Gastroschisis,
Malrotation,
and Chylous
Ascites
By David A. Lloyd
Pittsburgh, Pennsylvania l A newborn infant who had an uncomplicated repair of gastroschisis with modified Ladd’s procedure developed chylous ascites when fed. After 7 weeks of nonoperative management with total parenteral nutrition, the ascites resolved. Widening the mesentery may have damaged the lymphatics, and is not necessary for malrotation associated with gastroschisis. Prolonged nonoperative management is recommended for neonatal chylous ascites, avoiding paracentesis if possible. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: neonatal.
Gastroschisis;
malrotation;
chylous ascites,
A
NEWBORN infant developed chylous ascites following repair of gastroschisis. This rare complication may have resulted from the operative procedure done to correct the intestinal nonrotation. CASE
REPORT
The patient was the product of an uncomplicated pregnancy. Fetal ultrasound examination at 19 weeks’ gestation showed features consistent with gastroschisis. At 40 weeks’ gestation, a full-term male infant weighing 4.06 kg was delivered by cesarean section. There were no physical abnormalities apart from a classical gastroschisis with prolapsed small intestine and colon and failure of rotation. Most of the small intestine was covered with the remains of a sac and appeared grossly normal. A thin fibrinous peel covered the rest of the prolapsed intestine. The ascending colon was displaced to the left after incising the peritoneum along its right margin. In this way the base of the mesentery was widened. The appendix was removed. After stretching the abdominal wall and decompressing the intestine proximally and distally, primary closure of the abdominal wall was achieved. A Broviac central venous catheter was inserted. Postoperatively, the patient was maintained on parenteral nutrition. Enteral feedings (Portagen, Mead-Johnson, Evansville, IN) were begun on the 1lth postoperative day and were well tolerated. During the ensuing 5 days, there was increasing ascites and peripheral edema. On day 16 his weight was 4.69 kg. Paracentesis showed milky fluid containing fat and lymphocytes consistent with chylous ascites. Enteral feedings were discontinued and total parenteral nutrition (TPN) was maintained. The ascites and peripheral edema, which involved mainly the pelvis and lower limbs, persisted for the next 30 days. He also developed bilateral hydroceles. Because of severe respiratory distress associated with the intense abdominal distension, paracentesis was required on five
From the Children’s Hospital of Pittsburgh and the University of Pittsburgh School of Medicine, Pittsburgh, PA. Address reprint requests to DavidA. Lloyd, MChir (Cantab), FRCS, Institute of Child Health, Royal Liverpool Children’s Hospital Alder Hey, Eaton Rd, Liverpool L12 2AP, England. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2601-0030$03.00/O
106
occasions, removing between 135 and 410 mL of fluid each time. Following each paracentesis the ascitic fluid rapidly reaccumulated within 24 hours. The body weight and abdominal girth increased progressively, reaching maximum levels on day 44, when the body weight was 7.3 kg and the abdominal girth was 52 cm. After the initial two paracenteses, the serum albumin decreased to 1.5 g/dL. Subsequently, the protein removed at each paracentesis was replaced with an equal amount of 25% albumin, based on the ascitic fluid protein content of 2.2 g/dL. The parenteral nutrition amino-acid content was increased to 3 g of protein per kilogram per day. In response to these measures, the serum albumin increased to approximately 2.2 to 2.4 g/dL. There was one episode of central line infection that responded to vancomycin. By day 52 there had been no change in weight or abdominal girth. Respiratory distress was worsening and 410 mL of ascitic fluid was removed by paracentesis. Prior to this, 5 mL of cream was given by gavage, and in spite of this the ascitic fluid was clear. The ascitic fluid was replaced with 400 mL of 25% albumin and 65 mg of furosamide was given intravenously. The response was an intense diuresis for 48 hours. Four days later the weight was 6.19 kg and abdominal girth was 41 cm. Pregestimal (Mead-Johnson) feeds were started and were well tolerated. Breast feeding was begun 4 days later and the TPN was discontinued. The peripheral edema, hydroceles, and ascites progressively resolved. At 60 days of age the infant was discharged, at which time his weight was 6.02 kg, abdominal girth 40 cm, and serum albumin 3.6 g/dL. At follow-up 9 months later he was thriving on an unrestricted diet, had normal gastrointestinal function, and weighed 9.1 kg. DISCUSSION
Chylous ascites is a rare condition occurring twice as often during the first 2 months of life as in older children. A variety of causes have been documented, including obstruction of the mesenteric lymphatics in association with intestinal malrotation, intussusception, incarcerated hernia, adhesive bands, or retroperitoneal fibrosis; leaking lymphatics without obstruction; lymphangioma; and blunt abdominal trauma.“’ In more than half the affected infants the cause is not known, and the chylous ascites is attributed to undefined congenital malformations. There is no recorded association between chylous ascites and gastroschisis. Although the infant reported here had nonrotation of the intestine, a typical finding with gastroschisis, there was no gross evidence of volvulus or other obstructive lesion of the mesentery. At the time of operation the base of the mesentery was widened by dividing the peritoneal attachments of the ascending colon and displacing it to the left, as recommended by Ladd in 1936.3 With gastroschisis the mesentery is thickened and indurated, and it is possible that the mesenteric lymphatits were damaged during this dissection. However,
JournalofPediatricSurgery,
Vol26, No 1 (January),
1991: pp 106-107
GASTROSCHISIS,
MALROTATION,
107
& CHYLOUS ASCITES
the procedure has been used on previous occasions for malrotation with and without gastroschisis, without chylous ascites developing. In fact, Ladd’s procedure is not necessary for malrotation when it is associated with gastroschisis, because the risk of the thickened, edematous intestine undergoing volvulus is remote. Regarding the management of chylous ascites, this report confirms the value of prolonged bowel rest and TPN.’ A medium-chain triglyceride-rich formula (Portagen) was initially used in this infant, but the ascites worsened. Therefore, TPN was used. As the weeks pass, there may be increasing pressure on the surgeon to operate; this should be resisted as long as the TPN is being well tolerated and the serum albumin and lymphocyte count do not fall excessively. Two thirds of infants will respond to nonoperative therapy.J Further, there is no guarantee that a correctable abnormality will be encountered at laparotomy.
