Malrotation
Presenting Beyond the Neonatal Period
By Nitsana
Spigland,
Mary
L. Brandt,
and
Salam
Yazbeck
Montreal. Quebec l The diagnosis of malrotation is easily made in the neonatal period, but is often delayed in older patients. Among 82 patients treated for malrotation in this institution, 45 patients presented with symptoms related to their malrotation, seven were diagnosed at exploration for concomitant intrinsic duodenal obstruction, and 30 patients had malrotations discovered as incidental findings at leparotomy or autopsy. Among the 45 symptomatic patients, 25 (56%) underwent surgery in the first month of life, whereas 20 patients (44%) underwent surgery at an older age. In this last group, the mean age at surgery was 51.5 months (range, 2 months to 16 years), the mean age of onset of symptoms was 2 years (range, 0 to 15 years) and the mean delay in diagnosis was 1.7 years. Although bilious vomiting was the presenting symptom among all patients undergoing surgery in the neonatal period, clinical features of older patients included intestinal obstruction (7). chronic abdominal pain (4). malabsorption/diarrhea (3). peritonitis/ septic shock (2). solid food intolerance (1). common bile duct obstruction (1). abdominal distention (I), and delayed transit postappendectomy (1). The frequency of midgut volvulus was equal among both groups. Unusual forms of malrotation were more frequent in patients undergoing surgery beyond the neonatal period. In this group there was evidence of chronic venous and lymphatic obstruction with one case of superior mesenteric vein thrombosis and two cases of intestinal gangrene. A Ladd’s procedure was performed in all cases and the most frequent postoperative complication was adhesive intestinal obstruction. There were no deaths. Awareness of the unusual presentation in patients who present beyond the neonatal period may help reduce delays in diagnosis and surgical treatment. We believe that laparotomy is indicated in all patients with malrotation, even if they are asymptomatic. 0 1990 by W.B. Saunders Company.
duodenal obstruction. Thirty patients had malrotations discovered as incidental findings at laparotomy for other anomalies or at autopsy, and are excluded from this study. The distribution of associated congenital anomalies is shown in Tables 1 and 2. Among the 45 patients with symptomatic malrotations, there were 30 boys and 15 girls. Twenty-five patients (56%) underwent surgery in the neonatal period (less than 1 month of age), at a mean age of 9.8 days (range, 1 to 30 days). Twenty patients (44%) underwent surgery after the first month of life at a mean age of 51.5 months (range, 2 months to 16 years). Among the 20 patients who presented beyond the neonatal period, 30% (6) had symptoms since birth. In this group, the mean age of onset of symptoms was 2 years (range, 0 to 15 years), and the mean delay in diagnosis was I .7 years (range, several hours to 6 years). For those presenting in the neonatal period the age of onset of symptoms ranged from birth to 25 days, and the mean delay in diagnosis was 5 days.
INDEX WORDS: tion; volvulus.
Abdominal flat plates were obtained in 40 of 45 cases. Among the patients presenting at less than 1 month of age, 13 of 24 showed patterns suggestive of malrotation (double-bubble with distal air, or volvulus/corkscrew pattern). The remaining x-rays demonstrated gastric dilatation or nonspecific gas patterns. In patients who presented beyond the neonatal period, only five of 16 flat plates were suggestive of duodenal obstruction or volvulus, the remainder being non specific. Upper gastrointestinal contrast studies were performed in 38 of 45 patients. The diagnosis of malrotation with or without volvulus was made in 30 patients (79%). The most frequent erroneous diagnosis
Duodenal
obstruction:
intestinal
malrota-
T
HE DIAGNOSIS of malrotation is easily made in the neonatal period because bilious vomiting, which is the most frequent presenting symptom in the first month of life, usually prompts a rapid workup for duodenal obstruction. Malrotation presenting beyond the neonatal period is associated with a multiplicity of symptoms, which are often nonspecific and, consequently, are associated with delays in diagnosis. In this retrospective study, we reviewed 82 patients with malrotation seen over a IO-year period, focusing on those patients presenting beyond the neonatal period.
Clinical Data Bilious vomiting was the presenting symptom among all patients operated in the neonatal period. The clinical features of the older patients (> 1 month of age) are summarized in Table 3. Among this group, the major presenting feature was intestinal obstruction with intermittent episodes of bilious vomiting (7). Other presentations included chronic abdominal pain, with or without vomiting (4), and malabsorption/diarrhea syndrome associated with failure to thrive (3). Abdominal pain was generally postprandial but vague; one patient complained of cramps followed by diarrhea. Two patients presented with diffuse peritoneal signs and septic shock. One patient presented with obstructive jaundice and enlargement of the head of the pancreas, secondary to compression of the distal common bile duct and pancreatic duct by a right paramesocolic hernia with intermittent volvulus.
