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Laparoscopic Diagnostic Findings in Atypical Intestinal Malrotation In Original article 181 Laparoscopic diagnostic findings in atypical intestinal malrotation in children with equivocal imaging studies Maged Ismail, Rafik Shalaby, Ahmed Abdelgaffar Elgffar Helal, Samir Goda, Refat Badway, Abdelaziz Yehya, Ibrahim Gamaan, Mohammed Abd Elrazik, Mabrouk Akl and Omar Alsamahy Background Atypical presentations of intestinal congested mesenteric veins with lymphatic ectasia, malrotation are more common in older children with a generalized mesenteric lymphadenopathy, reversed diagnostic and therapeutic challenge. Upper relation of superior mesenteric artery and vein, and right- gastrointestinal (UGI) contrast study is essential for the sided small bowel and narrow mesenteric base. Four diagnosis of the majority of cases. Recently, laparoscopy patients had differed laparoscopic diagnosis. All the has been used in the management of malrotation. We procedures were completed laparoscopically. All the present our experience with laparoscopic management of patients achieved full recovery without intraoperative atypical presentations of intestinal malrotation in children, or postoperative complications. describing laparoscopic findings in these cases. Conclusion Laparoscopy permits direct evaluation and Patients and methods A total of 40 patients with atypical treatment of undocumented malrotation in children, with presentations of malrotation were included in this study. equivocal UGI contrast study. These newly described The main presentations were recurrent abdominal pain, laparoscopic findings are the key for the diagnosis of intermittent intestinal obstruction, recurrent bilious malrotation with atypical presentation. Ann Pediatr Surg vomiting, and failure to thrive. They all were subjected 11:181–184 c 2015 Annals of Pediatric Surgery. to thorough history taking, clinical examination, routine Annals of Pediatric Surgery 2015, 11:181–184 laboratory investigations, and UGI contrast study. No preoperative definitive diagnosis of malrotation was Keywords: Ladd’s procedure, laparoscopy, mesenteric lymphadenopathy, undocumented malrotation performed and all patients underwent laparoscopic evaluation. Pediatric Surgery Unit, Department of Pediatric Surgery, Al-Azhar University Hospitals, Cairo, Egypt Results Forty patients (25 males and 15 females) with a Correspondence to Ahmed A. Elgffar Helal, MD, Pediatric Surgery Unit, mean age of 7 ± 2.8 years were subjected to laparoscopy. Department of Pediatric Surgery, Al-Azhar University Hospital, 002 Cairo, Egypt Thirty-six patients (90%) were found to have definite Tel/fax: + 20 100 510 7647; e-mail: [email protected] laparoscopic findings in the form of markedly dilated Received 30 July 2013 accepted 15 June 2015 stomach and first part of duodenum, ectopic site of cecum, medial and low position of duodenojejunal junction, Introduction management of atypical presentations of intestinal Intestinal rotation abnormalities occur when normal malrotation, describing its laparoscopic findings. Some embryologic intestinal rotation and/or fixation of intest- of these laparoscopic findings, to the best of our inal mesentery fails to take place. Patients with malrota- knowledge, have not been described before. tion have a shortened mesenteric base that makes them susceptible to midgut volvulus, a condition that can Patients and methods result in significant morbidity and mortality. Accurate This study was conducted at Al-Azhar University and prompt diagnosis and timely surgical correction Hospitals and affiliated insurance hospitals in the period of malrotation are crucial to prevent catastrophic out- from May 2007 to October of 2012. It included 40 comes [1]. Classic malrotation with midgut volvulus is children presented with symptoms suggestive of intest- often discovered in a previously healthy term neonate. Up inal malrotation. They were subjected to clinical to 75% of the patients present during the first month of examination, laboratory investigation, and upper gastro- life. Another 15% present within the first year. Many intestinal (UGI) series. All patients were admitted, other cases will present less dramatically. Failure to hydrated with intravenous fluids, and scheduled for thrive, gastroesophageal reflux, early satiety, and mild laparoscopic exploration. Single intravenous dose of third abdominal discomfort are routinely reported. The diag- generation cephalosporin was given before surgery. nosis becomes more challenging with the older child or teenager because the symptoms are often very vague and seemingly unrelated to the abdomen. The role of Laparoscopic procedure laparoscopy in the pediatric patient with suspected The procedure was performed under general endotra- volvulus or an acute abdomen remains a controversy. cheal tube anesthesia with the patient positioned supine. However diagnostic laparoscopy with Ladd’s procedure A 5 mm incision was made in the supraumbilical site has been seen to be effective, even in the context of through which the first 5 mm trocar (for camera) was volvulus [2]. We present our experience with laparoscopic placed into the abdominal cavity by open Hasson’s 1687-4137 c 2015 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000469157.12636.6f Copyright r 2015 Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. 