Shimura H, Tanaka M, Shimizu, et al. Laparoscopic treatment of congenital choledochal cyst. Surg Endosc 1998;12:1268-71. 12. Li L, Feng W, Jing-Bo F, et al. Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy. J Pediatr Surg 2004;39:1663-6. 13. Lee H, Hirose S, Bratton B, et al. Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst. J Pediatr Surg 2004;39:804-7. 14. Shah R, Neto PN. An Innovative technique for reusable laparoscopic
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215 cannula stabilization and fixation. Pediatr Endosurg Innov Tech 1997;1: 59-61. van Heurn ELW, Saing H, Tam PKH. Portoenterostomy for biliary atresia: long term survival and prognosis after esophageal variceal bleeding. J Pediatr Surg 2004;39:6-9. Ure B, Nustede R, Becker H. Laparoscopic resection of congenital choledochal cyst, hepaticoyeyunostomy, and externally made Rouxen-Y anastomosis. J Pediatr Surg 2005;40:728-30. Lobe T. Adhesions and other myths (Editorial). Pediatr Endosurg Innov Tech 2000;2:107. Esposito C, De Petra MR, Palazzo G, et al. Is there a reduction of postoperative adhesion formation in the pediatric age group after laparoscopy compared with open surgery? Pediatr Endosurg Innov Tech 2000;2:115-9.
Laparoscopic treatment of biliary atresia and choledochal cyst Marcelo Martinez-Ferro, MD,a Edward Esteves, MD,b Pablo Laje, MDa a
From the J.P. Garrahan National Children’s Hospital, and Fundación Hospitalaria Private Children’s Hospital, Buenos Aires, Argentina; and the b Division de Cirurgia Pediatrica, Universidad de Goias, Goiania, Brazil. INDEX WORDS Minimally invasive surgery; Laparoscopy; Biliary atresia; Choledochal cyst
Minimally invasive surgery (MIS) has overcome many technical limitations and has evolved into a safe alternative for the treatment of many complex pediatric surgical procedures. The introduction of this approach for the correction of congenital biliary tract anomalies had to wait until instrumentation and surgeons’ skills improved enough. This happened not so long ago: less than 10 years have elapsed since the first reported case of a minimally invasive operation for choledochal cyst and less than 3 years since the first reported case of a laparoscopic Kasai. This article summarizes the experience gained by the authors in laparoscopic treatment of 41 patients with biliary atresia and 15 patients with choledochal cyst with similar surgical techniques, which are described in detail. Based on the encouraging results, the authors believe that MIS will soon become the gold standard for the correction of congenital biliary tract anomalies. © 2005 Elsevier Inc. All rights reserved.
Striking advances have been accomplished in pediatric minimally invasive surgery (MIS) since the first cases were reported two decades ago. Many of these techniques have evolved in a very short period of time and are safely applied to complex pediatric surgical procedures. Endosurgical procedures are already considered the gold standards for cholecystectomy, splenectomy, or fundoplication, and with rapidly evolving technological improvements and surgeons’ skills, other procedures are rapidly becoming feasible alternatives. However, some MIS procedures are only advisable at the end of the learning curve, and therefore remain in the hands of a few highly trained surgeons (eg, TEF repair, lung lobectomies, colectomies with ileal pouch reconstruction, or repair of duodenal atresia). Advanced biliary tract surgery involving specifically the treatment of congenital cystic
Address reprint requests and correspondence: Marcelo MartinezFerro, MD, Wineberg 3071, Olivos CP 1636, Buenos Aires, Argentina. E-mail: [email protected].
