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Intestinal rotation abnormalities without volvulus: the role of laparoscopy
Intestinal Rotation Abnormalities W i t h o u t Volvulus: T h e Role of Laparoscopy Mark V. Mazziotti, MD, Steven M. Strasberg, MD, FACS, and Jacob C. Langer, MD, FACS Background: Intestinal rotation disorders may be discovered during investigation for abdominal symptoms. Two questions are raised in this setting: are the patient's symptoms from the rotation abnormality, and is the base of the small bowel mesentery so narrow that it places the patient at risk for midgut vol~lus? Previously, laparotomy was necessary to answer these questions, and then it was necessary to do a Ladd procedure and appendectomy if necessary.
2 days). Resolution of symptoms was seen in 5 of the 7 patients, with a median foUowup of 15 months.
Conclusions: Laparoscopy is an excellent technique for the evaluation and definitive management of patients without midgut volvulus with intestinal rotation abnormalities. (J Am Coll Surg 1997;185:172-176. © 1997 by the American College of Surgeons) Intestinal malrotation usually presents within the first year o f life with bilious emesis or a b d o m i n a l distention f r o m m i d g u t volvulus (1). O t h e r patients might p r e s e n t without m i d g u t volvulus later in life, usually during c h i l d h o o d or adolescence. These patients often have vague long-standing abd o m i n a l complaints (2), a n d an u p p e r or lower intestinal contrast study might d e m o n s t r a t e an abnormality of intestinal rotation. In an individual case it can be u n c l e a r w h e t h e r the a b d o m i n a l complaints are the result o f the rotation abnormality, or what the m a g n i t u d e of risk for m i d g u t volvulus might be. T r e a t m e n t for this g r o u p o f patients is c o n t r o versial, with m o s t a u t h o r s a d v o c a t i n g L a d d procedure because of the potential morbidity and m o r t a l i t y o f m i d g u t volvulus. B e c a u s e o f the expanding applications and potential perioperative b e n e f i t s o f minimally invasive surgery, we assessed this t e c h n i q u e for t h e e v a l u a t i o n a n d t r e a t m e n t o f a g r o u p o f p a t i e n t s with r o t a t i o n a b n o r m a l i t i e s w i t h o u t volvulus.
Study Design: We used laparoscopic surgery to evaluate seven patients, ages 4 days to 23 years of age (median age 7 years), when upper gastrointestinal series revealed intestinal rotation abnormalities without volvulus.
Results: Two patients had nonrotation. One had Ladd's bands across the duodenum that were divided, and the appendix was removed. The other had diffuse peritoneal soilage from a ruptured appendix; irrigation and appendectomy were performed. Three patients had duodenal malrotation and underwent laparoscopic Ladd procedure and appendectomy. Two patients had combined duodenal and cecal malrotation. One of these patients had a previous appendectomy for what in retrospect was primary peritonitis; malrotation was confirmed radiologically after the operation. She underwent a laparoscopic Ladd procedure 3 months later. The other patient was believed to have combined duodenal and cecal malrotation based on radiographic studies performed during workup for gastroesophageal reflux. At laparoscopy the small bowel mesentery was believed to have a broad enough base to prevent midgut volvulus, and an appendectomy was done. No patient required conversion to an open procedure. The sole complication was intra-abdominal abscess in the child with ruptured appendicitis that required prolonged hospitalization and operative abscess drainage. Operative times ranged from 1.25-3.25 hours (median 2 hours). Time to a regular diet was 1-20 days (median
Methods Six patients with chronic a b d o m i n a l complaints a n d o n e n e w b o r n with bilious vomiting were f o u n d to have intestinal rotation abnormalities by u p p e r gastrointestinal (GI) contrast series. Some also h a d the rotation abnormality c o n f i r m e d by lower GI contrast study. All patients u n d e r w e n t laparoscopic evaluation with p o r t p l a c e m e n t as seen in Figure 1, although n o t all ports were placed in all patients. An o p e n insertion techn i q u e was p e r f o r m e d using a H a s s o n trocar
Received December 30, 1996; Revised April 4, 1997; Accepted May 8, 1997. From the Washington University School of Medicine, Department of Surgery, St. Louis, MO. Correspondence address: Jacob C. Langer, MD, Room 5W12, St. Louis Children's Hospital, One Children's Place, St. Louis, MO 63110. © 1997 by the American College of Surgeons Published by Elsevier Science Inc.
