Unusual Case

Early laparoscopic Kasai’s procedure in a low weight newborn

M Lopez, N Kalfa, D Forgues, M P Guibal, R B Galifer, H Allal Department of Visceral Pediatric , Lapeyronie Hospital, Montpellier, France

Address for correspondence: Dr. Hossein Allal, Chief of pediatric Video Surgery Division, Department of Pediatric Surgery, Montpellier University, France. E-mail: [email protected]

Abstract successful establishment of postoperative flow, the presence of microscopically demonstrable ductal The authors present an early laparoscopic treatment structures in the hilum, the degree of parenchyma in a newborn with .They describe the technical details of the Kasai laparoscopic disease at diagnosis and technical factors of the procedure. A 10-day-old girl, weight 2.4 kg, was anastomosis and diminish of jaundice and hepatic admitted with a history of jaundice and fecal acholia blood-test. Standard operation includes large, painful, since birth, with elevated total bilirubin and muscle-cutting to accomplish hilar abnormal hepatic test. Abdominal ultrasound dissection and porto-enterostomy according to Kasai showed a small with hyperechogenicity [3] in porta hepatis and absence of biliary principal duct. technique or variants. In addition children with Other metabolic and hematological tests were biliary atresia are frequently operated upon some normal. The procedure was performed at 20-day- degree of malnutrition and hepatic dysfunction and old by .The confirmed they show a high rate of peroperative complications the biliary atresia and Kasai’s procedure was due to surgical.com). trauma. [4] continued by laparoscopy and transumbilical extracorporeal Roux-Y approach. The duration of the procedure was 220 min, with good tolerance of Laparoscopic instrumentation and new techniques pneumoperitoneum due to the laparoscopy. have advanced. After first report by Esteves, the Feedings of breast milk began on the third day umbilical approach could be used for safe postoperative, presenting normal colored stools, .medknowextracorporeal Roux-en-Y enteric anastomosis in with normalization of the hepatic test. A 20 months [5] follow-up was without complications. babies with laparoscopic assistance. We report that (www Kasai porto-enterostomy can be performed in a low Keywords: Biliary atresia, Kasai’s procedure, weight newborn by laparoscopy without laparoscopy, newborn complications. The initial result was encouraging and This PDFa site is hostedavailable by forMedknowthe free patient download recovered Publications promptly from after this procedure.[6]

INTRODUCTION CASE REPORT

Before the introduction of the Kasai porto­ A 10-day-old girl, weight 2.5 kg, was admitted with enterostomy, biliary atresia survival rates were less a history of jaundice, choluria and fecal acholia since than 5% at 12 months of age.[1] Survival rates greater birth. The pregnancy had been uneventful and the than 50% after porto-enterostomy have been reported mother had addiction to morphine. All serologic and with the addition of transplantation, survival studies were normal; she was born by normal delivery rates greater than 90% should be now expected.[2] at 39 weeks’ gestation weighing 2.3 kg. Laboratory data by the age of 12 days showed that total bilirubin The major determinant factors of a satisfactory (Tbil) was 26.4 mg/dl (normal 0.2-1.0); direct bilirubin outcome following porto-enterostomy are: the (Dbil), 12.5 mg/dl; alanine aminotransferase (ALT) patient’s age at operation (under three months), the 94 IU/l (normal 5-32); aspartate aminotransferase (AST)

Journal of Minimal Access Surgery | April-June 2007 | Volume 3 | Issue 2 66 Lopez, et al.: Laparoscopic Kasai’s in a newborn

90IU/l (normal 9-32); alkaline phosphatase (AP) Treitz ligament and drawn to the umbilical port under 153 IU/l; and gamma-glutamyltransferase (GGT) 180 direct vision and withdrawn through the umbilicus. IU/l (normal 5-35). Other metabolic, hematological The pneumoperitoneum was interrupted to relieve and neonatal serologic test results were normal. An tension on the mesentery as necessary to make ultrasound scan (US) showed a small gallbladder, no extracorporeal transumbilical jejunal Roux-en-Y intra or extra hepatic bile ducts, with anastomosis. The jejunum was transected by stapler. hyperechogenicity at level hepatis portal that Another 40 cm segment beyond the distal was suggested the possibility of biliary atresia. At 20 days selected for lateral-end anastomosis and was placed of age, a laparoscopic exploration was elected. back into the abdomen. The correct position of the intestine was checked by laparoscopy. The procedure was performed with the patient positioned at the foot of the table, the surgeon at the The tied proximal jejunum was passed through the patient’s feet; the assistant with the camera at the right, mesocolon and the lateral-end running suture the second assistant and instrument nurse at the left. around the porta hepatis was done using intracorporeal self-block sutures posterior and Anesthesia was performed with inhaled and running suture anteriorly [Figure 2]. The percutaneus intravenous agents associated with short acting by hepafix needle was associated. intravenous opiates and muscle relaxants were administered to facilitate the surgical procedure. The patient was intubated and mechanically ventilated with the inspired oxygen fraction setting at 70%.