Radiological
Findings and Diagnosis
Between 1979 and 1989, 82 patients were diagnosed as having a malrotation at HBpital Sainte-Justine, Montreal. Forty-five patients presented with symptoms related to their malrotation. Seven patients were diagnosed at exploration for concomitant intrinsic
From Ste-Justine Hospital, and the Department of Surgery, University of Montreal, Montreal, Quebec. Presented at the 21st Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton, Alberta, September 20-23, 1989. Address reprint requests to Salam Yazbeck, MD, Ste-Justine Hospital, 3175 Ste-Catherine Rd. Montreal, Quebec H3T IC5, Canada. 0 1990 by W.B. Saunders Company. 0022-3468/90/2511-0009$03.00/O
Journal
142
MATERIALS
of Pediatric
Surgery,
AND
Vol 25,
METHODS
No 11 (November),
1990:
pp 1139-l
1139
1140
Table
SPIGLAND,
1. Distribution
of Associated
Incidental
Diagnosis
Anomalies
in Patients
duodenal
obstruction
(diaphragm
or atresiaj
Clinical
fistula
of Patients
the Neonatal
YAZBECK
With
Malrotation
Period
(n = 20) No. of Patwlts
obstruction
7
Chronic abdominal pain Malabsorption/diarrhea Peritonitis/septic shock
4 3 2
Solid food intolerance Obstructive jaundice
1 1
Abdominal
Vater syndrome Bladder exstrophy
Features Beyond
AND
Clinical Features
Intestinal
hernia
Imp&orate anus Jejunal atresia Tracheoesophageal
3.
No. of Patients
Omphalocele Gastroschisis Diaphragmatic
Table
Presenting
Anomaly Intrinsic
With
of Malrotation
BRANDT,
Delayed
distention transit
chylous
ascites
1 1
postappendectomy
Biliary atresia Renal anomalies Pulmonary hypoplasia lntussusception Achondroplasia Hirschsprung’s Trisomy
2 2
disease
1 1
2 1
of duodenal stenosis, web, or diaphragm. Barium enema was performed in 10 cases.All studies showed abnormal position of the cecum.
was that
RESULTS
The diagnosis of malrotation was confirmed in all patients at laparotomy. The site of obstruction was clearly indicated in 34 charts: it was at the second portion of the duodenum in 13 cases, and at the third portion in 21 cases. All patients undergoing surgery in the neonatal period presented with duodenal obstruction or volvulus. Midgut volvulus was observed in 17 of 25 patients (68%) in this group, but none required an intestinal resection for gangrene. Midgut volvulus was present in 13 of the 20 patients (65%) undergoing surgery after the neonatal period. Although malrotation with Ladds bands and/or volvulus was the most frequent cause of obstruction in this group, two patients had nonrotations and six patients presented with unusual types of malrotations. These included right paramesocolic hernia (4), malrotation of the duodenum and small bowel without malrotation Table
2.
Associated Anomalies in Patients Symptomatic Malrotations
of the colon (l), and reversed rotation with the colon passing behind the superior mesenteric artery and duodenum ( 1). Six patients in the older age group developed preoperative complications related to an acute or a chronic volvulus. Two patients had intestinal gangrene secondary to acute volvulus, requiring resection. One patient required resection of an enormously dilated jejunal loop contained in an internal hernia, which had undergone chronic, intermittent volvulus. One patient had colonic varices secondary to venous stasis, one patient had biliary obstruction, and one patient had chylous ascites and superior mesenteric vein thrombosis because of long-standing venous and lymphatic stasis due to chronic volvulus. All patients underwent a classical Ladd procedure. An appendectomy was performed in 39 of 45 cases, and a cecopexy was performed in two cases. The surgical procedures are listed in Table 4. Nine of 45 patients (20%) developed postoperative complications (Table 5). Intestinal obstruction was the most frequent postoperative complication (5). Two patients, who presented at 6 weeks and 3 years, were found to have adhesive postoperative intestinal obstruction and one patient who was reoperated on the 10th postoperative day was found to have two different sites of small bowel intussusception. Intestinal obstruction resolved with conservative therapy in two patients. Other complications are listed in Table 5. One patient who underwent jejunal resection, je-
With Table
4. Surgical
Procedures
(45 Patients)
No. of Anomalv
Tetralogy of Fallot Atrial septal defect Coarctation of the aorta Turner’s syndrome Jejunal atresia Diaphragmatic hernia Hydronephrosis Idiopathic hypercalciuria Congenital deafness
Patients
No. of Patients
Procedure
Ladd procedure Ladd + APP Ladd + APP + cecopexy Ladd + APP + Heineke-Mickulicz Ladd + bowel resection Ladd + jejunal resection
2 30 duodenoplasty
(acute + chronic (atresiaj
Ladd + APP + antireflux procedure Ladd + APP + repair of left Bochdalek Abbreviation:
APP, appendectomy.