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p e r f o r m e d . T h e L a d d p r o c e d u r e consisted of: 1. c o m p l e t e mobilization of the right colon, which was t h e n reflected to the left, 2. complete mobilization o f the d u o d e n u m , including division of Ladd's bands a n d straightening o f the C-loop, 3. division o f adhesions a r o u n d the superior mesenteric artery to b r o a d e n the mesenteric base, a n d 4. appendectomy. Age at the time o f diagnosis r a n g e d f r o m 4 days to 23 years ( m e d i a n 7 years). Clinical features i n c l u d e d vague c h r o n i c a b d o m i n a l pain (4), early satiety (1), n a u s e a (1), vomiting (3), a n d peritoneal signs (2). T h e patients were divided into three groups d e p e n d i n g on the type o f intestinal rotation abnormality identified: 1. n o n r o t a t i o n (Fig. 2A), 2. d u o d e n a l malrotation (Fig. 2B), and 3. c o m b i n e d d u o d e n a l and cecal malrotation (Fig. 2C). T h e i r clinical courses follow.
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FIG l. Port placement for laparoscopic evaluation and treatment of intestinal rotational abnormalities. t h r o u g h a periumbilical incision. T h e a b d o m e n was insufflated to a pressure o f 8 - 1 5 m m Hg, d e p e n d i n g on the age of the patient. Exploratory laparoscopy was p e r f o r m e d , a n d the intestinal rotation abnormality was evaluated. T h e base o f the small bowel m e s e n t e r y was assessed by identifying the d u o d e n o j e j u n a l j u n c t i o n a n d the ileocecal j u n c t i o n . If the length of the mesenteric base was perceived as less t h a n half o f the transverse diameter o f the peritoneal cavity, a L a d d p r o c e d u r e was
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Results
Nonrotation Patient 1. A 16-year-old girl p r e s e n t e d with a
1-month history o f vague a b d o m i n a l pain. During h e r evaluation she h a d an u p p e r GI contrast series a n d a b a r i u m e n e m a , that revealed that h e r intestines were in a state o f nonrotation. She was followed clinically because she was not at risk for m i d g u t volvulus. She subsequently p r e s e n t e d with an acute exacerbation o f a b d o m i n a l pain with the d e v e l o p m e n t o f peritoneal signs. At laparoscopic exploration n o n r o t a t i o n was c o n f i r m e d a n d a rup-
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173
C
FIG 2. (A) Nonrotation, (B) duodenal malrotation, (C) combined duodenal and cecal malrotation.
174 J AM COli SURG AUGUST1997
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tured appendix was f o u n d b e h i n d the right ovary. Her postoperative course was complicated by intra-abdominal abscess formation requiring operative drainage. She recovered, but 1 m o n t h after discharge she was readmitted for a partial small bowel obstruction that did n o t r e s p o n d to conservative m a n a g e m e n t a n d required laparotomy. She was discharged 9 days later and has since d o n e well, with no complaints, 16 m o n t h s later. Patient 2. A 23-year-old w o m a n presented with long-standing lower abdominal pain, n o t associated with vomiting. She h a d been extensively evaluated and treated with several medical regimens without relief. An u p p e r GI series d e m o n s t r a t e d nonrotation of the bowel. Laparoscopic exploration confirmed a left-sided colon and revealed several congenital bands crossing over the duoden u m . These were divided, and the mesentery was t h e n e x a m i n e d and f o u n d to be broad-based. An a p p e n d e c t o m y was c o m p l e t e d without complications, and the patient was discharged on the day after surgery. The patient had early i m p r o v e m e n t in symptoms, b u t 15 m o n t h s later she had the same complaints that she had before the procedure. Duodenal mabvtation Patient 3. A 61½-year-old boy with multiple med-
ical problems including spina bifida, hydrocephalus, coarctation of the aorta, and a solitary right kidney presented with intermittent nonbilious Vomiting since infancy. The vomiting was postprandial and n o t associated with pain. An u p p e r GI series revealed no evidence of gastroesophageal reflux and showed the ligament of Treitz to the right o f the spine with the cecum in n o r m a l position. He u n d e r w e n t laparoscopic exploration that confirmed the position of the ligament of Treitz in the right u p p e r quadrant with the ileocecal j u n c t i o n in the right lower quadrant. Numerous Ladd's bands in the right u p p e r q u a d r a n t were divided, a n d an a p p e n d e c t o m y was perf o r m e d laparoscopically. The presence of a ventriculoperitoneal shunt did n o t pose any technical problems d u r i n g the procedure. He tolerated a regular diet a n d was discharged on the third postoperative day. Thirteen m o n t h s after discharge he h a d intermittent vomiting, but his m o t h e r n o t e d that the frequency decreased. Patient 4. A 17-year-old girl h a d several m o n t h s of intermittent u p p e r abdominal pain that was usually postprandial or nocturnal and was n o t associated with vomiting. U p p e r GI series showed that the ligament of Treitz was in the right u p p e r q u a d r a n t with n o r m a l cecal position. T h e patient u n d e r w e n t exploratory laparoscopy, and the ce-