A 7 mm incision was made in the upper umbilical ring by open technique for the telescope trocar .com). position. The CO² was insufflated to 8 mmHg. Four additional 3.0 mm trocars were placed under direct laparoscopic vision in the left and right upper quadrant, left iliac fossa and epigastric area. .medknow The examination confirmed a liver cirrhotic appearance an extra hepatic bile-duct atresia by (www cholangiography with a small, fibrotic gallbladder Figure 1: Porta hepatis dissection without passage into biliary duct. For liver exposure we used the palpatorThis in epigastric PDFa site area is hostedwhichavailable rotates by forMedknow free download Publications from upward to expose the porta hepatis (Figure 1). Thereafter, mobilization of the gallbladder following the cystic duct, to the junction with the extra-hepatic duct remnants was performed. The reliquant was divided distally and then dissected toward the hepatic hilum, just to find the division of the portal vein and the hepatic arteries’ entry point. This exposition allowed a complete transection of “porta hepatis” just under the liver surface with scissors leaving a surface with ducts draining bile [Figure 1].

With the epigastria trocar the left colon was up forward and the jejunum was secured 20 cm from Figure 2: Biliary-digestive anastomosis

67 Journal of Minimal Access Surgery | April-June 2007 | Volume 3 | Issue 2 Lopez, et al.: Laparoscopic Kasai’s in a newborn

The tolerance of pneumoperitoneum was acceptable. possible complications related to this approach The operative time was 220 min. The infant needed include: pains, breathing limitation leading to no respiratory support postoperatively. Feeding pulmonary complications, nerves damage, prolonged began on the third day, producing normal colored ileus, wound dehiscence, large or recurrent incision stools on the same day. , rib damage, peritoneal adhesions. Dehiscence and hernias are usually presented in Broad-spectrum antibiotics were continued for only patients with hypoproteinemia, ascitis, which are three days postoperative and when oral feedings common features in these patients.[7] When the began, amoxicillin and clavulonic was given orally. procedure was started laparoscopically, its utility was The jaundice progressively declined with limited only to evaluate the liver status: for irregular normalization hepatic function with a follow-up of liver surface and greenish-brown color in patients 20 months. with EHBA. The presence or absence of the gallbladder, as well as biliary ducts is obviously atretic DISCUSSION by cholangiography. Progressive improvements in instrumentation over the last 15 years have led to Prolonged neonatal jaundice may be caused by the development of 3 mm, short laparoscopic different medical and surgical pathologies. Extra instruments designed specifically for use in the [7] hepatic biliary atresia (EHBA) is one of the causes. pediatric population allowing new techniques and Early diagnosis of EHBA in jaundiced infants is new complex procedures. mandatory, because the success rate of the operation is inversely proportional to the age of the patient, The original technique reported by Esteves showed 80% if the operation is performed when the infant is a new approach for Laparoscopic hilar dissection, younger than 60 days, but the success rate decreases extracorporeal entero-anastomosis and porto - [8,9] 20% if the child is older than three months of age. enterostomy with.com). good results. Today this procedure The diagnostic investigations have to be accelerated could decrease theses complications with advantages and the admittance of the patient to the surgical unit of minimally invasive technique, safe, short-lasted, must not be delayed. The evaluation of an infant with the intraabdominal viscera are minimally touched, prolonged jaundice consists of biochemical and the opening is small,[14-15] showing significantly lower serologic tests and abdominal ultrasonography. The .medknowincidences of adhesions.[16] Amplified, well-illuminated positive “triangular cord sign” in the abdominal visibility of the tiny hilar structures is guaranteed by ultrasonography is highly suggestive of EHBA,[10] laparoscopy. We believe that laparoscopic porto- hepatobiliary scintigraphy and fine-needle(www liver enterostomy and extracorporeal transumbilical biopsy is carried out.[6,11,12] jejunal anastomosis is a promising technique that may be performed in a low weight newborn without Laparoscopic instrumentationThis PDFa siteand is techniques hostedavailable have by forMedknowcomplications, free download Publications allowing from the patient to recover advanced to the point that laparoscopic, has found a promptly. The initial results have been encouraging great spectrum of indications in pediatric surgery and and its real impact will be revealed after additional now it is used also in biliary atresia cases.[13] Before cases. Theses procedures should be performed by a the introduction of laparoscopy, laparotomy had to surgeon experienced both in biliary surgery and be performed on the infants with prolonged jaundice advanced laparoscopic to increase the chances for in whom neonatal hepatitis could not be confirmed successful results.[17] with the laboratory, radiology and histopathology investigations. Conventional surgical procedures in REFERENCES biliary atresia, used to gain some bile drainage into the intestine in children; have been achieved through 1. Adelman S. Prognosis of uncorrected biliary atresia: An update. J Pediatr Surg 1978;13:389-91. large laparotomy in the upper abdomen. One of the 2. Grosfeld JL, Fitzgerald JF, Predaina R, West KW, Vane DW, Rescorla largest incisions in pediatric abdominal surgery, with FJ. The efficacy of hepatoportoenterostomy in the biliary atresia.