volvulusj
hernia
2 5 3 1 1 1
POSTNEONATAL
PRESENTATION
Table
5. Postoperative
OF MALROTATION
Complications
Intestinal obstruction Delayed transit Blind loop syndrome Klebsiella sepsis
junoileal anastomosis, and Bishop-Koop ileostomy at the initial operation, developed postoperative dilatation of the proximal jejunal pouch with recurrent symptoms of malabsorption, requiring resection of the jejunal pouch at a subsequent operation. Resolution of symptoms occurred after the second operation. There were no deaths in this series. All patients had a resolution of their symptoms after an average follow-up of 14 months (range, 1 month to 5 years). DISCUSSION
The diagnosis of malrotation is often difficult when it presents beyond the neonatal period because of the wider and more obscure constellation of clinical symptoms observed in older patients. In this series, the mean delay in diagnosis was 1.7 years in patients presenting beyond the neonatal period, whereas it was only 5 days for those presenting in the first month of life. Delays in diagnosis among older patients have been reported by other authors.’ Those patients who present with symptoms of acute duodenal obstruction are diagnosed rapidly,* whereas among those with histories of vague, chronic abdominal pain, intermittent vomiting, or feeding problems, years may elapse before the diagnosis is rnade.lA5 Their symptoms are often attributed to milk intolerance, gastrointestinal allergies, or psychogenic causes.’ Abdominal pain is generally vague, postprandial, and recurrent in nature. Others have described severe abdominal cramping followed by diarrhea suggestive of chronic volvulus.l Vomiting may or may not be bilious and it is variable in duration and frequency. Another well-described presentation is a malabsorption pattern associated with diarrhea, nutritional deficiencies, and failure to thrive.‘,2,6 Two patients in this series underwent jejunal biopsies to eliminate celiac disease and sprue before the diagnosis of malrotation was made. One patient in this series presented with chronic diarrhea, steatorrhea, and anemia at age 18 months. She was found to have a right paramesocolic hernia containing a very dilated jejunal loop that underwent intermittent volvulus and obstruction, causing a blind loop syndrome. Other authors have postulated that diarrhea and malabsorption may be caused by bowel lymphedema resulting from lymphatic obstruction by chronic volvulus and resulting in loss of proteins into the bowel lumen.* Rare presentations of chronic volvulus included one
1141
case of obstructive jaundice by mechanical compression of the biliary tract; only one such case has been previously reported in the surgical literature.7 Another patient presented with chylous ascites and superior mesenteric vein thrombosis, secondary to long-standing lymphatic and venous obstruction. Lymphatic hypertension and disruption has been postulated to occur secondary to torsion of the small bowel mesentery.’ Other reported symptoms included constipation, solid food intolerance, and gastroesophageal reflux. 1,2,3,9.10 Abdominal x-rays and contrast studies that do not carefully evaluate the ligament of Treitz and upper jejunum are often interpreted as normal. Additionally, the diagnosis of internal hernias is difficult and, therefore, often missed.’ We stress the importance of obtaining a detailed contrast study to evaluate the entire upper gastrointestinal tract, including the first several feet of jejunum, when malrotation is suspected. In addition to delays in diagnosis, delays in treatment occur because of misconceptions regarding surgical therapy for malrotation. Some authors”.‘2 have evoked the notion that older patients with malrotations are asymptomatic. This misconception has led many clinicians to attribute gastrointestinal symptoms to other causes, and overlook malrotation as a potential source of chronic abdominal pain. Wang and Welch13 have dispelled the notion that malrotation is asymptomatic in older patients by showing that 26 of 50 adult patients with malrotations developed symptoms referable to their rotational anomaly. Of the 26 symptomatic cases, 13 suffered from severe abdominal pain caused by volvulus of the bowel. The present series, as well as others,lv4 confirms the fact that older children and adolescents with malrotation may develop symptoms at any age. Among these 20 older patients, only six (30%) presented with symptoms dating since birth. Midgut volvulus is the most feared complication of intestinal malrotation. Firor and Steiger’ described two patients (15 and 20 years of age) who required home parenteral hyperalimentation because of extensive small bowel resection secondary to acute volvulus. Most large series emphasize that patients who present with symptoms of malrotation from the first few days to the first month of life are at a greater risk of developing a midgut volvu1us.3~4~‘4~15 Others report that the majority of patients with volvulus were less than 1 year of age. 4s,‘5 The frequency of midgut volvulus among infants presenting in the neonatal period has ranged from 45% to 80%.3,4.‘4.‘5 By contrast the frequency of midgut volvulus among older patients has been reported to be as low as 14%.3 In contradistinction, this series shows that the inci-
