80 Pictorial Essay
Bowl Obstruction Due to an Incomplete Obliterated Omphalomesenteric Duct Ileus aufgrund eines nicht obliterierten Ductus omphaloentericus
Authors
A. Wiegering1, F. Endter2, V. A. Wiegering3, C.-T. Germer1, J. Maroske2
Affiliations
1
Key words ▶ Meckel’s diverticulum ● ▶ ileus ● ▶ vitelline duct ● ▶ appendicitis ●
Introduction
Discussion
Abdominal pain is a common cause of consultation, especially for emergency medical services. Even if appendicitis is the most prevalent cause for acute abdomen and emergency surgery in infants, there are certain differential diagnoses, which should not be ignored. Beside frequent causes like gastroenteritis, a wide range of disease can present with different grade of abdominal discomfort ranging from tumors, internal hernias and malabsorption to congenital malformations [1, 2, 4, 6, 7, 11, 12]. The Meckel’s diverticulum (MD) is one of those.
During embryogenesis the omphalomesenteric duct (vitelline duct) connects the yolk sac to the midgut and is normally completely obliterated between the 6th and 8th week of gestation [8, 10]. Incomplete obliteration of the vitelline duct can cause a variety of anomalies ranging from complete patent omphalomesenteric duct with stool loss over the umbilicus, to cysts and fibrous cords connecting the small intestine to the umbilicus, as well as the classic MD. MD occurs in the terminal ileum, 45–90 cm proximal to the ileocecal valve on the antimesenteric border. The MD is the most common residue of the vitelline duct and overall it is the most common malformation of the gastrointestinal tract, with an incidence of about 2 % [8, 10]. Most individuals with a MD remain asymptomatic throughout life; only 4–6 % becomes symptomatic, with widespread clinical manifestation as infections, bleeding, perforation as well as intestinal obstruction [7, 8, 10]. Obstruction is the second common cause for symptomatic MD, this includes intussusception, volvulus, diverticulum lithiasis or internal hernia [7]. Torsion around a remaining vitelline duct is a more rare cause of intestinal obstruction. Intestinal obstruction by a congenital band is difficult to diagnose. In the literature there are few cases of image-based diagnoses of intestinal obstruction due to a vitelline duct [3, 5, 6, 9]. In most cases, the diagnosis of intestinal obstruction was reached with an X-ray-based tool such as CT scan or contrast enema. However, Chao Sun et al. [9] reported 2 cases of ultrasound-based diagnosis of volvulus and small intestine strangulation due to a remaining vitelline duct. Sonographic diagnostic has the advantages of no radiation exposure; it is widely available, produce real time images in high quality and can be repeated when necessary. On the other hand, intestinal gas and incompliant or obese patients pose problems. Furthermore, results vary
▼
Case Report
▼
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1366988 Published online: February 19, 2014 Klin Padiatr 2014; 226: 80–81 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Armin Wiegering Department of General Visceral, Vascular and Paediatric Surgery University Hospital Wuerzburg Oberdürrbacher Straße 6 97080 Würzburg Germany Tel.: + 49/931/20131 516 Fax: + 49/931/20131 709 [email protected]
A 10-year-old boy was admitted for the fourth time since the age of 3 with lower abdominal pain. He had no history of abdominal surgery. Examination showed an acute abdomen, white blood count (18.3000/μl) and CrP (2.5 mg/dl) were elevated without other pathologic findings. Free abdominal fluid and distended small intestine were documented with ultrasound. Appendicitis was speculated, although the appendix could not be seen by ultrasound. Due to this hypothesis, an explorative laparoscopy was performed. Intra-abdominal, the distended small intestine was haemorrhagic. Laparoscopy provided no sufficient overview, so conversion to lower abdominal midline laparotomy (umbilicus to symphysis) had to be performed. The cause for the abdominal symptomatic was found to be a MD ▶ Fig. 1a, b). with a fibrous cord to the umbilicus (● The small intestine with the diverticulum was twisted 360 ° around the fixation to the umbilicus and thereby strangulated. The cord was cut and the MD resected. Small intestine was perfused normally after releasing the strangulation and no resection was necessary. The patient was discharged in good condition 6 days after surgery.
Wiegering A et al. Bowl Obstruction Due to … Klin Padiatr 2014; 226: 80–81
▼
Downloaded by: University of Pittsburgh. Copyrighted material.
