Journal of Pediatric Surgery 53 (2018) 537–539
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Congenital chylous ascites in infants: another presentation of intestinal malrotation☆,☆☆ Li Long a,b,⁎, Chen Zhen a,b, Wei Yandong a, Dong Ning a, Li Qi a, Gao Qing a a b
Department of Pediatric Surgery, Capital Institute of Pediatrics-Peking University Teaching Hospital, Beijing, 100021, People's Republic of China Medical Department, Peking University, Beijing, 100000, People's Republic of China
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Article history: Received 27 January 2017 Received in revised form 29 May 2017 Accepted 30 May 2017 Key words: Chylous ascites Intestinal malrotation Infant
a b s t r a c t Objective: The cause of the chylous ascites in infants isn't completely clear. The purpose of this study is to discuss our experience of recognition of intestinal malrotation as a cause of congenital chylous ascites in infants. Methods: Medical information of 10 infants with chylous ascites, who were admitted to the hospital between 2001 and 2014, was retrospective analyzed. Preoperatively, all patients underwent a period of conservative treatment. Results: We found that nine of ten patients with intestinal malrotation, six of them underwent laparoscopic Ladd's procedure and three patients underwent open Ladd's procedure. The remaining one patient suffered from mesenteric lymph nodes rupture and laparoscopic resection was performed. The cylous ascites subsided in all patients after the surgery and no significant recurrence was encountered during follow-up time. Conclusions: Our study demonstrates that congenital chylous ascites could be caused by intestinal malrotation, causing the obstruction of the lymphatic flow in the mesenteric lymphatic channels. Ladd's procedure maybe a safe and effective treatment for infantile intractable chylous ascites. Type of study: Treatment study. Level of evidence: Level IV. © 2017 Elsevier Inc. All rights reserved.
Congenital chylous ascites is rare, but it is the most common cause of infants' peritoneal effusion [1]. Prenatal ultrasound may suggest the existence of chylous ascites and paracentesis would confirm the diagnosis. The pathogenesis of chylous ascites in infants is poorly understood, but recent work has shone light on some of the etiologies: lymphatic abnormalities, trauma, and obstruction [2–4]. Intestinal malrotation, an uncommon cause of chylous ascites, has been reported previously [5]. If chylous ascites don't respond well to non-operative management, which we regard as refractory chylous ascites(RCA), surgery is recommended as the mortality rate is as high as 13% in infants [6]. The outcome of surgical treatment of infants RCA is not clear. The aim of the present study is to report our initial experience on recognition of intestinal malrotation as a cause of the congenital chylous ascites. This could be effectively treated by Ladd procedure. 1. Material and methods Ten patients, seven boys and three girls, with refractory infant chylous ascites, were admitted into our center between October 2001 and ☆ Funding source: There was no funding source for this study. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit for publication. ☆☆ Conflict of interest statement: None declared. ⁎ Corresponding author at: Yabao Road2#, Chaoyang, Beijing, 100021, China. Tel.:+86 13261195776. E-mail address:
[email protected] (L. Long). http://dx.doi.org/10.1016/j.jpedsurg.2017.05.031 0022-3468/© 2017 Elsevier Inc. All rights reserved.
December 2014. Their age ranged between 22 days and 10 months, with an average of 4.7 months. The chylous ascites was diagnosed by abdominal paracentesis and laboratory examination. The conservative treatment, including fasting, low-fat diet, and intravenous nutrition for more than 4 weeks, was carried out before the operations. Fasting combined with parental nutrition reduced the abdominal distention. However, once the feeding was resumed, the chylous ascites recurred. The radiography of upper and lower digestive tract, computed tomography (CT), and magnetic resonance imaging (MRI) were routinely performed preoperatively. Nine out of 10 patients with intestinal malformation underwent Ladd operations. The remaining patient was found to have a lymph leak from ruptured lymph nodes and underwent the lymph node resection. The abdominal drain was placed, and low-fat milk feeding was restarted after the operation. When the daily drainage was reduced to less than 50 mL, the drain was removed. All patients underwent regular follow-up and abdominal ultrasound examination. The growth and development of patients were recorded.