1055-8586/$ -see front matter © 2005 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2005.06.003
dilatations and atresias belonged to that category of intrinsically difficult procedures until recently. Less than 10 years have elapsed since the first reported case of a minimally invasive treatment of a choledochal cyst,1 and less than 3 years since the first reported case of a laparoscopic Kasai procedure.2 Taking into account that conventional biliary surgery in children requires an open large approach that creates dense adhesions that makes recipient hepatectomy extremely difficult when liver transplantation (LT) is ultimately necessary, it is easy to understand why a turn toward MIS may be a desirable goal. The greatest breakthrough in the treatment of patients with biliary atresia (BA) was provided by Kasai in 1959,3 with the introduction of the portoenterostomy (PE). Since then, several modifications have been proposed, but none of them have achieved better results in terms of bile flow and long-term survival. Furthermore, as soon as LT became a therapeutic option for patients with BA, several trials were conducted in an attempt to determine whether PE was better
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than LT alone, and how a previous PE could interfere with a future LT.4,5 It is known that, due to the presence of multiple adhesions, LT is a much more difficult operation when performed after a PE. Different authors identified these difficulties in terms of operative time, blood loss, postoperative complications (eg, higher bowel perforation rate), and overall survival rate, thus suggesting that perhaps the first and only therapy for patients with BA should be LT.6,7 On the other hand, Kasai PE offers a prolonged pretransplant interval, allowing patients to gain weight, grow, and develop.8 Although several clinical and histological scores have been used in an effort to predict the outcome of BA patients to define the need of a PE versus LT, none of these scores gained popularity.9 Endosurgical approach for biliary atresia would make sense if patients could benefit from the proven advantages of MIS10 plus another often ignored attribute: the absence of postoperative adhesions. If this last statement proves to be true and laparoscopic Kasai (LK) proves to be equal or better than open Kasai in terms of long-term bile flow and liver function, then LK might become a new gold standard for the initial surgical treatment for BA. Since Farello’s first report of MIS for choledochal cyst in 1995,1 a few subsequent authors have proposed improvements in technical aspects that have led to the technique presented in this report.11-13 Basically, the use of mechanical sutures has been abandoned and the number of ports reduced to four or even three with the aid of percutaneous liver stay sutures. The age at surgery has been reduced to 2 months old (the youngest in our series). All of our cases belonged to type I of Todani’s classification, but the same surgical technique can be applied to types II and IV. Finally, we believe that as more surgeons gain experience in MIS, the laparoscopic treatment of CC will also become the standard approach.
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Figure 1 Patient is placed across the table and positioned over an elevated platform in order to achieve maximum instrument mobility. Surgeon and both assistants are located at the patient’s feet, facing the monitor.
advanced to the sigmoid colon, to evacuate any residual air or intestinal contents.
Operative technique Preoperative treatment Bowel preparation Bowel preparation deserves special consideration when planning a laparoscopic approach of the biliary tree. Given the complexity of these techniques, the surgeon needs optimal vision of the surgical field and enough room to introduce needles, sutures, and instruments. Therefore, distension of intraabdominal hollow viscera may seriously interfere with the different surgical steps. Bowel cleansing with 100 mL/kg polyethylene glycol 3350 solution (GoLYTELY®, BAREX®) is the procedure of choice; otherwise, three to four saline enemas (30 mL/kg each) are indicated at 6, 3, and 1 hour before surgery. Once in the operating room and under general anesthesia, a soft rubber catheter is inserted through the anus and
Patient positioning Positioning is basically the same for BA and CC, except for older CC patients. Neonates and small infants are placed in a supine position across the table or at the lower end of the table. The surgeon stands at the center of the patient’s feet and both assistants at his left and right. To increase the range of movement of the instruments, patients are raised up by means of a custom-made 10-cm-high platform that we call “the altar” (Figure 1). Older children are placed in a supine position at the lower end of the table in a frog-legged position, with the same set up as for a Nissen fundoplication.
Surgical techniques Laparoscopic approach of the biliary tree is technically challenging and demands fully trained surgeons in mini-
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Figure 3 Liver retraction as described by Esteves. Percutaneous trans-hepatic stay sutures are placed on the left and right lobes. If needed, another percutaneous stitch from just below the xyphoid process can be used to snare the round ligament and retract the liver superiorly.
Figure 2 Positioning of the four trocars and most frequently used instruments passed through them. Notice the Shah-Neto technique for trocar fixation detailed in the upper left quadrant of the picture.