c u m was f o u n d to be quite mobile. T h e lateral peritoneal attachments of the ascending colon were divided up to the hepatic flexure, and the ascending colon was mobilized to the left side of the a b d o m e n . The d u o d e n u m was then visualized, and adhesions between the d u o d e n u m a n d colon were divided, placing the bowel in complete nonrotation. A laparoscopic a p p e n d e c t o m y completed the procedure, and the patient was discharged h o m e on the second postoperative day. She h a d no complaints 15 m o n t h s later. Patient 5. A 2,845 g boy was born by spontaneous vaginal delivery after an uncomplicated, term pregnancy. Soon after birth he developed bilious vomiting with each feeding. His a b d o m e n was dist e n d e d but n o n t e n d e r . Supine and u p r i g h t plain radiographs of the a b d o m e n d e m o n s t r a t e d dilated small bowel loops with air fluid levels and a paucity of air in the rectum. A Hypaque e n e m a d e m o n s t r a t e d a n o r m a l cecum in the right lower quadrant. An u p p e r GI study revealed a low-lying d u o d e n o j e j u n a l j u n c t i o n and no evidence of obstruction. Rectal biopsy was normal. O n his f o u r t h day of life he u n d e r w e n t laparoscopic exploration, and, despite the a b n o r m a l position of the l i g a m e n t of Treitz, his small bowel mesentery was greater t h a n half the transverse diameter of his a b d o m e n . N o L a d d ' s b a n d s were found. Postoperatively he tolerated feedings without difficulty and was discharged 2 days later. He is now 10-months old and has since h a d no difficulty feeding. Combined duodenal and cecal malrotation Patient 6. A 7-year-old, previously healthy girl presented with signs and symptoms suggesting perforated appendicitis. She u n d e r w e n t exploration t h r o u g h a right lower q u a d r a n t incision, and a moderate a m o u n t of cloudy fluid was seen, which ultimately was culture positive for Streptococcus. T h e cecum a n d a p p e n d i x were f o u n d in the right u p p e r q u a d r a n t a n d a p p e a r e d injected w i t h o u t obvious p e r f o r a t i o n . No o t h e r perforations or abnormalities could be identified on exploration, and the appendix was n o t inflamed on pathologic examination. Malrotation was confirmed postoperatively with an u p p e r GI series, and the patient u n d e r w e n t laparoscopic exploration 3 m o n t h s later. There were few adhesions from her previous peritonitis, and a laparoscopic Ladd p r o c e d u r e was performed. The mesentery was mobilized from the ligament of Treitz to the ileocecal valve a n d a broad-based mesentery was formed. The small bowel was then placed on the right side of the a b d o m e n with the large bowel placed on the left. T h e patient was discharged
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INTESTINALROTATION
h o m e on the first postoperative day a n d h a d n o c o m p l a i n t s 16 m o n t h s after laparoscopic exploration. Patient 7. A 3-year-old girl with chronic cough a n d mild gastroesophageal reflux u n d e r w e n t an u p p e r GI series that revealed the ligament of Treitz to be just to the left of the spine and a cecum elevated from its normal position in the right iliac fossa. Because of the incomplete rotation, it was unclear whether the patient was at risk for m i d g u t volvulus, so laparoscopic exploration was r e c o m m e n d e d . During the p r o c e d u r e the liga m e n t of Treitz and ileocecal j u n c t i o n were identified. T h e distance between t h e m was greater than half of the transverse diameter of the peritoneal cavity, and we t h o u g h t it was broad e n o u g h to prevent m i d g u t volvulus. A laparoscopic appendectomy was p e r f o r m e d to complete the procedure. T h e patient was discharged on the day after surgery and had no additional coughing or other complaints 16 m o n t h s later. Overall results. Median operative time in this g r o u p of.patients was 2.0 hours (range 1.25-3.25 hours). They r e s u m e d a regular diet after a median of 2 days (range 1-20 days) a n d were discharged a m e d i a n of 2 days after surgery (range 1-21 days). None of the operations required conversion to an o p e n procedure. Symptoms completely resolved in 5-7 patients and m e d i a n followup was 15 m o n t h s (range 10-16 months). Discussion
Malrotation of the intestine can result in abnormal mesenteric attachments and a narrowed mesenteric base, which places the patient at risk of m i d g u t volvulus. T h e treatment of malrotation is based on the classic description by Ladd (3), a n d includes: 1. laparotomy with reduction of m i d g u t volvulus, if present, 2. division of peritoneal bands obstructing the d u o d e n u m , 3. p l a c e m e n t of the small and large bowel in a state of nonrotation, a n d 4. appendectomy. The principles and indications for operation have r e m a i n e d the same since the time of Ladd's address to the New H a m p s h i r e Medical Society in 1936. Most patients with malrotation seem to present with symptoms in the first 6 m o n t h s of life, with bilious vomiting in the majority of cases (4). Intestinal rotational abnormalities can be present in m u c h older patients who are ultimately investigated with intestinal contrast studies for various abdominal symptoms. Whether these symptoms are secondary to the intestinal rotational abnormality or simply coincidental is often unclear (5).