Schlüsselwörter ▶ Meckelsches Divertikel ● ▶ Ileus ● ▶ Ductus vittelinus ● ▶ Appendizitis ●
Department of General, Visceral, Vascular and Paediatric Surgery, University Hospital Wuerzburg, Germany Department of General Surgery, Hospital Rothenburg o.d.Tauber, Germany 3 Department of Pediatrics, University Hospital Wuerzburg, Germany 2
Pictorial Essay 81
b
Fig. 1 Meckel’s diverticulum with a fibrous cord to the umbilicus. a Intra-operative abdominal situs. The MD (dagger) with a fibrous cord (white arrow) to the umbilicus (black arrow) is shown; the small intestine with the diverticulum is twisted 360 °. b Intra-operative abdominal situs. The fibrous cord was cut, MD is on the antimesenteric border (dagger).
between examiners also. High expertise and long training is necessary for diagnosing a vitelline duct properly. In this presented case, the preoperative ultrasound showed free abdominal fluid and distended small intestine; both are consequences of small bowel obstruction, but are not specific and could also be signs of other pathology. A remaining vitelline duct could not be diagnosed preoperatively. A plain abdomen x-ray can help to exclude a perforation or could show an abnormal gas distribution due to an ileus. Also malrotation might sometimes be seen in an abdomen x-ray. When x-ray and ultrasound are not conclusive, an MRI could be helpful but has the disadvantage of not being always available, especially in smaller hospitals, outside normal working hours and due to its long imaging period anesthesia might be necessary in young patients. We were not able to diagnose MD before surgery, mainly due to the critical state of the child with acute abdomen and peritonitis and the need of prompt action. A diagnose of the MD during the 3 prior admissions might have allowed a laparoscopic solution. But due to the acute strangulation with massive distended small intestine laparoscopy did not achieve a sufficient overview and conversion to laparotomy had to be performed. In general, 2 kinds of diverticulectomies must be distinguished: those caused by the symptomatic MD itself and incidental diverticulectomies during laparotomy or laparoscopy for other surgical problems. In a large series with 815 patients who underwent MD resection at 3 Paediatric Medical Centre in the USA, ¾ were performed using an open approach, independent of the cause of surgery (for MD or another surgical problem) [5]. 5 % were reported as “conversion from laparoscopic to open” approach. Postoperatively, our patient was discharged on the sixth day after operation without any complications, which is in the reported range (5.4 ± 4.8 days) [5]. In summary, although symptomatic MD is rare, it is the most prevalent congenital abnormality of the gastrointestinal tract
and it is difficult to diagnose due to the many facets of presentation. Therefore, it is important for clinicians to keep this malformation in mind when looking at cases of suspected or atypical appendicitis.
Conflict of interest: The authors have no conflict of interest to disclose. References 1 Bode SF, Greiner P, Franke M et al. Late presentation of congenital diaphragmatic hernias – report of 3 cases. Klin Padiatr 2012; 224: 465–466 2 Brecht IB, Agaimy A, Besendörfer M et al. Malignant peritoneal mesothelioma in a 16-year-old girl: presentation of a rare disease. Klin Padiatr 2012; 224: 170–173 3 Fenton LZ, Buonomo C, Share JC et al. Small intestinal obstruction by remnants of the omphalomesenteric duct: findings on contrast enema. Pediatr Radiol 2000; 30: 165–167 4 Flemming GMC, Oberschmid B, Siebolts U et al. Abdominal tuberculosis in children and adolescents: to this day a diagnostic challenge. Klin Padiatr 2013; 225: 47–49 5 Ruscher KA, Fisher JN, Hughes CD et al. National trends in the surgical management of Meckel’s diverticulum. J Ped Surg 2011; 46: 893–896 6 Ko SF, Tiao MM, Huang FC et al. Internal hernia associated with Meckel’s diverticulum in 2 pediatric patients. Am J Emerg Med 2008; 26: 86–90 7 Menezes M, Tareen F, Saeed A et al. Symptomatic Meckel’s diverticulum in children: a 16-year review. Pediatr Surg Int 2008; 24: 575–577 8 Moore TC. Omphalomesenteric duct malformations. Semin Pediatr Surg 1996; 5: 116–123 9 Sun C, Hu X, Huang L. Intestinal obstruction due to congenital bands from vitelline remnants. J Ultrasound Med 2012; 31: 2035–2038 10 Uppal K, Tubbs S, Matusz P et al. Meckel’s diverticulum: A review. Clinical Anatomy 2011; 24: 416–422 11 Wintermeyer P, Baur M, Pilic D et al. Fructose malabsorption in children with recurrent abdominal pain: positive effects of dietary treatment. Klin Padiatr 2012; 224: 17–21 12 You JS, Chung SP, Park YS et al. A case of strangulated small bowel obstruction caused by Meckel’s diverticulum in an adult. J Emerg Med 2007; 33: 133–135
Wiegering A et al. Bowl Obstruction Due to … Klin Padiatr 2014; 226: 80–81
Downloaded by: University of Pittsburgh. Copyrighted material.