mally invasive techniques. Although BA and CC are different entities, both share many common technical aspects that can basically be divided in five main steps: 1. Port placement Four trocars are placed as shown in Figure 2. Technical specifications and diameters of the cannulae are detailed in Table 1. For the insertion of the first trocar, access to the peritoneal cavity is accomplished by using an open infraumbilical port. A purse string suture is tied around the opening and a 6-mm trocar is introduced. The purse string suture is tightened around the cannula and extra fixation of the trocar is achieved by using the Shah-Neto technique14 by means of a rubber suction
Table 1
tubing that is placed wrapping the cannula. A stay suture is then tied around the tubing and around the insufflation stopcock (Figure 2). This trocar stabilization technique is used for all ports, thus avoiding dislodging of the cannulae and CO2 subcutaneous emphysema. The first trocar is used for the insertion of a short (18 cm) 4-mm, 30-degree-wide angle telescope (Karl Storz, 28731 BWA). We recommend using a wide-angle scope because it provides a better vision in a limited working space. This trocar is also used for starting CO2 insufflation. A pressure of 8 mm Hg with a flow of 4 to 6 L/min provides an excellent surgical field and is well tolerated by patients. A second trocar is placed in the left flank. This is a 6-mm trocar with an extra 3-mm rubber reducer. If possible, we prefer a threaded cannula that enhances the fixation to the abdominal wall. This trocar must have a silicone leaflet valve or a similar device for the introduction of a curved needle. A third 3.5-mm trocar is placed at the right flank and is used for the introduction of grasping and dissection forceps. The fourth 3.5-mm trocar is introduced at the right lower quadrant and is mainly used for the introduction of a 3-mm aspiration/ irrigation device.
Position, diameters, and specifications of the trocars
Trocars
ø mm
Length (cm)
Device
Accesories (extra)
Position
1 2 3 and 4
6 6 3.9
8.5 6 5
Storz 30160WX Storz 30120EKX Storz 30117KPK
3-mm rubber sealing cap. (reducer) 3-mm rubber sealing cap. (reducer)
Umbilicus Left flank Right flank Right lower quadrant
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Figure 4 Technique for biliary atresia. (A) Most of the dissection and resection of the atretic biliary tract is performed using a 3-mm monopolar hook instrument. (B) The portal plate is carefully sectioned and excised with 3-mm scissors.
2. Liver retraction and exposure Liver stay sutures are very convenient for an adequate exposure of the biliary tree. As originally described by one of the authors,2 two percutaneous trans-hepatic stitches are placed (Figure 3; also see Figure 6A) entering the abdominal cavity near the border of the left and right costal margins, passing through the liver parenchyma and exiting the abdominal cavity 1 cm away from its entrance point. For additional retraction, another percutaneous stitch from just below the xyphoid process can be used to snare the round ligament and retract the liver superiorly. In our experience, none of the patients bled after this maneuver regardless of the diagnosis or the degree of liver fibrosis.
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using a 3-mm hook. The atretic gallbladder and cystic duct are dissected free from the liver and the dissection carried toward the fibrous remnant of the common bile duct and hepatic duct (Figure 4A). The dissection is then progressed to the duodenum and pancreas distally following the choledochal remnants, which are transected using the monopolar hook. Proximally, the atretic biliary tree leads directly to the portal plate. At this point, careful resection of enlarged hilar lymph nodes is needed to access the hilar vessels. Once both hepatic arteries and the portal vein are recognized, the portal plate has to be gently dissected using a 3-mm Kelly dissector. Special attention must be given the small portal vessels that emerge vertically from the portal plate to the portal vein; it is advised to coagulate them with the hook to increase the portal plate surface. Main hepatic arteries (left and right) and the portal vein are the anatomical landmarks that establish the boundaries of the portal plate. Finally, the fibrous remnant of the portal plate is excised sharply with a 3-mm curved endoscopic scissors (Figure 4B). In the majority of cases, we expect to observe under magnification bile flowing from small bile ducts still patent at the portal plate (Figure 5). Most times, profuse bleeding will appear after cutting the plate. It is advised to avoid the use of monopolar cautery as still patent microscopic bile ducts can be destroyed. We recommend packing the plate site with Surgicel® (Ethicon, Inc., Somerville, NJ) and waiting while the Roux-en-Y is performed. The hemostatic pack must be retrieved later before starting the anastomosis. (b) Technique for choledochal cyst. Before starting the dissection, the need for a cholangiography must be considered. We do not routinely perform contrast studies in these cases, but, if needed, a procedure similar to that used for biliary atresia can be performed at any time during surgery. The gallbladder is dissected free from the liver and the cystic
3. Dissection and resection of the abnormal biliary tree (a) Technique for biliary atresia. Initial inspection of the atretic biliary tree is important to determine whether there is a real need for performing cholangiography. In most cases, no study is needed as experienced surgeons can precisely determine whether the biliary tree is patent or atretic. If cholangiography is needed, a 22-gauge angiocath is used to percutaneously access the gallbladder and to perform a contrast study using diatrizoate meglumine contrast and regular fluoroscopy. Once the presence of an atretic biliary tree is confirmed, dissection is started by the gallbladder
Figure 5 Great magnification provided by the method enhances the surgeon’s view of the portal plate as seen in this picture, taken after the resection of the fibrous remnants. The Roux-en-Y limb will be placed at the encircled area. RHA, right hepatic artery; PV, portal vein; LHA, left hepatic artery.