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Surgery 1989;106:692-701. biliary tract abnormalities after portoenterostomy. J Pediatr Surg 3. Bates MD, Bucuvalas JC, Alonso MH, Ryckman FC. Biliary atresia: 1991;26:32-6. Pathogenesis and treatment. Semin Liver Dis 1998;18:281-93. 12. Andres JM. Neonatal hepatobiliary disorders. Clin Perinatol 4. Sandler AD, Azarow KS, Superina RA. The impact of a previous 1996;23:321-52. Kasai procedure on for biliary atresia. J Pediatr 13. Park WH, Choi SO, Lee HJ. The ultrasonographic ‘triangular cord’ Surg 1997;32:416-9. coupled with gallbladder images in the diagnostic prediction of 5. Esteves E, Clemente Neto E, Ottaiano Neto M, Devanir J Jr, Esteves biliary atresia from infantile intrahepatic cholestasis. J Pediatr Pereira R. Laparoscopic Kasai portoenterostomy for biliary atresia. Surg 1999;34:1706-10. Pediatr Surg Int 2002;18:737-40. 14. Leape LL, Ramenofsky ML. Laparoscopy in infants and children. J 6. Ferro M. Laparoscopic Kasai operation. Technical details and Pediatr Surg 1977;12:929-38. preliminary results of a promising technique. Cir Pediatr 15. Schier F, Waldschmidt J. Experience with laparoscopy for the 2004;17:36-9. evaluation of cholestasis in newborns. Surg Endosc 1990;4:13-4. 7. Grosfeld JL. Biliary tract disorders: Cholestasis in infancy. In: 16. Bozkurt P, Kaya G, Yeker Y, Tunali Y, Altintas F. The cardiorespiratory Sabiston DC, Lyerly HK, editors. Sabiston Textbook of Surgery. effects of laparoscopic procedures in infants. Anesthesia Saunders: Philadelphia, PA; 1997. p. 1256-9. 1999;54:831-4. 8. Hirsig J, Rickham PP. Early differential diagnosis between neonatal 17. Polymeneas G, Theodosopoulos T, Stamatiadis A, Kourias E. A hepatitis and biliary atresia. J Pediatr Surg 1980;15:13-5. comparative study of postoperative adhesion formation after 9. Mieli Vergani G, Howard ER, Portman B, Mowat AP. Late referral laparoscopic vs open . Surg Endos 2001;15:41-3. for biliary atresia Missed opportunities for effective surgery. Lancet 1989;1:421-3. Cite this article as: Lopez M, Kalfa N, Forgues D, Guibal MP, Galifer RB, 10. Shah AH, Spiwak W. Neonatal cholestasis: New approaches to Allal H. Early laparoscopic Kasai’s in a low weight newborn. J Min Access diagnostic evaluation and therapy. Pediatr Clin North Am Surg 2007;3: 66-9. 1994;41:943-66. Date of submission: 11/12/06, Date of acceptance: 22/02/07 11. Nakama T, Kitamura T, Matsui A, Makino S, Senyuz OF, Kanazawa Source of Support: Nil, Conflict of Interest: None declared. K. Ultrasonographic findings and management of intrahepatic

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1) Include the referees’ remarks and point to point clarification to those remarks at the beginning in the revised article file itself. In addition, mark the changes as underlined or coloured text in the article. Please include in a single file a. referees’ comments b. point to point clarifications on the comments c. revised article with text highlighting the changes done 2) Include the original comments of the reviewers/editor with point to point reply at the beginning of the article in the ‘Article File’. To ensure that the reviewer can assess the revised paper in timely fashion, please reply to the comments of the referees/editors in the following manner. • There is no data on follow-up of these patients. Authors’ Reply: The follow up of patients have been included in the results section [Page 3, para 2] • Authors should highlight the relation of complication to duration of diabetes. Authors’ Reply: The complications as seen in our study group has been included in the results section [Page 4, Table]

69 Journal of Minimal Access Surgery | April-June 2007 | Volume 3 | Issue 2