a
Bowl Obstruction Due to an Incomplete Obliterated Omphalomesenteric Duct Ileus aufgrund eines nicht obliterierten Ductus omphaloentericus
Authors
A. Wiegering1, F. Endter2, V. A. Wiegering3, C.-T. Germer1, J. Maroske2
Affiliations
1
Key words ▶ Meckel’s diverticulum ● ▶ ileus ● ▶ vitelline duct ● ▶ appendicitis ●
Introduction
Discussion
Abdominal pain is a common cause of consultation, especially for emergency medical services. Even if appendicitis is the most prevalent cause for acute abdomen and emergency surgery in infants, there are certain differential diagnoses, which should not be ignored. Beside frequent causes like gastroenteritis, a wide range of disease can present with different grade of abdominal discomfort ranging from tumors, internal hernias and malabsorption to congenital malformations [1, 2, 4, 6, 7, 11, 12]. The Meckel’s diverticulum (MD) is one of those.
During embryogenesis the omphalomesenteric duct (vitelline duct) connects the yolk sac to the midgut and is normally completely obliterated between the 6th and 8th week of gestation [8, 10]. Incomplete obliteration of the vitelline duct can cause a variety of anomalies ranging from complete patent omphalomesenteric duct with stool loss over the umbilicus, to cysts and fibrous cords connecting the small intestine to the umbilicus, as well as the classic MD. MD occurs in the terminal ileum, 45–90 cm proximal to the ileocecal valve on the antimesenteric border. The MD is the most common residue of the vitelline duct and overall it is the most common malformation of the gastrointestinal tract, with an incidence of about 2 % [8, 10]. Most individuals with a MD remain asymptomatic throughout life; only 4–6 % becomes symptomatic, with widespread clinical manifestation as infections, bleeding, perforation as well as intestinal obstruction [7, 8, 10]. Obstruction is the second common cause for symptomatic MD, this includes intussusception, volvulus, diverticulum lithiasis or internal hernia [7]. Torsion around a remaining vitelline duct is a more rare cause of intestinal obstruction. Intestinal obstruction by a congenital band is difficult to diagnose. In the literature there are few cases of image-based diagnoses of intestinal obstruction due to a vitelline duct [3, 5, 6, 9]. In most cases, the diagnosis of intestinal obstruction was reached with an X-ray-based tool such as CT scan or contrast enema. However, Chao Sun et al. [9] reported 2 cases of ultrasound-based diagnosis of volvulus and small intestine strangulation due to a remaining vitelline duct. Sonographic diagnostic has the advantages of no radiation exposure; it is widely available, produce real time images in high quality and can be repeated when necessary. On the other hand, intestinal gas and incompliant or obese patients pose problems. Furthermore, results vary
▼
Case Report
▼
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1366988 Published online: February 19, 2014 Klin Padiatr 2014; 226: 80–81 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0300-8630 Correspondence Dr. Armin Wiegering Department of General Visceral, Vascular and Paediatric Surgery University Hospital Wuerzburg Oberdürrbacher Straße 6 97080 Würzburg Germany Tel.: + 49/931/20131 516 Fax: + 49/931/20131 709 [email protected]
A 10-year-old boy was admitted for the fourth time since the age of 3 with lower abdominal pain. He had no history of abdominal surgery. Examination showed an acute abdomen, white blood count (18.3000/μl) and CrP (2.5 mg/dl) were elevated without other pathologic findings. Free abdominal fluid and distended small intestine were documented with ultrasound. Appendicitis was speculated, although the appendix could not be seen by ultrasound. Due to this hypothesis, an explorative laparoscopy was performed. Intra-abdominal, the distended small intestine was haemorrhagic. Laparoscopy provided no sufficient overview, so conversion to lower abdominal midline laparotomy (umbilicus to symphysis) had to be performed. The cause for the abdominal symptomatic was found to be a MD ▶ Fig. 1a, b). with a fibrous cord to the umbilicus (● The small intestine with the diverticulum was twisted 360 ° around the fixation to the umbilicus and thereby strangulated. The cord was cut and the MD resected. Small intestine was perfused normally after releasing the strangulation and no resection was necessary. The patient was discharged in good condition 6 days after surgery.
Wiegering A et al. Bowl Obstruction Due to … Klin Padiatr 2014; 226: 80–81
▼
Downloaded by: University of Pittsburgh. Copyrighted material.