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Seminars in Pediatric Surgery, Vol 14, No 4, November 2005 needed to seal the distal end of the cyst. If needed, interrupted stitches or titanium clips may be applied at this point. The proximal common hepatic duct is left closed until the Roux-en-Y loop is performed. 4. Construction of the Roux-en-Y The ligament of Treitz is easily identified by laparoscopy, and the proximal jejunum at 20 to 40 cm distal to the ligament is grasped, marked, and subsequently exteriorized. Marking the bowel correctly is crucial at this point to identify precisely the proximal and distal ends of the loop after exteriorization. For this purpose, we mark the proximal end with one dot and the distal end with two dots. Marks are placed by gently applying the tip of the monopolar hook to the seromuscular surface of the jejunum. The marked jejunum is exteriorized through the umbilical port wound and divided. The previously marked area (1 to 2 cm long) must be completely resected to avoid complications. It is wise to vertically enlarge the aponeurosis at the umbilical port site up to 15 mm so that the anastomosis can be performed smoothly (Figure 8). A 30-cm Roux-en-Y limb is created, and its proximal end is treated differently according to the
Figure 6 Technique for choledochal cyst. (A) Percutaneous transhepatic Esteves’s retraction sutures as used for BA are placed to expose the choledochal cyst. (B) Using the gall bladder as a grip for traction, the abnormally dilated biliary structures are dissected free from the liver parenchyma.
artery is divided by using a monopolar hook (Figure 6B). In older patients, cystic artery division required the use of Ligasure or Autosonix. Using the gallbladder as a grip for traction, the dilated common bile duct (cyst) is rotated to permit its posterior dissection from both sides. In younger patients, this is an easy maneuver and the posterior wall of the cyst is freely dissected from the hepatic artery and the portal vein without the need for opening the anterior cystic wall. In older patients, due to chronic cholangitis, it is safer to open the cyst transversely and evacuate its contents to separate the posterior wall from the surrounding tissues. In most of our cases, the dissection was completed without the need for opening the cyst wall. Dissection must be carried up to the proximal narrowing of the cyst, just before it divides into the left and right hepatic ducts. At this point, two 2 to 0 Ethibond threads are passed and temporarily tied around the common hepatic duct just at the proximal end of the cyst using an external Roeder knot to avoid continuous bile leaking from the proximal biliary tree. Then, the common hepatic duct is sectioned between both ties and dissection is carried on toward the pancreas (Figure 7A). In most cases, the cyst can be dissected free from the duodenum and pancreas using the hook (Figure 7B) and no sutures are
Figure 7 Technique for choledochal cyst. (A) The common hepatic duct is temporarily ligated in order to avoid continuous bile leakage into the operative field. Then it is completely transected between both ties. (B) In most cases, the cyst can be dissected free from the duodenum and pancreas, using the hook instrument.
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Figure 8 The Roux-en-Y limb is performed outside the peritoneal cavity using the previously dilated umbilical port wound.
diagnosis. In patients with BA, the end is left open and returned to the peritoneal cavity for an end-to-end anastomosis. In CC cases, the end is closed manually or by means of mechanical suture, so that an end-to-side anastomosis can be performed. A new purse string suture using a thicker thread (0 or 1 nylon) must be placed around the umbilical site to avoid CO2 leaking. Finally, the Roux-en-Y limb is passed either antecolic or retrocolic up to the porta hepatis. 5. Bilio-intestinal anastomosis
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Figure 10 Technique for choledochal cyst. An end-to-side anastomosis is preferred for choledochal cyst cases. A lateral intestinal incision can be performed in situ, thus permitting an exact diameter correlation between the intestinal and the biliary tract ends.
both posterior corners of the anastomosis (Figure 9). This maneuver will facilitate the precise placement of the posterior central stitches that must enter the portal plate near its posterior border and exit very close to the portal vein. For the anastomosis, we use extracorporeal Roeder knot tying. The anterior face of the porto-jejunostomy is easily performed by means of interrupted stitches.