1142
SPIGLAND,
dence of volvulus was equal among both groups (65% v 68%). Firor and Steiger’ report four cases of volvulus among nine older children with malrotations. The risk of developing midgut volvulus at any age has been documented16 and the potential to develop sudden onset of acute midgut volvulus with intestinal gangrene despite many years of chronic, undiagnosed abdominal pain has been demonstrated in this series as well as others.‘,16 Both patients in this series, who were found to have intestinal gangrene as a consequence of acute volvulus, presented beyond the neonatal period and were not symptomatic since birth. Although much emphasis in the literature is placed on sequelae of acute midgut volvulus, few authors stress the morbidity associated with chronic volvulus. Four patients in this series who presented beyond the neonatal period had major complications resulting from chronic volvulus, including biliary and pancreatic duct obstruction, chylous ascites with superior mesenteric vein thrombosis, colonic varices, and blind loop syndrome requiring intestinal resection. Although it is generally agreed that symptomatic patients with documented malrotations require surgical intervention, the question as to whether surgery is indicated in the asymptomatic patient is more controversia1.5~‘2*‘5~‘6 Filston and KirksI have reported a case of 360° volvulus with venous occlusion in an asymptom-
BRANDT,
AND
YAZBECK
atic baby. One of the patients in this series, who presented at 1 year of age, developed acute volvulus with intestinal gangrene despite being previously asymptomatic. Filston and Kirks” advise exploration of the asymptomatic infant less than 2 years of age in whom malrotation has been demonstrated, because of the increased risk of volvulus in this group. By contrast we found in this series that the risk of volvulus is not reduced among those patients presenting beyond infancy. Fifty percent of adults reviewed in a large series developed symptoms referable to their malrotation.‘3 The complications associated with intestinal malrotation are based on anatomical reasons that do not change with age. Therefore, there is no logical reason to consider that a volvulus can occur at 5 days or at 15 years of age and not at 50 years of age. The potential to develop sudden onset of acute midgut volvulus in the asymptomatic patient, at any age, exists. High morbidity is associated with both chronic and acute volvulus. Because of these findings, we favor surgical correction of documented malrotation in all patients, regardless of age. Awareness of the possibility of malrotation in patients who present beyond the neonatal period may help reduce unnecessary delays in diagnosis and treatment, as well as minimize associated morbidity.
REFERENCES 1. Firor HV, Steiger E: Morbidity of rotational abnormalities of the gut beyond infancy. Cleve Clin Q 50:303-309, 1983 2. Brandt ML, Pokorny WJ, MC Gill CW, et al: Late presentations of midgut malrotation in children. Am J Surg 150:767-771, 1985 3. Yanez R, Spitz L: Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 61:682-685, 1986 4. Stewart DR, Colodny AL, Daggett WC: Malrotation of the bowel in infants and children: A 15 year review. Surgery 79:7 16-720, 1976 5. Kullendorf CM, Mikaelsson C, Ivancev K: Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Pediatr Stand 74:296-299, 1985 6. Howell CG, Vozza F, Shaw S, et al: Malrotation, malnutrition and ischemic bowel disease. J Pediatr Surg 17:469-472, 1982 7. Spitz L, Orr JD, Harries JT: Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child 58:383-385, 1983 8. Schwartz DL, So HB, Schneider KM, et al: Recurrent chylous ascites associated with intestinal malrotation and lymphatic rupture. J Pediatr Surg 18:177-179, 1983
9. Jolley SG, gastric emptying Surg 20:627-631,
Tune11 WP, Thomas S, et al: The significance of in children with intestinal malrotation. J Pediatr 1985
10. Kumar D, Brereton RJ, Spitz L, et al: Gastro-oesophageal reflux and intestinal malrotation in children. Br J Surg 75:533-535, 1988 11. Gohl ML, Demeester TR: Midgut nonrotation aggressive approach. Am J Surg 129:3 19-323, 1975 12. Silverman A, Roy C: Pediatric Clinical 3). St Louis, MO, Mosby, 1983, pp 62-66 13. Wang CA, Welch CE: adolescents and adults. Surgery
and
Gastroenterology
in
14. Andrassy RJ, Mahour HG: Malrotation of the midgut infants and children. Arch Surg 116:158-160, 1981
in
HC, Surg
Kirks DR: 16:614-620,
of intestinal 1963
(ed
rotation
15. Filston aly. J Pediatr
Anomalies 54:839-855,
in adults
Malrotation-The 1981
16. Berardi RS: Anomalies of midgut Gynecol Obstet 15 1: 113- 124, 1980
rotation
ubiquitous in the adult.