ABNORMALITIES
WITHOUT VOLVULUS 175
It is well known that a spectrum of rotational abnormalities can exist, including nonrotation, d u o d e n a l malrotation with n o r m a l cecal position, and c o m b i n e d malrotation of the d u o d e n u m and cecum. Radiographically, n o r m a l fixation of the intestines can be d e t e r m i n e d based on the positions of the d u o d e n o j e j u n a l j u n c t i o n and the cecum. T h e duodenojejunal j u n c t i o n is normally located to the left of the second lumbar vertebra, and the cecum is normally located in the right iliac fossa. Using these radiographic positions as standards, Stringer has classified all types of intestinal rotation abnormalities into Type I (nonrotation), Type II ( d u o d e n a l malrotation), or Type III (combined d u o d e n a l a n d cecal malrotation) (6). T h e relative increased mobility of various structures in the infant and child can confuse the issue. For instance, the cecum generally becomes fixed in the right lower quadrant, but in up to 36% of patients it can be on a mobile mesentery (7). Also, t h e laxity in the attachments of the distal duoden u m to the r e t r o p e r i t o n e u m in neonates a n d in children up to 4 years of age can result in a significant mobility of the d u o d e n o j e j u n a l junction, including mobility to the right of the spine (8). Others have r e p o r t e d chronic abdominal pain in patients with normally rotated but nonfixed intestines (9). Thus, because of the laxity of the various retroperitoneal attachments, it can be difficult to distinguish an abnormality at risk for m i d g u t volvulus from an abnormality n o t at risk using radiologic studies alone. Patients with vague abdominal complaints and intestinal contrast studies that suggest an intestinal rotational abnormality provide a diagnostic and therapeutic d i l e m m a for the surgeon. Because of the risk of m i d g u t volvulus, these patients must be expeditiously evaluated and treated appropriately. Patients with even a question of this diagnosis have historically u n d e r g o n e l a p a r o t o m y with L a d d p r o c e d u r e , if the m e s e n t e r i c base is narrow, a n d a p p e n d e c t o m y if the c e c u m is in an a b n o r m a l position. We believed that minimally invasive surgical t e c h n i q u e s would be able to accomplish the identical evaluation a n d t r e a t m e n t of this g r o u p of patients but without the associated morbidity of a laparotomy. Although others have described a laparoscopic approach in children with suspected volvulus (10), we have c o n t i n u e d to use laparotomy for these patients. Nonrotation of the intestine provides a particularly challenging circumstance. These patients would n o t be expected tO have symptoms secondary to their rotation abnormality because the Ladd p r o c e d u r e places the intestines in a position of
176 J AM COLL SUI~G AUGUST1997
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nonrotation, but congenital bands and adhesions still can be present in nonrotation and can potentially result in symptoms. This is illustrated by patient 2, in whom duodenal, bands were noted and divided at exploration. This patient had continued postoperative complaints despite this intervention. The advantage of laparoscopy in a patient with chronic abdominal symptoms is that i t c a n be used to diagnose and eliminate a mechanical cause with minimal morbidity and a reduced risk of adhesion formation. The persistence of pain in this setting implies a nonmechanical etiology. Our data show that using a minimally invasive approach, the mesenteric base can be directly evaluated, and a determination of adequate width to prevent midgut volvulus can be made. Furthermore, an appendectomy or Ladd procedure can also be performed laparoscopically, if indicated. The median operative time was acceptable, and n o n e of the patients required conversion to an open procedure. Except for one child who had complications from a r u p t u r e d appendix, there was no morbidity, and hospital stay was very short. In five of our seven patients, the preoperative symptoms resolved. In an additional patient the frequency of vomiting was noticeably reduced after surgery.
Because of our experience and success with evaluating and treating these patients, we feel that laparoscopy is an excellent technique for the management of intestinal rotational abnormalities without volvulus. References 1. Ford EG, Senac MO Jr, Srikanth MS, and Weitzman ~. Malrotation of the intestine in children. Ann Surg 1992;215: 172-8. 2. Powell DM, Otherson HB, and Smith CD. Malrotation of the intestines in children: the effect of age on presentation and therapy. J Pediatr Surg 1989;24:777-80. 3. Ladd WE. Surgical diseases of the alimentary tract in infants. N Engl J Med 1936;215:705-8. 4. Torres AM, and Ziegler MM. Malrotation of the intestine. WorldJ Surg 1993;17:326-31. 5. Berardi RS. Anomalies of midgut rotation in the adult. Surg Gynecol Obstet 1980;151:113-24. 6. Stringer DA. Pediatric gastrointestinal imaging. Philadelphia: B.C. Decker, Inc., 1989. 7. Steiner GM. The misplaced caecum and the root of the mesentery. Br J Radiol 1977;51:406-13. 8. Katz ME, Siegel MJ, Shackelford GD, and McAllister WH. The position and mobility of the duodenum in children. Am J Radiol 1987;148:947-51. 9. Janik JS, and Ein SH. Normal intestinal rotation with nonfixation: a cause of chronic abdominal pain. J Pediatr Surg 1979;14:670-4. 10. Van der Zee DC, and Bax NMA. Laparoscopic repair of acute volvulus in a neonate with malrotation. Surg Endosc 1995;9: 11'23-4.