(a) Technique for biliary atresia (porto-enterostomy). The anastomosis to the portal plate is performed with 5/0 PDS® (Ethicon, Inc.) with a C1 needle. Because it is featured with a silicone leaflet valve, all the sutures are passed in and out of the peritoneal cavity through the 6-mm left flank trocar. Although the anastomosis can be performed directly, we recommend passing two initial percutaneous stay sutures at
(b) Technique for choledochal cyst (hepato-jejunostomy). Because the diameter of the transected common bile duct may vary from patient to patient and most of the times that of the jejunum twice or three times the diameter of the biliary tree, we prefer to perform an end-to-side anastomosis. The advantage of this technique is that the lateral intestinal incision can be performed in situ, thus allowing an exact diameter correlation between the intestinal and biliary tract ends
Figure 9 Technique for biliary atresia. Percutaneous stay sutures are placed at each corner of the posterior wall of the anastomosis. This maneuver facilitates the correct placement of the rest of the posterior wall stitches as they pass close to the portal vein margin.
Figure 11 Technique for choledochal cyst. Percutaneous stay sutures are placed and the posterior wall of the anastomosis is performed with a running suture of 5/0 PDS and the anterior aspect with interrupted sutures of the same material.
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Figure 12 External appearance at 7th postoperative day after a laparoscopic Kasai PE for BA, before removal of the skin sutures (arrows).
(Figure 10). Once the antimesenteric wall of the jejunum is opened, two percutaneous stay sutures are placed on each corner of the posterior wall of the anastomosis (Figure 11). Subsequently, the posterior wall is sutured with running 5/0 PDS® (Ethicon, Inc.) and the anterior aspect with interrupted stitches of the same material. In contrast to BA cases, this is a high-flow anastomosis and special attention must been taken to avoid bile leakage.
Results Biliary atresia The first patient with BA that underwent a laparoscopic Kasai was operated on in February 2001 by one of the
Table 2
EE MMF
authors (E.E.). Since then, 41 cases have been treated by both first authors. Long-term results were assessed following the classification proposed by van Heurn and coworkers15: bile flow was defined as “good” if total bilirubin (TBil) levels returned to 3 mg/dL or less within 3 months after surgery, “partial” if TBil levels decreased after the operation but did not return to normal within 3 months after surgery, and “poor” if bilirubin levels remained high with acolic stools. The Brazilian patients (n ⫽ 19) were operated on at the Hospital of the University of Goias, Goiania, Brazil and other 4 centers by one of the authors (E.E.). The mean age at surgery was 43 days (range 24 to 150). One patient needed conversion due to profuse bleeding from an anomalous portal vein branch that was accidentally sectioned. Mean operative time was 2.5 hours (range 2 to 4.2 hours). After surgery, mean time for oral feedings was 18 hours, and mean hospital stay was 3.9 days. No other important operative complications were observed. At this institution, 35% of the patients received 0.2 mg/kg of prednisolone up to 4 to 6 months postoperatively. Following Van Heurn’s classification, 16/19 (84.2%) patients had good bile flow, 2/19 had partial bile flow (10.5%), and 1/19 (5.3%) had poor flow. Patients with good and partial bile flow represent 94.7%. From this series, 6/19 patients received a liver transplantation at a mean age of 14.1 months. As all patients were transplanted elsewhere, no data regarding the presence and nature of adhesions were available. The Argentinian patients (n ⫽ 22) were operated on at two institutions: The “J.P.Garrahan” National Children’s Hospital (JPGNCH) and the Fundacion Hospitalaria Children’s Hospital, Buenos Aires. Only patients operated on or supervised by one of the authors (M.M-F.) were included in this analysis. The mean age at surgery was 79.1 days (range 20 to 135 days). No conversion was needed. Two intraoperative complications were observed: an accidental section of the left hepatic artery without clinical consequences and an intraoperative pneumothorax not related to the surgical procedure but to airway anesthetic instrumentation. Mean length of surgery was 3.5 hours (range 2.5 to 4.5 hours). After surgery, mean time to oral feedings was 48 hours (range 20 to 600 hours). Mean hospital stay was 5 days (range 2 to 40 days). Only two patients received postoperative prednisolone. Immediate bile flow recovery was defined by the presence of colored stools up to 1 month after surgery, and this was observed in 89% of the cases. On long-term assessment, 7/22 (32%) had good bile flow, 9/22 (41%) had partial flow, and 6/22 (27%) had poor flow.