anomSurg
Presenting Beyond the Neonatal Period
By Nitsana
Spigland,
Mary
L. Brandt,
and
Salam
Yazbeck
Montreal. Quebec l The diagnosis of malrotation is easily made in the neonatal period, but is often delayed in older patients. Among 82 patients treated for malrotation in this institution, 45 patients presented with symptoms related to their malrotation, seven were diagnosed at exploration for concomitant intrinsic duodenal obstruction, and 30 patients had malrotations discovered as incidental findings at leparotomy or autopsy. Among the 45 symptomatic patients, 25 (56%) underwent surgery in the first month of life, whereas 20 patients (44%) underwent surgery at an older age. In this last group, the mean age at surgery was 51.5 months (range, 2 months to 16 years), the mean age of onset of symptoms was 2 years (range, 0 to 15 years) and the mean delay in diagnosis was 1.7 years. Although bilious vomiting was the presenting symptom among all patients undergoing surgery in the neonatal period, clinical features of older patients included intestinal obstruction (7). chronic abdominal pain (4). malabsorption/diarrhea (3). peritonitis/ septic shock (2). solid food intolerance (1). common bile duct obstruction (1). abdominal distention (I), and delayed transit postappendectomy (1). The frequency of midgut volvulus was equal among both groups. Unusual forms of malrotation were more frequent in patients undergoing surgery beyond the neonatal period. In this group there was evidence of chronic venous and lymphatic obstruction with one case of superior mesenteric vein thrombosis and two cases of intestinal gangrene. A Ladd’s procedure was performed in all cases and the most frequent postoperative complication was adhesive intestinal obstruction. There were no deaths. Awareness of the unusual presentation in patients who present beyond the neonatal period may help reduce delays in diagnosis and surgical treatment. We believe that laparotomy is indicated in all patients with malrotation, even if they are asymptomatic. 0 1990 by W.B. Saunders Company.
duodenal obstruction. Thirty patients had malrotations discovered as incidental findings at laparotomy for other anomalies or at autopsy, and are excluded from this study. The distribution of associated congenital anomalies is shown in Tables 1 and 2. Among the 45 patients with symptomatic malrotations, there were 30 boys and 15 girls. Twenty-five patients (56%) underwent surgery in the neonatal period (less than 1 month of age), at a mean age of 9.8 days (range, 1 to 30 days). Twenty patients (44%) underwent surgery after the first month of life at a mean age of 51.5 months (range, 2 months to 16 years). Among the 20 patients who presented beyond the neonatal period, 30% (6) had symptoms since birth. In this group, the mean age of onset of symptoms was 2 years (range, 0 to 15 years), and the mean delay in diagnosis was I .7 years (range, several hours to 6 years). For those presenting in the neonatal period the age of onset of symptoms ranged from birth to 25 days, and the mean delay in diagnosis was 5 days.
INDEX WORDS: tion; volvulus.
Abdominal flat plates were obtained in 40 of 45 cases. Among the patients presenting at less than 1 month of age, 13 of 24 showed patterns suggestive of malrotation (double-bubble with distal air, or volvulus/corkscrew pattern). The remaining x-rays demonstrated gastric dilatation or nonspecific gas patterns. In patients who presented beyond the neonatal period, only five of 16 flat plates were suggestive of duodenal obstruction or volvulus, the remainder being non specific. Upper gastrointestinal contrast studies were performed in 38 of 45 patients. The diagnosis of malrotation with or without volvulus was made in 30 patients (79%). The most frequent erroneous diagnosis
Duodenal
obstruction:
intestinal
malrota-
T
HE DIAGNOSIS of malrotation is easily made in the neonatal period because bilious vomiting, which is the most frequent presenting symptom in the first month of life, usually prompts a rapid workup for duodenal obstruction. Malrotation presenting beyond the neonatal period is associated with a multiplicity of symptoms, which are often nonspecific and, consequently, are associated with delays in diagnosis. In this retrospective study, we reviewed 82 patients with malrotation seen over a IO-year period, focusing on those patients presenting beyond the neonatal period.
Clinical Data Bilious vomiting was the presenting symptom among all patients operated in the neonatal period. The clinical features of the older patients (> 1 month of age) are summarized in Table 3. Among this group, the major presenting feature was intestinal obstruction with intermittent episodes of bilious vomiting (7). Other presentations included chronic abdominal pain, with or without vomiting (4), and malabsorption/diarrhea syndrome associated with failure to thrive (3). Abdominal pain was generally postprandial but vague; one patient complained of cramps followed by diarrhea. Two patients presented with diffuse peritoneal signs and septic shock. One patient presented with obstructive jaundice and enlargement of the head of the pancreas, secondary to compression of the distal common bile duct and pancreatic duct by a right paramesocolic hernia with intermittent volvulus.