2. Result 2.1. Clinical characteristics and preoperative findings The ascites was detected in 7 out of 10 infants during the prenatal ultrasound examination. The bilateral patent processus vaginalis with hydrocele was found in six patients. The hydrocele was found in all seven boys, and inguinal hernia was found in two girls. Besides severe
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Fig. 1. Barium enema revealing the location of ileocecum in the right upper quadrant.
abdominal distention, no patient presented with a history of bilious vomiting. On abdominal paracentesis, milky white chylemia was found in 9 patients, and yellowish chylemia in one patient. Ultrasound examination indicated a normal relationship between the superior mesenteric artery and the superior mesenteric vein and no sign of intestinal volvulous was found. CT and MRI revealed a large amount of ascites. The upper digestive tract radiography showed positioning of the duodenojejunal flexure to the left of the spine at the level of the duodenal bulb and no appearance of obstructed duodenum. A barium enema showed a high cecum in the right upper quadrant of the abdomen in seven of ten patients (Fig. 1), and in the right lower quadrant of the abdomen in the other three patients. 2.2. Intraoperative findings The operations were performed using laparoscopic approach in 7 patients and by upper Medline incision in the remaining three. In 9 of 10 cases, incomplete rotation of cecum with an obvious Ladd ligament was noticed, and a higher cecum position was found in 7 patients. In the other 2 case, the cecocolic limb was fixed only by a Ladd's band on the ascending colon with the mobile cecum. The position of the ligament of Treitz was on the left of the spine at the level of the pancreas. The Ladd's band was seen traveling across the root of the mesentery. Chylous leak secondary to lymphatic obstruction in the mesentery by the compression from the Ladd's band was found. The bowel and mesenteric volvulus was not encountered in this group of the patients. No patients were found to suffered lymphangiectasia, cystic hygroma of the abdomen, lymphatic dysplasia, or inflammatory enteritis (Fig. 2). Ladd's bands from the cecum to the right abdominal wall was completely divided and the cecocolic limb was mobilized and fixed to the left
Fig. 3. Chyle drainage (in ml/d) after operation.
abdominal wall. The dissection then continued to the base of the mesentery by incising the bands on the anterior mesenteric leaflet. In this way, the compression of the mesenteric radix was completely eliminated. A drain was placed in the pelvic cavity after operation. Laparoscopic exploration revealed the ileocecum located in the right lower quadrant of the abdomen without the Ladd's band in one patient. However, the hypertrophic lymph nodes at the mesentery of the intestine was found. One of the lymph nodes ruptured with the lymphatic fluid leakage. All the ruptured lymph nodes were excised laparoscopically, and the mesentery was repaired using absorbable sutures. 2.3. Postoperative follow-ups The patients were followed up for 3.5 years(range 6 months to 9 years). Shortly after the operations, and the daily amount of abdominal drainage was gradually reduced to less than 50 mL within 2 weeks, and mean time that we left the abdominal drainage was 7.6 d (3–14 d) (Fig. 3). Eight out of 10 patients had no recurrent abdominal distension or ascites after the drain was removed. Mild abdominal distension was observed in one patient when the drainage tube was removed, and ultrasound showed a small amount of ascites. The patient was tolerant to oral low-fat milk powder. The abdominal distension disappeared 1 month later, and ascites also disappeared 3 months later. In addition, another one patient had intermittent mild abdominal distension at the early postoperative stage, which exacerbated into moderate abdominal distension with fever, although the abdominal distension decreased when the fever was controlled. The abdominal distension completely disappeared when the low-fat milk powder feeding lasted for 3 months, and ultrasound detected no ascites. No recurrent ascites was reported till date. The hospital stay ranged between 7 and 15 days. No incision dehiscence, wound infection, intra-abdominal infection, adhesion or obstruction of the intestine was observed. No issue related to development or nutrition were reported up to date. 3. Discussion
Fig. 2. Laparoscopic operation revealing the location of ileocecum (indicated by arrow) in the right upper quadrant.