Comparative results of laparoscopic Kasai
Patients (n)
Age at Kasai (days)
Conversions
Op. time (hours)
Good ⫹ partial bile flow (%)
Steroids
Liver transplant
Age at transplant (months)
19 22
43 79.1
1 0
2.5 3.5
94.7 73
19/19 (100%) 2/22 (9%)
6/19 (31.6%) 10/22 (45.4%)
14.1 13
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Table 3 Comparison between historic series of conventional Kasai (1997 to 2001) and the laparoscopic cases from 2001 at the JPGNCH Conventional Kasai (n ⫽ 29)
Laparoscopic Kasai (n ⫽ 22)
Age at Kasai (days) Good ⫹ partial flow Poor flow Liver transplant
82.4 52% 48% 15/29 (51.7%)
Age at liver transplant (months)
14
79.15 73% 27% 10/22 (45.45%) 13
Patients with good and partial bile flow represent 73% of the series. From these patients, 10/22 patients received a liver transplantation at a mean age of 13 months. Excellent cosmetic results were observed in all patients (Figure 12); four patients presented with an umbilical hernia and two required further surgical repair. Table 2 summarizes the experience of both first authors. The Argentinian series was compared in a retrospective analysis with 29 patients that received a Conventional Kasai at the JPGNCH from 1997 to 2001. No difference in age at surgery was observed. As seen in Table 3, overall bile flow was better for the laparoscopic group. A similar number of patients required liver transplantation in both groups and at a similar age. As a second stage of analysis, further data were collected in a prospective study regarding the presence or absence of adhesions at the moment of laparotomy for liver resection. Adhesions were classified as unique or multiple. Multiple adhesions were subclassified in nine groups depending on the compromised organs. In this study, six patients received a Laparoscopic Kasai and were transplanted by the same transplant team at the JPGNCH; they were compared with six patients who received a conventional Kasai elsewhere and were transplanted in the same period at the JPGNCH. Both groups had similar characteristics regarding age, weight, and type of transplant. As seen in Table 4, although surgical time and blood loss were better for the laparoscopic group, the statistical analysis revealed no significant differ-
Table 4 Prospective analysis of complications encountered at the time of liver transplantation in patients with BA who previously received conventional versus laparoscopic Kasai
Blood loss Operative time Multiple adhesions Unique adhesion
Conventional Kasai
Laparoscopic Kasai
370 ml 180 min 6/6 (100%) 0
170 ml 120 min 0 1/6 (16.6%)
*Mann Whitney U test. † Fisher test (P ⬎ 0.05).
P 0.07* 0.1* 0.0015†
Figure 13 Intraoperative view of the peritoneal cavity at the time of liver transplantation of a patient with BA, and a previous laparoscopic Kasai PE. Absolute absence of adhesions is characteristic in these patients. Notice the tortuous appearance of the re-vascularized umbilical vein due to portal hypertension.
ence. However, when analyzing the presence of peritoneal adhesions, most patients in the laparoscopic group revealed an absolute absence of intraabdominal adhesions (Figure 13). When compared with the open group, a highly significant difference was observed (Table 4).
Choledochal cyst Fourteen patients with choledochal cyst were treated between both first authors at the above-mentioned institutions. Only one patient needed conversion due to severe adhesions secondary to recurrent cholangitis; even after conversion, the surgery remained extremely difficult to perform. Patients operated in Brazil received an end-toend anastomosis. One patient of the Argentinian Series presented a partial leak at the posterior wall of the hepato-intestinal anastomosis that needed open reoperation with a good final outcome. All patients are doing well, no patients presented complications at long-term follow up, and all remain free of complications. Overall results are detailed in Table 5.