182 Annals of Pediatric Surgery 2015, Vol 11 No 3 technique. The intra-abdominal pressure was maintained Table 1 The demographic data and laparoscopic diagnostic from 8 to 12 mmHg according to age. Two additional 3 mm findings of patients trocars were placed under direct vision in the right and left Number of patients 40 Sex mid to lower abdominal wall for the working instruments. Male 25 Exploratory laparoscopy was performed, and the intestinal Female 15 rotation abnormality was evaluated. Special attention was Age (years) Mean 7 ± 2.8 paid to identify the position of the cecum, duodenojejunal Range 2–14 junction (DJJ), and the size of the stomach. The operative Presenting symptoms N (%) Recurrent abdominal pain 30 (75) table was tilted to different positions during the procedure Intermittent intestinal obstruction 36 (90) according to the need. Ladd’s bands were identified and Recurrent bilious vomiting 36 (90) divided with laparoscopic adhesiolysis. Care must be taken Failure to thrive 40 (100) Laparoscopic findings throughout the procedure, carefully identifying and pre- Ectopic site of cecum 36 (90) serving blood supply without causing torsion. Then the Subhepatic 30 (7) entire bowel is examined, to exclude other obstructive Central 5 (12.50) Left sided 1 (2.50) bands or kinks. The base of the mesentery is assessed, and Hugely dilated stomach 30 (75) ifthelengthwasperceivedaslessthanhalfofthe Abnormal position of DJJ 36 (90) Ladd’s bands 30 (75) transverse diameter of the peritoneal cavity, base was Mesenteric lymphadenopathy with congested mesenteric vessels 36 (90) widened by incising the peritoneum and gaining length Chronic appendicitis 2 (5) between the vascular arcades of the superior mesenteric Annular pancreas 1 (2.5) Negative exploration 1 (2.5) artery. The small bowel is then placed on the right of the abdomen and the colon placed on the left. DJJ, duodenojejunal junction. This study was approved by the research ethical committee at our hospital, with a detailed written informed consent signed by the parents. Discussion Atypical presentations of intestinal malrotation are more Results commonly seen in older children. The symptoms are more Clinical presentation chronic, intermittent, and result from partial or intermit- A total of 40 patients with atypical symptoms of intestinal tent duodenal obstruction or a chronic midgut volvulus. malrotation were included in this study. They were 25 These include abdominal pain and vomiting associated males and 15 females, with a mean age of 7 ± 2.8 years with weight loss or failure to thrive, and other nonspecific (range = 2–14 years). The demographic data and laparo- gastrointestinal complaints. These patients with vague scopic diagnostic findings of patients are showed abdominal complaints provide a diagnostic and therapeutic in Table 1. All patients’ symptoms were dating since challenge [3]. Maxson and colleagues report that the most birth. Preoperative UGI contrast study was equivocal (we frequent complaints of intestinal malrotation are chronic consider it equivocal if the final report explicitly stated vomiting (68%), intermittent colicky abdominal pain uncertainty or if differential diagnosis was considered). (55%), diarrhea (9%), hematemesis (5%), and constipation No preoperative definitive diagnosis could be reached. (5%) [4]. Partial volvulus leads to mesenteric venous and lymphatic obstruction and subsequently impairs nutrient Laparoscopic findings absorption [2]. Absorption and nutrient transport can be Thirty-six patients (90%) were found undergoing laparo- impaired by venous and lymphatic stasis, leading eventually scopy having definitive laparoscopic findings in the form of to protein-caloric malnutrition in severe cases of long- huge dilated stomach and first part of duodenum, ectopic standing incomplete rotation with partial obstruction. An site of cecum central in the abdomen or under the liver increased predisposition to infection has also been (Table 1 and Fig. 1a), generalized mesenteric lymphadeno- observed. Failure to suspect this diagnosis has resulted in pathy (Fig. 1b), medial and low position of DJJ, Ladd’s band dietary manipulation and even psychiatric evaluation in (Fig. 2), congested mesenteric veins
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