2 days). Resolution of symptoms was seen in 5 of the 7 patients, with a median foUowup of 15 months.
Conclusions: Laparoscopy is an excellent technique for the evaluation and definitive management of patients without midgut volvulus with intestinal rotation abnormalities. (J Am Coll Surg 1997;185:172-176. © 1997 by the American College of Surgeons) Intestinal malrotation usually presents within the first year o f life with bilious emesis or a b d o m i n a l distention f r o m m i d g u t volvulus (1). O t h e r patients might p r e s e n t without m i d g u t volvulus later in life, usually during c h i l d h o o d or adolescence. These patients often have vague long-standing abd o m i n a l complaints (2), a n d an u p p e r or lower intestinal contrast study might d e m o n s t r a t e an abnormality of intestinal rotation. In an individual case it can be u n c l e a r w h e t h e r the a b d o m i n a l complaints are the result o f the rotation abnormality, or what the m a g n i t u d e of risk for m i d g u t volvulus might be. T r e a t m e n t for this g r o u p o f patients is c o n t r o versial, with m o s t a u t h o r s a d v o c a t i n g L a d d procedure because of the potential morbidity and m o r t a l i t y o f m i d g u t volvulus. B e c a u s e o f the expanding applications and potential perioperative b e n e f i t s o f minimally invasive surgery, we assessed this t e c h n i q u e for t h e e v a l u a t i o n a n d t r e a t m e n t o f a g r o u p o f p a t i e n t s with r o t a t i o n a b n o r m a l i t i e s w i t h o u t volvulus.
Study Design: We used laparoscopic surgery to evaluate seven patients, ages 4 days to 23 years of age (median age 7 years), when upper gastrointestinal series revealed intestinal rotation abnormalities without volvulus.
Results: Two patients had nonrotation. One had Ladd's bands across the duodenum that were divided, and the appendix was removed. The other had diffuse peritoneal soilage from a ruptured appendix; irrigation and appendectomy were performed. Three patients had duodenal malrotation and underwent laparoscopic Ladd procedure and appendectomy. Two patients had combined duodenal and cecal malrotation. One of these patients had a previous appendectomy for what in retrospect was primary peritonitis; malrotation was confirmed radiologically after the operation. She underwent a laparoscopic Ladd procedure 3 months later. The other patient was believed to have combined duodenal and cecal malrotation based on radiographic studies performed during workup for gastroesophageal reflux. At laparoscopy the small bowel mesentery was believed to have a broad enough base to prevent midgut volvulus, and an appendectomy was done. No patient required conversion to an open procedure. The sole complication was intra-abdominal abscess in the child with ruptured appendicitis that required prolonged hospitalization and operative abscess drainage. Operative times ranged from 1.25-3.25 hours (median 2 hours). Time to a regular diet was 1-20 days (median
Methods Six patients with chronic a b d o m i n a l complaints a n d o n e n e w b o r n with bilious vomiting were f o u n d to have intestinal rotation abnormalities by u p p e r gastrointestinal (GI) contrast series. Some also h a d the rotation abnormality c o n f i r m e d by lower GI contrast study. All patients u n d e r w e n t laparoscopic evaluation with p o r t p l a c e m e n t as seen in Figure 1, although n o t all ports were placed in all patients. An o p e n insertion techn i q u e was p e r f o r m e d using a H a s s o n trocar
Received December 30, 1996; Revised April 4, 1997; Accepted May 8, 1997. From the Washington University School of Medicine, Department of Surgery, St. Louis, MO. Correspondence address: Jacob C. Langer, MD, Room 5W12, St. Louis Children's Hospital, One Children's Place, St. Louis, MO 63110. © 1997 by the American College of Surgeons Published by Elsevier Science Inc.
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ISSN 1072-7515/97/$17.00 PII $1072-7515 (97) 00058-6
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INTESTINAL ROTATION ABNORMALITIES W I T H O U T VOLVULUS
p e r f o r m e d . T h e L a d d p r o c e d u r e consisted of: 1. c o m p l e t e mobilization of the right colon, which was t h e n reflected to the left, 2. complete mobilization o f the d u o d e n u m , including division of Ladd's bands a n d straightening o f the C-loop, 3. division o f adhesions a r o u n d the superior mesenteric artery to b r o a d e n the mesenteric base, a n d 4. appendectomy. Age at the time o f diagnosis r a n g e d f r o m 4 days to 23 years ( m e d i a n 7 years). Clinical features i n c l u d e d vague c h r o n i c a b d o m i n a l pain (4), early satiety (1), n a u s e a (1), vomiting (3), a n d peritoneal signs (2). T h e patients were divided into three groups d e p e n d i n g on the type o f intestinal rotation abnormality identified: 1. n o n r o t a t i o n (Fig. 2A), 2. d u o d e n a l malrotation (Fig. 2B), and 3. c o m b i n e d d u o d e n a l and cecal malrotation (Fig. 2C). T h e i r clinical courses follow.