Laparotomy has been recommended after a trial of TPN of 4 to 6 weeks’; this may not be long enough, because from our experience and that of others it is clear that the lymphatic leak may not close for at least 6 weeks. Cochran et al* reported an infant who was treated with home TPN for 10 weeks, during which time the chylous ascites resolved. With increasing intraabdominal pressure, progressive respiratory distress may appear to necessitate paracentesis. However, following paracentesis the chylous ascites rapidly reaccumulates, seemingly to a greater extent each time, depleting the patient of serum proteins and lymphocytes. Left untapped, the increasing intraabdominal pressure has a tamponading effect, slowing the rate of lympatic leak, and infants with respiratory distress may adapt to the increased intraabdominal pressure. Therefore, paracentesis should be avoided for as long as possible, and reserved for severe respiratory compromise.
REFERENCES 1.
PK: Ascites, In Welch KJ, Randolph JG. Ravitch MM. et al (eds): Pediatric Surgery. Chicago, IL, Year Book, 1986 2. Cochran WJ, Klish WJ, Brown MR. et al: Chylous ascites in infants and children: A case report and literature review. J Pediatr Gastroenterol Nutr 4:668-673. 1985 Kottmeier
3. Ladd WE: Surgical diseases of the alimentary N Engl J Med 215:705-708, 1936 4. Unger SW, Chandler JG: Chylous ascites children. Surgery 93:455-461.1983
tract in infants. in infants
and
Malrotation,
and Chylous
Ascites
By David A. Lloyd
Pittsburgh, Pennsylvania l A newborn infant who had an uncomplicated repair of gastroschisis with modified Ladd’s procedure developed chylous ascites when fed. After 7 weeks of nonoperative management with total parenteral nutrition, the ascites resolved. Widening the mesentery may have damaged the lymphatics, and is not necessary for malrotation associated with gastroschisis. Prolonged nonoperative management is recommended for neonatal chylous ascites, avoiding paracentesis if possible. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: neonatal.
Gastroschisis;
malrotation;
chylous ascites,
A
NEWBORN infant developed chylous ascites following repair of gastroschisis. This rare complication may have resulted from the operative procedure done to correct the intestinal nonrotation. CASE
REPORT
The patient was the product of an uncomplicated pregnancy. Fetal ultrasound examination at 19 weeks’ gestation showed features consistent with gastroschisis. At 40 weeks’ gestation, a full-term male infant weighing 4.06 kg was delivered by cesarean section. There were no physical abnormalities apart from a classical gastroschisis with prolapsed small intestine and colon and failure of rotation. Most of the small intestine was covered with the remains of a sac and appeared grossly normal. A thin fibrinous peel covered the rest of the prolapsed intestine. The ascending colon was displaced to the left after incising the peritoneum along its right margin. In this way the base of the mesentery was widened. The appendix was removed. After stretching the abdominal wall and decompressing the intestine proximally and distally, primary closure of the abdominal wall was achieved. A Broviac central venous catheter was inserted. Postoperatively, the patient was maintained on parenteral nutrition. Enteral feedings (Portagen, Mead-Johnson, Evansville, IN) were begun on the 1lth postoperative day and were well tolerated. During the ensuing 5 days, there was increasing ascites and peripheral edema. On day 16 his weight was 4.69 kg. Paracentesis showed milky fluid containing fat and lymphocytes consistent with chylous ascites. Enteral feedings were discontinued and total parenteral nutrition (TPN) was maintained. The ascites and peripheral edema, which involved mainly the pelvis and lower limbs, persisted for the next 30 days. He also developed bilateral hydroceles. Because of severe respiratory distress associated with the intense abdominal distension, paracentesis was required on five
From the Children’s Hospital of Pittsburgh and the University of Pittsburgh School of Medicine, Pittsburgh, PA. Address reprint requests to DavidA. Lloyd, MChir (Cantab), FRCS, Institute of Child Health, Royal Liverpool Children’s Hospital Alder Hey, Eaton Rd, Liverpool L12 2AP, England. Copyright o 1991 by W.B. Saunders Company 0022-3468/91/2601-0030$03.00/O
106