Radiological
Findings and Diagnosis
Between 1979 and 1989, 82 patients were diagnosed as having a malrotation at HBpital Sainte-Justine, Montreal. Forty-five patients presented with symptoms related to their malrotation. Seven patients were diagnosed at exploration for concomitant intrinsic
From Ste-Justine Hospital, and the Department of Surgery, University of Montreal, Montreal, Quebec. Presented at the 21st Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton, Alberta, September 20-23, 1989. Address reprint requests to Salam Yazbeck, MD, Ste-Justine Hospital, 3175 Ste-Catherine Rd. Montreal, Quebec H3T IC5, Canada. 0 1990 by W.B. Saunders Company. 0022-3468/90/2511-0009$03.00/O
Journal
142
MATERIALS
of Pediatric
Surgery,
AND
Vol 25,
METHODS
No 11 (November),
1990:
pp 1139-l
1139
1140
Table
SPIGLAND,
1. Distribution
of Associated
Incidental
Diagnosis
Anomalies
in Patients
duodenal
obstruction
(diaphragm
or atresiaj
Clinical
fistula
of Patients
the Neonatal
YAZBECK
With
Malrotation
Period
(n = 20) No. of Patwlts
obstruction
7
Chronic abdominal pain Malabsorption/diarrhea Peritonitis/septic shock
4 3 2
Solid food intolerance Obstructive jaundice
1 1
Abdominal
Vater syndrome Bladder exstrophy
Features Beyond
AND
Clinical Features
Intestinal
hernia
Imp&orate anus Jejunal atresia Tracheoesophageal
3.
No. of Patients
Omphalocele Gastroschisis Diaphragmatic
Table
Presenting
Anomaly Intrinsic
With
of Malrotation
BRANDT,
Delayed
distention transit
chylous
ascites
1 1
postappendectomy
Biliary atresia Renal anomalies Pulmonary hypoplasia lntussusception Achondroplasia Hirschsprung’s Trisomy
2 2
disease
1 1
2 1
of duodenal stenosis, web, or diaphragm. Barium enema was performed in 10 cases.All studies showed abnormal position of the cecum.
was that
RESULTS
The diagnosis of malrotation was confirmed in all patients at laparotomy. The site of obstruction was clearly indicated in 34 charts: it was at the second portion of the duodenum in 13 cases, and at the third portion in 21 cases. All patients undergoing surgery in the neonatal period presented with duodenal obstruction or volvulus. Midgut volvulus was observed in 17 of 25 patients (68%) in this group, but none required an intestinal resection for gangrene. Midgut volvulus was present in 13 of the 20 patients (65%) undergoing surgery after the neonatal period. Although malrotation with Ladds bands and/or volvulus was the most frequent cause of obstruction in this group, two patients had nonrotations and six patients presented with unusual types of malrotations. These included right paramesocolic hernia (4), malrotation of the duodenum and small bowel without malrotation Table
2.
Associated Anomalies in Patients Symptomatic Malrotations
of the colon (l), and reversed rotation with the colon passing behind the superior mesenteric artery and duodenum ( 1). Six patients in the older age group developed preoperative complications related to an acute or a chronic volvulus. Two patients had intestinal gangrene secondary to acute volvulus, requiring resection. One patient required resection of an enormously dilated jejunal loop contained in an internal hernia, which had undergone chronic, intermittent volvulus. One patient had colonic varices secondary to venous stasis, one patient had biliary obstruction, and one patient had chylous ascites and superior mesenteric vein thrombosis because of long-standing venous and lymphatic stasis due to chronic volvulus. All patients underwent a classical Ladd procedure. An appendectomy was performed in 39 of 45 cases, and a cecopexy was performed in two cases. The surgical procedures are listed in Table 4. Nine of 45 patients (20%) developed postoperative complications (Table 5). Intestinal obstruction was the most frequent postoperative complication (5). Two patients, who presented at 6 weeks and 3 years, were found to have adhesive postoperative intestinal obstruction and one patient who was reoperated on the 10th postoperative day was found to have two different sites of small bowel intussusception. Intestinal obstruction resolved with conservative therapy in two patients. Other complications are listed in Table 5. One patient who underwent jejunal resection, je-
With Table
4. Surgical
Procedures
(45 Patients)
No. of Anomalv
Tetralogy of Fallot Atrial septal defect Coarctation of the aorta Turner’s syndrome Jejunal atresia Diaphragmatic hernia Hydronephrosis Idiopathic hypercalciuria Congenital deafness
Patients
No. of Patients
Procedure
Ladd procedure Ladd + APP Ladd + APP + cecopexy Ladd + APP + Heineke-Mickulicz Ladd + bowel resection Ladd + jejunal resection
2 30 duodenoplasty
(acute + chronic (atresiaj
Ladd + APP + antireflux procedure Ladd + APP + repair of left Bochdalek Abbreviation:
APP, appendectomy.