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10
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10 5
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~1~/. ~
FIG l. Port placement for laparoscopic evaluation and treatment of intestinal rotational abnormalities. t h r o u g h a periumbilical incision. T h e a b d o m e n was insufflated to a pressure o f 8 - 1 5 m m Hg, d e p e n d i n g on the age of the patient. Exploratory laparoscopy was p e r f o r m e d , a n d the intestinal rotation abnormality was evaluated. T h e base o f the small bowel m e s e n t e r y was assessed by identifying the d u o d e n o j e j u n a l j u n c t i o n a n d the ileocecal j u n c t i o n . If the length of the mesenteric base was perceived as less t h a n half o f the transverse diameter o f the peritoneal cavity, a L a d d p r o c e d u r e was
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Results
Nonrotation Patient 1. A 16-year-old girl p r e s e n t e d with a
1-month history o f vague a b d o m i n a l pain. During h e r evaluation she h a d an u p p e r GI contrast series a n d a b a r i u m e n e m a , that revealed that h e r intestines were in a state o f nonrotation. She was followed clinically because she was not at risk for m i d g u t volvulus. She subsequently p r e s e n t e d with an acute exacerbation o f a b d o m i n a l pain with the d e v e l o p m e n t o f peritoneal signs. At laparoscopic exploration n o n r o t a t i o n was c o n f i r m e d a n d a rup-
,•
/
/
B
173
C
FIG 2. (A) Nonrotation, (B) duodenal malrotation, (C) combined duodenal and cecal malrotation.
174 J AM COli SURG AUGUST1997
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tured appendix was f o u n d b e h i n d the right ovary. Her postoperative course was complicated by intra-abdominal abscess formation requiring operative drainage. She recovered, but 1 m o n t h after discharge she was readmitted for a partial small bowel obstruction that did n o t r e s p o n d to conservative m a n a g e m e n t a n d required laparotomy. She was discharged 9 days later and has since d o n e well, with no complaints, 16 m o n t h s later. Patient 2. A 23-year-old w o m a n presented with long-standing lower abdominal pain, n o t associated with vomiting. She h a d been extensively evaluated and treated with several medical regimens without relief. An u p p e r GI series d e m o n s t r a t e d nonrotation of the bowel. Laparoscopic exploration confirmed a left-sided colon and revealed several congenital bands crossing over the duoden u m . These were divided, and the mesentery was t h e n e x a m i n e d and f o u n d to be broad-based. An a p p e n d e c t o m y was c o m p l e t e d without complications, and the patient was discharged on the day after surgery. The patient had early i m p r o v e m e n t in symptoms, b u t 15 m o n t h s later she had the same complaints that she had before the procedure. Duodenal mabvtation Patient 3. A 61½-year-old boy with multiple med-
ical problems including spina bifida, hydrocephalus, coarctation of the aorta, and a solitary right kidney presented with intermittent nonbilious Vomiting since infancy. The vomiting was postprandial and n o t associated with pain. An u p p e r GI series revealed no evidence of gastroesophageal reflux and showed the ligament of Treitz to the right o f the spine with the cecum in n o r m a l position. He u n d e r w e n t laparoscopic exploration that confirmed the position of the ligament of Treitz in the right u p p e r quadrant with the ileocecal j u n c t i o n in the right lower quadrant. Numerous Ladd's bands in the right u p p e r q u a d r a n t were divided, a n d an a p p e n d e c t o m y was perf o r m e d laparoscopically. The presence of a ventriculoperitoneal shunt did n o t pose any technical problems d u r i n g the procedure. He tolerated a regular diet a n d was discharged on the third postoperative day. Thirteen m o n t h s after discharge he h a d intermittent vomiting, but his m o t h e r n o t e d that the frequency decreased. Patient 4. A 17-year-old girl h a d several m o n t h s of intermittent u p p e r abdominal pain that was usually postprandial or nocturnal and was n o t associated with vomiting. U p p e r GI series showed that the ligament of Treitz was in the right u p p e r q u a d r a n t with n o r m a l cecal position. T h e patient u n d e r w e n t exploratory laparoscopy, and the ce-