Schlüsselwörter ▶ Meckelsches Divertikel ● ▶ Ileus ● ▶ Ductus vittelinus ● ▶ Appendizitis ●
Department of General, Visceral, Vascular and Paediatric Surgery, University Hospital Wuerzburg, Germany Department of General Surgery, Hospital Rothenburg o.d.Tauber, Germany 3 Department of Pediatrics, University Hospital Wuerzburg, Germany 2
Pictorial Essay 81
b
Fig. 1 Meckel’s diverticulum with a fibrous cord to the umbilicus. a Intra-operative abdominal situs. The MD (dagger) with a fibrous cord (white arrow) to the umbilicus (black arrow) is shown; the small intestine with the diverticulum is twisted 360 °. b Intra-operative abdominal situs. The fibrous cord was cut, MD is on the antimesenteric border (dagger).
between examiners also. High expertise and long training is necessary for diagnosing a vitelline duct properly. In this presented case, the preoperative ultrasound showed free abdominal fluid and distended small intestine; both are consequences of small bowel obstruction, but are not specific and could also be signs of other pathology. A remaining vitelline duct could not be diagnosed preoperatively. A plain abdomen x-ray can help to exclude a perforation or could show an abnormal gas distribution due to an ileus. Also malrotation might sometimes be seen in an abdomen x-ray. When x-ray and ultrasound are not conclusive, an MRI could be helpful but has the disadvantage of not being always available, especially in smaller hospitals, outside normal working hours and due to its long imaging period anesthesia might be necessary in young patients. We were not able to diagnose MD before surgery, mainly due to the critical state of the child with acute abdomen and peritonitis and the need of prompt action. A diagnose of the MD during the 3 prior admissions might have allowed a laparoscopic solution. But due to the acute strangulation with massive distended small intestine laparoscopy did not achieve a sufficient overview and conversion to laparotomy had to be performed. In general, 2 kinds of diverticulectomies must be distinguished: those caused by the symptomatic MD itself and incidental diverticulectomies during laparotomy or laparoscopy for other surgical problems. In a large series with 815 patients who underwent MD resection at 3 Paediatric Medical Centre in the USA, ¾ were performed using an open approach, independent of the cause of surgery (for MD or another surgical problem) [5]. 5 % were reported as “conversion from laparoscopic to open” approach. Postoperatively, our patient was discharged on the sixth day after operation without any complications, which is in the reported range (5.4 ± 4.8 days) [5]. In summary, although symptomatic MD is rare, it is the most prevalent congenital abnormality of the gastrointestinal tract
and it is difficult to diagnose due to the many facets of presentation. Therefore, it is important for clinicians to keep this malformation in mind when looking at cases of suspected or atypical appendicitis.
Conflict of interest: The authors have no conflict of interest to disclose. References 1 Bode SF, Greiner P, Franke M et al. Late presentation of congenital diaphragmatic hernias – report of 3 cases. Klin Padiatr 2012; 224: 465–466 2 Brecht IB, Agaimy A, Besendörfer M et al. Malignant peritoneal mesothelioma in a 16-year-old girl: presentation of a rare disease. Klin Padiatr 2012; 224: 170–173 3 Fenton LZ, Buonomo C, Share JC et al. Small intestinal obstruction by remnants of the omphalomesenteric duct: findings on contrast enema. Pediatr Radiol 2000; 30: 165–167 4 Flemming GMC, Oberschmid B, Siebolts U et al. Abdominal tuberculosis in children and adolescents: to this day a diagnostic challenge. Klin Padiatr 2013; 225: 47–49 5 Ruscher KA, Fisher JN, Hughes CD et al. National trends in the surgical management of Meckel’s diverticulum. J Ped Surg 2011; 46: 893–896 6 Ko SF, Tiao MM, Huang FC et al. Internal hernia associated with Meckel’s diverticulum in 2 pediatric patients. Am J Emerg Med 2008; 26: 86–90 7 Menezes M, Tareen F, Saeed A et al. Symptomatic Meckel’s diverticulum in children: a 16-year review. Pediatr Surg Int 2008; 24: 575–577 8 Moore TC. Omphalomesenteric duct malformations. Semin Pediatr Surg 1996; 5: 116–123 9 Sun C, Hu X, Huang L. Intestinal obstruction due to congenital bands from vitelline remnants. J Ultrasound Med 2012; 31: 2035–2038 10 Uppal K, Tubbs S, Matusz P et al. Meckel’s diverticulum: A review. Clinical Anatomy 2011; 24: 416–422 11 Wintermeyer P, Baur M, Pilic D et al. Fructose malabsorption in children with recurrent abdominal pain: positive effects of dietary treatment. Klin Padiatr 2012; 224: 17–21 12 You JS, Chung SP, Park YS et al. A case of strangulated small bowel obstruction caused by Meckel’s diverticulum in an adult. J Emerg Med 2007; 33: 133–135
Wiegering A et al. Bowl Obstruction Due to … Klin Padiatr 2014; 226: 80–81
Downloaded by: University of Pittsburgh. Copyrighted material.
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