Data on infantile chylous ascites are scanty [7,8]. The present findings indicated that a majority of infantile chylous ascites was congenital. Manifestations of abdominal effusion and bilateral patent process vaginalis were observed during the fetal stage. This study indicated that 9 out of 10 patients had congenital intestinal malrotation, and 1 patient had a rupture of mesenteric lymph nodes. Intestinal malrotation is the most common congenital malformation in children [9,10]. The counterclockwise rotation of the intestine was
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arrested at the early embryonic stage when the intestine returned to the abdominal cavity; the cecum was fixed to the right upper quadrant and failed to move to the right lower quadrant, which led to abnormal hypertrophic Ladd's band. Therefore, it was believed that the duodenum was pressed by the Ladd ligament leading to the obstruction. In addition, the mesentery failed to expand and was fixed to the right lower quadrant, which likely leads to the midgut volvulus. The present study indicates that intestinal malrotation was closely associated with congenital infant chylous ascites, with 90% of the patients having intestinal malrotation. The Ladd procedure was effective for treatment of chylous ascites, implying that intestinal malrotation was one of the potential causes of ascites [11]. In the intestinal malrotation, the Ladd ligament travels cross the duodenum and mesentery. In the mesenteric root, the following important structures exist, including the superior artery and vein, lymphatic and nerve systems. The superior mesenteric arterial pressure is highest, followed by that of the superior mesenteric vein, and the mesenteric lymph fluid flows at the lower pressure. If the mesenteric root is compressed at the different levels of pressures, it may lead to different outcomes: severe compression from the two to three complete revolutions may result in complete obstruction of the superior mesenteric artery and bowel gangrene; moderate compression from the revolution at the pressure level between the superior mesenteric artery and vein may lead to an obstruction of the superior mesenteric vein and subsequently results in bowel congestion and bloody ascites. However, our study demonstrated that a mild compression from the Ladd's band, which would not lead to the obstructions of the superior mesenteric artery, or vein and the duodenum, causes the lymphatic flow obstruction and leakage. In our study 9 of the 10 infant with chylous ascites presents with the intestinal malrotation and effectively treated by Ladd's procedure. We speculate that Ladd's band, fixed at the right upper quadrant retroperitoneum, forms a mild compression band across the mesenteric root, leading to mesenteric lymphatic obstruction. As the lymphatic flow pressure increases, chyle leakage and chylous ascites ensue. Lymphatic vessels with a thin wall are easily obstructed and its rupture results in lymph extravasation to form ascites or chyloperitoneum. The present study demonstrates that in seven out of nine patients with intestinal malrotation, the ascites disappeared immediately after the Ladd's procedure. Delayed disappearance of cylous ascites in the remaining two cases after the surgery indicated that
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damaged lymphatic vessels by Ladd's band compression or by the surgery might need a period of time for re-canalization. All patients underwent fasting and intravenous nutrition preoperatively. Although the amount of ascites reduced during the parenteral nutrition, the chylous ascites recurred once the normal milk feeding was restarted. Therefore, we believe that refractory chylous ascites might not ameliorate without decompression from intestinal malrotation, and surgical operation is effective in treating infantile refractory chylous ascites. In this study, seven out of nine patients with intestinal malrotation were diagnosed preoperatively by means of barium enema. Another two patients underwent surgical exploration and showed intestinal malrotation with a mobile cecum, indicating that surgical exploration was a reliable way to diagnose intestinal malrotation. Acknowledgments We thank those patients who supported our study and authors who provided us with the full-text. References [1] Te Pas AB, vd Ven K, Stokkel MP, et al. Intractable congenital 1 chylous ascites. Acta Paediatr 2004;93(10):1403–5. [2] Herman TE, Siegel MJ. Congenital chylous ascites. J Perinatol 2009;29(2):178–80. [3] Bellini C, Ergaz Z, Radicioni M, et al. Congenital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study. Lymphology 2012;45(3):91–102. [4] Ford EG, Senac Jr MO, Srikanth MS, et al. Malrotation of the intestine in children. Ann Surg 1992;215(2):172–8. [5] Hoeffel JC. Intestinal malrotation in infants. AJR Am J Roentgenol 1993;160(6): 1362–3. [6] Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ 2013;347:f6949. [7] Li SL, Li YC, Yu ZW, et al. Laparoscopic diagnosis and treatment for intestinal malrotation in children[J]. Chin J Pediatr Surg 2008;29(10):577–9. [8] Yan XQ, Zheng NN, Huang SG, et al. Meta-analysis for laparoscopic surgery in treatment of intestinal malrotation. Chin J Gen Surg 2013;22(10):1314–8. [9] Koh YX, Chng JK, Tan HC, et al. Intestinal torsion causing chylous ascites: a rare occurrence. Singapore Med J 2013;54(4):e88–90. [10] Wei Feng, Chen YW. The treatment of infant chylous ascites with 7 cases. J Clin Pediatr Surg 2013;12(3):219–20. [11] Alliët P, Young C, Lebenthal E, et al. Chylous ascites: total parenteral nutrition as primary therapeutic modality[J]. Eur J Pediatr 1992;151(3):213–4.