Discussion Laparoscopic approach to CC and BA is no longer an idealistic and impracticable issue in children. Although
214 Table 5
EE MMF
Seminars in Pediatric Surgery, Vol 14, No 4, November 2005 Laparoscopic treatment of patients with choledochal cyst, comparative results Patients (n)
Age at surgery (years)
Conversions
Op. time (hours)
Operative complications
Postop. complications
First feeding (hours)
9 6
2.5 4.8
0 1
3.5 3.5
0 0
0 1(leak)
48 36
BA and CC share some technical aspects, they have very different physiopathology, natural history, pre- and postoperative management, and long-term prognosis. There is a clear rationale for the laparoscopic approach of CC, as it is well known that, once the abnormal dilated ducts are excised, the majority of these patients will enjoy a good quality of life. Under these circumstances, the excellent cosmetic results observed after MIS are obviously welcomed in comparison with the large incisions usually required for conventional operation. All the other known benefits of MIS (less pain, prompt oral feedings, and rapid hospital discharge) were also observed in all of our patients. There is a worldwide increasing experience with the laparoscopic approach of CC,16 and most series show good results with low conversion rates and outcomes similar to those observed with open surgery. Our series reveal similar results as those of other authors.12,13 In our experience, the only limitation for this approach was the presence of severe adhesions secondary to chronic and recurrent episodes of cholangitis in an older child. Considering the above-mentioned evidence, the laparoscopic treatment of CC has become the procedure of choice at our Institutions. Is there a rationale for laparoscopic approach to BA if almost 90% of the patients will end up with a LT, and what would be the real benefit of offering these patients a temporary good cosmetic result? We believe that this approach is beneficial: magnification provided by the telescope undoubtedly helps to optimize the dissection and identification of the anatomy of the portal plate, and the other benefits of MIS, like fast recovery, prompt oral feedings, less pain, and rapid hospital discharge, are also applicable to these patients. Nevertheless, all these arguments are still weak considering the poor prognosis of liver function in most patients. Although the Kasai portoenterostomy remains the first definitive surgical procedure for infants with BA, it invariably leads to the formation of intraabdominal adhesions, particularly in the supracolic compartment. In the frequent eventuality of liver transplantation months or years later, the presence of firm adhesions with profuse vascularization due to portal hypertension, together with coagulation defects associated with liver dysfunction in end-stage liver disease, add considerable technical difficulties and risks to the already complex transplant procedure. We showed in our series that patients treated by laparoscopic Kasai had almost no adhesions at the time of liver transplantation. Reduction of postoperative adhesion formation is a frequently ignored
attribute of MIS and has been analyzed by few authors.17,18 This feature now acquires maximum relevance in the surgical treatment of BA. A practical example that illustrates this situation is the fact that liver transplant surgeons at our institution encourage us now to operate all the BA cases by MIS techniques. The differences in bile flow and proportion of transplanted patients between the Brazilian and the Argentinian series may be explained by the different ages at surgery (43 days versus 79 days) and the use of postoperative steroids (35% versus 9%). The comparison of laparoscopic versus open procedures between two similar groups of patients from the same institution (Table 3) showed a better postoperative bile flow rate for the laparoscopic group and a similar transplantation rate and age at transplantation. Laparoscopic Kasai can be performed safely in infants. Our initial results reveal similar or even better postoperative results than those observed with the open conventional approach as well as the enormous advantage of preventing postoperative adhesions, thus facilitating subsequent liver transplantation. If all of these statements remain accurate, laparoscopic Kasai may become the gold standard procedure for initial treatment of BA.
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215 cannula stabilization and fixation. Pediatr Endosurg Innov Tech 1997;1: 59-61. van Heurn ELW, Saing H, Tam PKH. Portoenterostomy for biliary atresia: long term survival and prognosis after esophageal variceal bleeding. J Pediatr Surg 2004;39:6-9. Ure B, Nustede R, Becker H. Laparoscopic resection of congenital choledochal cyst, hepaticoyeyunostomy, and externally made Rouxen-Y anastomosis. J Pediatr Surg 2005;40:728-30. Lobe T. Adhesions and other myths (Editorial). Pediatr Endosurg Innov Tech 2000;2:107. Esposito C, De Petra MR, Palazzo G, et al. Is there a reduction of postoperative adhesion formation in the pediatric age group after laparoscopy compared with open surgery? Pediatr Endosurg Innov Tech 2000;2:115-9.