occasions, removing between 135 and 410 mL of fluid each time. Following each paracentesis the ascitic fluid rapidly reaccumulated within 24 hours. The body weight and abdominal girth increased progressively, reaching maximum levels on day 44, when the body weight was 7.3 kg and the abdominal girth was 52 cm. After the initial two paracenteses, the serum albumin decreased to 1.5 g/dL. Subsequently, the protein removed at each paracentesis was replaced with an equal amount of 25% albumin, based on the ascitic fluid protein content of 2.2 g/dL. The parenteral nutrition amino-acid content was increased to 3 g of protein per kilogram per day. In response to these measures, the serum albumin increased to approximately 2.2 to 2.4 g/dL. There was one episode of central line infection that responded to vancomycin. By day 52 there had been no change in weight or abdominal girth. Respiratory distress was worsening and 410 mL of ascitic fluid was removed by paracentesis. Prior to this, 5 mL of cream was given by gavage, and in spite of this the ascitic fluid was clear. The ascitic fluid was replaced with 400 mL of 25% albumin and 65 mg of furosamide was given intravenously. The response was an intense diuresis for 48 hours. Four days later the weight was 6.19 kg and abdominal girth was 41 cm. Pregestimal (Mead-Johnson) feeds were started and were well tolerated. Breast feeding was begun 4 days later and the TPN was discontinued. The peripheral edema, hydroceles, and ascites progressively resolved. At 60 days of age the infant was discharged, at which time his weight was 6.02 kg, abdominal girth 40 cm, and serum albumin 3.6 g/dL. At follow-up 9 months later he was thriving on an unrestricted diet, had normal gastrointestinal function, and weighed 9.1 kg. DISCUSSION
Chylous ascites is a rare condition occurring twice as often during the first 2 months of life as in older children. A variety of causes have been documented, including obstruction of the mesenteric lymphatics in association with intestinal malrotation, intussusception, incarcerated hernia, adhesive bands, or retroperitoneal fibrosis; leaking lymphatics without obstruction; lymphangioma; and blunt abdominal trauma.“’ In more than half the affected infants the cause is not known, and the chylous ascites is attributed to undefined congenital malformations. There is no recorded association between chylous ascites and gastroschisis. Although the infant reported here had nonrotation of the intestine, a typical finding with gastroschisis, there was no gross evidence of volvulus or other obstructive lesion of the mesentery. At the time of operation the base of the mesentery was widened by dividing the peritoneal attachments of the ascending colon and displacing it to the left, as recommended by Ladd in 1936.3 With gastroschisis the mesentery is thickened and indurated, and it is possible that the mesenteric lymphatits were damaged during this dissection. However,
JournalofPediatricSurgery,
Vol26, No 1 (January),
1991: pp 106-107
GASTROSCHISIS,
MALROTATION,
107
& CHYLOUS ASCITES
the procedure has been used on previous occasions for malrotation with and without gastroschisis, without chylous ascites developing. In fact, Ladd’s procedure is not necessary for malrotation when it is associated with gastroschisis, because the risk of the thickened, edematous intestine undergoing volvulus is remote. Regarding the management of chylous ascites, this report confirms the value of prolonged bowel rest and TPN.’ A medium-chain triglyceride-rich formula (Portagen) was initially used in this infant, but the ascites worsened. Therefore, TPN was used. As the weeks pass, there may be increasing pressure on the surgeon to operate; this should be resisted as long as the TPN is being well tolerated and the serum albumin and lymphocyte count do not fall excessively. Two thirds of infants will respond to nonoperative therapy.J Further, there is no guarantee that a correctable abnormality will be encountered at laparotomy.
Laparotomy has been recommended after a trial of TPN of 4 to 6 weeks’; this may not be long enough, because from our experience and that of others it is clear that the lymphatic leak may not close for at least 6 weeks. Cochran et al* reported an infant who was treated with home TPN for 10 weeks, during which time the chylous ascites resolved. With increasing intraabdominal pressure, progressive respiratory distress may appear to necessitate paracentesis. However, following paracentesis the chylous ascites rapidly reaccumulates, seemingly to a greater extent each time, depleting the patient of serum proteins and lymphocytes. Left untapped, the increasing intraabdominal pressure has a tamponading effect, slowing the rate of lympatic leak, and infants with respiratory distress may adapt to the increased intraabdominal pressure. Therefore, paracentesis should be avoided for as long as possible, and reserved for severe respiratory compromise.
REFERENCES 1.
PK: Ascites, In Welch KJ, Randolph JG. Ravitch MM. et al (eds): Pediatric Surgery. Chicago, IL, Year Book, 1986 2. Cochran WJ, Klish WJ, Brown MR. et al: Chylous ascites in infants and children: A case report and literature review. J Pediatr Gastroenterol Nutr 4:668-673. 1985 Kottmeier
3. Ladd WE: Surgical diseases of the alimentary N Engl J Med 215:705-708, 1936 4. Unger SW, Chandler JG: Chylous ascites children. Surgery 93:455-461.1983
tract in infants. in infants
and