volvulusj
hernia
2 5 3 1 1 1
POSTNEONATAL
PRESENTATION
Table
5. Postoperative
OF MALROTATION
Complications
Intestinal obstruction Delayed transit Blind loop syndrome Klebsiella sepsis
junoileal anastomosis, and Bishop-Koop ileostomy at the initial operation, developed postoperative dilatation of the proximal jejunal pouch with recurrent symptoms of malabsorption, requiring resection of the jejunal pouch at a subsequent operation. Resolution of symptoms occurred after the second operation. There were no deaths in this series. All patients had a resolution of their symptoms after an average follow-up of 14 months (range, 1 month to 5 years). DISCUSSION
The diagnosis of malrotation is often difficult when it presents beyond the neonatal period because of the wider and more obscure constellation of clinical symptoms observed in older patients. In this series, the mean delay in diagnosis was 1.7 years in patients presenting beyond the neonatal period, whereas it was only 5 days for those presenting in the first month of life. Delays in diagnosis among older patients have been reported by other authors.’ Those patients who present with symptoms of acute duodenal obstruction are diagnosed rapidly,* whereas among those with histories of vague, chronic abdominal pain, intermittent vomiting, or feeding problems, years may elapse before the diagnosis is rnade.lA5 Their symptoms are often attributed to milk intolerance, gastrointestinal allergies, or psychogenic causes.’ Abdominal pain is generally vague, postprandial, and recurrent in nature. Others have described severe abdominal cramping followed by diarrhea suggestive of chronic volvulus.l Vomiting may or may not be bilious and it is variable in duration and frequency. Another well-described presentation is a malabsorption pattern associated with diarrhea, nutritional deficiencies, and failure to thrive.‘,2,6 Two patients in this series underwent jejunal biopsies to eliminate celiac disease and sprue before the diagnosis of malrotation was made. One patient in this series presented with chronic diarrhea, steatorrhea, and anemia at age 18 months. She was found to have a right paramesocolic hernia containing a very dilated jejunal loop that underwent intermittent volvulus and obstruction, causing a blind loop syndrome. Other authors have postulated that diarrhea and malabsorption may be caused by bowel lymphedema resulting from lymphatic obstruction by chronic volvulus and resulting in loss of proteins into the bowel lumen.* Rare presentations of chronic volvulus included one
1141
case of obstructive jaundice by mechanical compression of the biliary tract; only one such case has been previously reported in the surgical literature.7 Another patient presented with chylous ascites and superior mesenteric vein thrombosis, secondary to long-standing lymphatic and venous obstruction. Lymphatic hypertension and disruption has been postulated to occur secondary to torsion of the small bowel mesentery.’ Other reported symptoms included constipation, solid food intolerance, and gastroesophageal reflux. 1,2,3,9.10 Abdominal x-rays and contrast studies that do not carefully evaluate the ligament of Treitz and upper jejunum are often interpreted as normal. Additionally, the diagnosis of internal hernias is difficult and, therefore, often missed.’ We stress the importance of obtaining a detailed contrast study to evaluate the entire upper gastrointestinal tract, including the first several feet of jejunum, when malrotation is suspected. In addition to delays in diagnosis, delays in treatment occur because of misconceptions regarding surgical therapy for malrotation. Some authors”.‘2 have evoked the notion that older patients with malrotations are asymptomatic. This misconception has led many clinicians to attribute gastrointestinal symptoms to other causes, and overlook malrotation as a potential source of chronic abdominal pain. Wang and Welch13 have dispelled the notion that malrotation is asymptomatic in older patients by showing that 26 of 50 adult patients with malrotations developed symptoms referable to their rotational anomaly. Of the 26 symptomatic cases, 13 suffered from severe abdominal pain caused by volvulus of the bowel. The present series, as well as others,lv4 confirms the fact that older children and adolescents with malrotation may develop symptoms at any age. Among these 20 older patients, only six (30%) presented with symptoms dating since birth. Midgut volvulus is the most feared complication of intestinal malrotation. Firor and Steiger’ described two patients (15 and 20 years of age) who required home parenteral hyperalimentation because of extensive small bowel resection secondary to acute volvulus. Most large series emphasize that patients who present with symptoms of malrotation from the first few days to the first month of life are at a greater risk of developing a midgut volvu1us.3~4~‘4~15 Others report that the majority of patients with volvulus were less than 1 year of age. 4s,‘5 The frequency of midgut volvulus among infants presenting in the neonatal period has ranged from 45% to 80%.3,4.‘4.‘5 By contrast the frequency of midgut volvulus among older patients has been reported to be as low as 14%.3 In contradistinction, this series shows that the inci-