c u m was f o u n d to be quite mobile. T h e lateral peritoneal attachments of the ascending colon were divided up to the hepatic flexure, and the ascending colon was mobilized to the left side of the a b d o m e n . The d u o d e n u m was then visualized, and adhesions between the d u o d e n u m a n d colon were divided, placing the bowel in complete nonrotation. A laparoscopic a p p e n d e c t o m y completed the procedure, and the patient was discharged h o m e on the second postoperative day. She h a d no complaints 15 m o n t h s later. Patient 5. A 2,845 g boy was born by spontaneous vaginal delivery after an uncomplicated, term pregnancy. Soon after birth he developed bilious vomiting with each feeding. His a b d o m e n was dist e n d e d but n o n t e n d e r . Supine and u p r i g h t plain radiographs of the a b d o m e n d e m o n s t r a t e d dilated small bowel loops with air fluid levels and a paucity of air in the rectum. A Hypaque e n e m a d e m o n s t r a t e d a n o r m a l cecum in the right lower quadrant. An u p p e r GI study revealed a low-lying d u o d e n o j e j u n a l j u n c t i o n and no evidence of obstruction. Rectal biopsy was normal. O n his f o u r t h day of life he u n d e r w e n t laparoscopic exploration, and, despite the a b n o r m a l position of the l i g a m e n t of Treitz, his small bowel mesentery was greater t h a n half the transverse diameter of his a b d o m e n . N o L a d d ' s b a n d s were found. Postoperatively he tolerated feedings without difficulty and was discharged 2 days later. He is now 10-months old and has since h a d no difficulty feeding. Combined duodenal and cecal malrotation Patient 6. A 7-year-old, previously healthy girl presented with signs and symptoms suggesting perforated appendicitis. She u n d e r w e n t exploration t h r o u g h a right lower q u a d r a n t incision, and a moderate a m o u n t of cloudy fluid was seen, which ultimately was culture positive for Streptococcus. T h e cecum a n d a p p e n d i x were f o u n d in the right u p p e r q u a d r a n t a n d a p p e a r e d injected w i t h o u t obvious p e r f o r a t i o n . No o t h e r perforations or abnormalities could be identified on exploration, and the appendix was n o t inflamed on pathologic examination. Malrotation was confirmed postoperatively with an u p p e r GI series, and the patient u n d e r w e n t laparoscopic exploration 3 m o n t h s later. There were few adhesions from her previous peritonitis, and a laparoscopic Ladd p r o c e d u r e was performed. The mesentery was mobilized from the ligament of Treitz to the ileocecal valve a n d a broad-based mesentery was formed. The small bowel was then placed on the right side of the a b d o m e n with the large bowel placed on the left. T h e patient was discharged
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INTESTINALROTATION
h o m e on the first postoperative day a n d h a d n o c o m p l a i n t s 16 m o n t h s after laparoscopic exploration. Patient 7. A 3-year-old girl with chronic cough a n d mild gastroesophageal reflux u n d e r w e n t an u p p e r GI series that revealed the ligament of Treitz to be just to the left of the spine and a cecum elevated from its normal position in the right iliac fossa. Because of the incomplete rotation, it was unclear whether the patient was at risk for m i d g u t volvulus, so laparoscopic exploration was r e c o m m e n d e d . During the p r o c e d u r e the liga m e n t of Treitz and ileocecal j u n c t i o n were identified. T h e distance between t h e m was greater than half of the transverse diameter of the peritoneal cavity, and we t h o u g h t it was broad e n o u g h to prevent m i d g u t volvulus. A laparoscopic appendectomy was p e r f o r m e d to complete the procedure. T h e patient was discharged on the day after surgery and had no additional coughing or other complaints 16 m o n t h s later. Overall results. Median operative time in this g r o u p of.patients was 2.0 hours (range 1.25-3.25 hours). They r e s u m e d a regular diet after a median of 2 days (range 1-20 days) a n d were discharged a m e d i a n of 2 days after surgery (range 1-21 days). None of the operations required conversion to an o p e n procedure. Symptoms completely resolved in 5-7 patients and m e d i a n followup was 15 m o n t h s (range 10-16 months). Discussion
Malrotation of the intestine can result in abnormal mesenteric attachments and a narrowed mesenteric base, which places the patient at risk of m i d g u t volvulus. T h e treatment of malrotation is based on the classic description by Ladd (3), a n d includes: 1. laparotomy with reduction of m i d g u t volvulus, if present, 2. division of peritoneal bands obstructing the d u o d e n u m , 3. p l a c e m e n t of the small and large bowel in a state of nonrotation, a n d 4. appendectomy. The principles and indications for operation have r e m a i n e d the same since the time of Ladd's address to the New H a m p s h i r e Medical Society in 1936. Most patients with malrotation seem to present with symptoms in the first 6 m o n t h s of life, with bilious vomiting in the majority of cases (4). Intestinal rotational abnormalities can be present in m u c h older patients who are ultimately investigated with intestinal contrast studies for various abdominal symptoms. Whether these symptoms are secondary to the intestinal rotational abnormality or simply coincidental is often unclear (5).