1142
SPIGLAND,
dence of volvulus was equal among both groups (65% v 68%). Firor and Steiger’ report four cases of volvulus among nine older children with malrotations. The risk of developing midgut volvulus at any age has been documented16 and the potential to develop sudden onset of acute midgut volvulus with intestinal gangrene despite many years of chronic, undiagnosed abdominal pain has been demonstrated in this series as well as others.‘,16 Both patients in this series, who were found to have intestinal gangrene as a consequence of acute volvulus, presented beyond the neonatal period and were not symptomatic since birth. Although much emphasis in the literature is placed on sequelae of acute midgut volvulus, few authors stress the morbidity associated with chronic volvulus. Four patients in this series who presented beyond the neonatal period had major complications resulting from chronic volvulus, including biliary and pancreatic duct obstruction, chylous ascites with superior mesenteric vein thrombosis, colonic varices, and blind loop syndrome requiring intestinal resection. Although it is generally agreed that symptomatic patients with documented malrotations require surgical intervention, the question as to whether surgery is indicated in the asymptomatic patient is more controversia1.5~‘2*‘5~‘6 Filston and KirksI have reported a case of 360° volvulus with venous occlusion in an asymptom-
BRANDT,
AND
YAZBECK
atic baby. One of the patients in this series, who presented at 1 year of age, developed acute volvulus with intestinal gangrene despite being previously asymptomatic. Filston and Kirks” advise exploration of the asymptomatic infant less than 2 years of age in whom malrotation has been demonstrated, because of the increased risk of volvulus in this group. By contrast we found in this series that the risk of volvulus is not reduced among those patients presenting beyond infancy. Fifty percent of adults reviewed in a large series developed symptoms referable to their malrotation.‘3 The complications associated with intestinal malrotation are based on anatomical reasons that do not change with age. Therefore, there is no logical reason to consider that a volvulus can occur at 5 days or at 15 years of age and not at 50 years of age. The potential to develop sudden onset of acute midgut volvulus in the asymptomatic patient, at any age, exists. High morbidity is associated with both chronic and acute volvulus. Because of these findings, we favor surgical correction of documented malrotation in all patients, regardless of age. Awareness of the possibility of malrotation in patients who present beyond the neonatal period may help reduce unnecessary delays in diagnosis and treatment, as well as minimize associated morbidity.
REFERENCES 1. Firor HV, Steiger E: Morbidity of rotational abnormalities of the gut beyond infancy. Cleve Clin Q 50:303-309, 1983 2. Brandt ML, Pokorny WJ, MC Gill CW, et al: Late presentations of midgut malrotation in children. Am J Surg 150:767-771, 1985 3. Yanez R, Spitz L: Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 61:682-685, 1986 4. Stewart DR, Colodny AL, Daggett WC: Malrotation of the bowel in infants and children: A 15 year review. Surgery 79:7 16-720, 1976 5. Kullendorf CM, Mikaelsson C, Ivancev K: Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Pediatr Stand 74:296-299, 1985 6. Howell CG, Vozza F, Shaw S, et al: Malrotation, malnutrition and ischemic bowel disease. J Pediatr Surg 17:469-472, 1982 7. Spitz L, Orr JD, Harries JT: Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child 58:383-385, 1983 8. Schwartz DL, So HB, Schneider KM, et al: Recurrent chylous ascites associated with intestinal malrotation and lymphatic rupture. J Pediatr Surg 18:177-179, 1983
9. Jolley SG, gastric emptying Surg 20:627-631,
Tune11 WP, Thomas S, et al: The significance of in children with intestinal malrotation. J Pediatr 1985
10. Kumar D, Brereton RJ, Spitz L, et al: Gastro-oesophageal reflux and intestinal malrotation in children. Br J Surg 75:533-535, 1988 11. Gohl ML, Demeester TR: Midgut nonrotation aggressive approach. Am J Surg 129:3 19-323, 1975 12. Silverman A, Roy C: Pediatric Clinical 3). St Louis, MO, Mosby, 1983, pp 62-66 13. Wang CA, Welch CE: adolescents and adults. Surgery
and
Gastroenterology
in
14. Andrassy RJ, Mahour HG: Malrotation of the midgut infants and children. Arch Surg 116:158-160, 1981
in
HC, Surg
Kirks DR: 16:614-620,
of intestinal 1963
(ed
rotation
15. Filston aly. J Pediatr
Anomalies 54:839-855,
in adults
Malrotation-The 1981
16. Berardi RS: Anomalies of midgut Gynecol Obstet 15 1: 113- 124, 1980
rotation
ubiquitous in the adult.
anomSurg