ABNORMALITIES
WITHOUT VOLVULUS 175
It is well known that a spectrum of rotational abnormalities can exist, including nonrotation, d u o d e n a l malrotation with n o r m a l cecal position, and c o m b i n e d malrotation of the d u o d e n u m and cecum. Radiographically, n o r m a l fixation of the intestines can be d e t e r m i n e d based on the positions of the d u o d e n o j e j u n a l j u n c t i o n and the cecum. T h e duodenojejunal j u n c t i o n is normally located to the left of the second lumbar vertebra, and the cecum is normally located in the right iliac fossa. Using these radiographic positions as standards, Stringer has classified all types of intestinal rotation abnormalities into Type I (nonrotation), Type II ( d u o d e n a l malrotation), or Type III (combined d u o d e n a l a n d cecal malrotation) (6). T h e relative increased mobility of various structures in the infant and child can confuse the issue. For instance, the cecum generally becomes fixed in the right lower quadrant, but in up to 36% of patients it can be on a mobile mesentery (7). Also, t h e laxity in the attachments of the distal duoden u m to the r e t r o p e r i t o n e u m in neonates a n d in children up to 4 years of age can result in a significant mobility of the d u o d e n o j e j u n a l junction, including mobility to the right of the spine (8). Others have r e p o r t e d chronic abdominal pain in patients with normally rotated but nonfixed intestines (9). Thus, because of the laxity of the various retroperitoneal attachments, it can be difficult to distinguish an abnormality at risk for m i d g u t volvulus from an abnormality n o t at risk using radiologic studies alone. Patients with vague abdominal complaints and intestinal contrast studies that suggest an intestinal rotational abnormality provide a diagnostic and therapeutic d i l e m m a for the surgeon. Because of the risk of m i d g u t volvulus, these patients must be expeditiously evaluated and treated appropriately. Patients with even a question of this diagnosis have historically u n d e r g o n e l a p a r o t o m y with L a d d p r o c e d u r e , if the m e s e n t e r i c base is narrow, a n d a p p e n d e c t o m y if the c e c u m is in an a b n o r m a l position. We believed that minimally invasive surgical t e c h n i q u e s would be able to accomplish the identical evaluation a n d t r e a t m e n t of this g r o u p of patients but without the associated morbidity of a laparotomy. Although others have described a laparoscopic approach in children with suspected volvulus (10), we have c o n t i n u e d to use laparotomy for these patients. Nonrotation of the intestine provides a particularly challenging circumstance. These patients would n o t be expected tO have symptoms secondary to their rotation abnormality because the Ladd p r o c e d u r e places the intestines in a position of
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nonrotation, but congenital bands and adhesions still can be present in nonrotation and can potentially result in symptoms. This is illustrated by patient 2, in whom duodenal, bands were noted and divided at exploration. This patient had continued postoperative complaints despite this intervention. The advantage of laparoscopy in a patient with chronic abdominal symptoms is that i t c a n be used to diagnose and eliminate a mechanical cause with minimal morbidity and a reduced risk of adhesion formation. The persistence of pain in this setting implies a nonmechanical etiology. Our data show that using a minimally invasive approach, the mesenteric base can be directly evaluated, and a determination of adequate width to prevent midgut volvulus can be made. Furthermore, an appendectomy or Ladd procedure can also be performed laparoscopically, if indicated. The median operative time was acceptable, and n o n e of the patients required conversion to an open procedure. Except for one child who had complications from a r u p t u r e d appendix, there was no morbidity, and hospital stay was very short. In five of our seven patients, the preoperative symptoms resolved. In an additional patient the frequency of vomiting was noticeably reduced after surgery.
Because of our experience and success with evaluating and treating these patients, we feel that laparoscopy is an excellent technique for the management of intestinal rotational abnormalities without volvulus. References 1. Ford EG, Senac MO Jr, Srikanth MS, and Weitzman ~. Malrotation of the intestine in children. Ann Surg 1992;215: 172-8. 2. Powell DM, Otherson HB, and Smith CD. Malrotation of the intestines in children: the effect of age on presentation and therapy. J Pediatr Surg 1989;24:777-80. 3. Ladd WE. Surgical diseases of the alimentary tract in infants. N Engl J Med 1936;215:705-8. 4. Torres AM, and Ziegler MM. Malrotation of the intestine. WorldJ Surg 1993;17:326-31. 5. Berardi RS. Anomalies of midgut rotation in the adult. Surg Gynecol Obstet 1980;151:113-24. 6. Stringer DA. Pediatric gastrointestinal imaging. Philadelphia: B.C. Decker, Inc., 1989. 7. Steiner GM. The misplaced caecum and the root of the mesentery. Br J Radiol 1977;51:406-13. 8. Katz ME, Siegel MJ, Shackelford GD, and McAllister WH. The position and mobility of the duodenum in children. Am J Radiol 1987;148:947-51. 9. Janik JS, and Ein SH. Normal intestinal rotation with nonfixation: a cause of chronic abdominal pain. J Pediatr Surg 1979;14:670-4. 10. Van der Zee DC, and Bax NMA. Laparoscopic repair of acute volvulus in a neonate with malrotation. Surg Endosc 1995;9: 11'23-4.