Revista Societatii de Medicina Interna
Articolul face parte din revista :
Nr.4 din luna 2011
Autor Mihaela Enache,M.Iriciuc,I.Copaci,T.Artenie,A.Dima,F.Vasilescu,M.Sotcan,C.Jurcut,C.Rusu
Titlu articolJEJUNOJEJUNAL INTUSSUSCEPTION DETEMINED BY A GASTROINTESTINAL STROMAL TUMOR (GIST)
Cuvinte cheieintestinal intussusception, gastrointestinal stromal tumour
Articol
Mihaela Enache, M. Iriciuc, Iulian Copaci, T. Artenie, A. Dima, F. Vasilescu, M. Şotcan, Ciprian Jurcuţ, C. Rusu
Central Military Emergency University Hospital „Dr. Carol Davila”, Bucharest
We present the case of a 57-year old patient, who committed in our department for abdominal pain, nausea and vomiting occurring for 4 days. In the last 6 months she has been experiencing repeated similar episodes, of high intensity, which resolved spontaneously or with symptomatic medication. Every time the paraclinic data (biological, ultrasound, endoscopic) were normal.
Upon commitment, the patient had an altered general condition, was spilling food and bilious secretion, had skin and mucosal dehydration signs and the abdomen was diffusely painful upon palpation. This time as well, the biological parameters were normal, the EKG and the pulmonary x-ray, performed for routine control were, also, normal. The direct abdominal x-ray indicated diffuse gaseous intestinal distention. The abdominal ultrasound, performed immediately, identifies in the right parumbilical mesogastrium an image in target (concentric rings with increasing and decreasing echogenicity and thick intestinal wall), which easily alters its form by moving the intestinal liquid once the progression of the peristaltic waves, with proximal intestine dilatation of the same structure, suggesting intestinal intussusception (figure no. 1). This was subsequently confirmed by the CT scan, which shows the presence of the intestinal intussusception cylinders on a length of 15 cm (figure no. 2).
The intestinal intussusception and intestinal obstruction diagnosis was established and an emergency intervention took place (figure no. 3). At about 300 cm from the duodenojejunal (distal jejunum) angle the enteral intussusception segment with a length of about 15-20 cm and the perilesional adenopathies with a maximum diameter of about 1,5-2 cm stand out. After the dessintussusception of the enteral segments, without difficulty, a firm, round-oval shaped tumour developed in the intestinal wall, with a diameter of around 4/3 cm and partial stenosis, without obvious distinction in the enteral serosa (the cause for telescoping the proximal intestine by the latter in the distal segment) is discovered; proximal obstruction dilated enteral loops. A segmentary enterectomy is made, by lifting the intussusception segment and the loco-regional adenopathies (extemporaneously resulting from a neighbourhood node - without elements de malignancy), with the restoration of the digestive continuity by entero-enteroanastomosis latero-lateral suture in double layer.
The histopathology exam of the tumour from the intestinal wall established the gastrointestinal stromal tumour diagnosis (GIST) with low mitotic index (figure no. 4), which required no additional treatment. 6 months post-surgery the patient was completely asymptomatic.
Comments
The intussusception is intestinal the telescoping of one portion of the intestine in the immediately adjacent segment, altering the passage of the intestinal content and compromising the vascular flow. It can occur at the junction of the segments moving freely and the fixed retroperitoneal segments.
It is common with children (the clinical triad: cramps, vomiting, bloody diarrhea), in whose case it is usually primary and benign and rare in adults (5% of the causes digestive obstruction), in whose case it is almost always secondary to another pathological conditions, which alters the bowel movements (table 1).
The case history provides no reliable diagnostic elements in the intestinal intussusception. The abdominal colicative pains, nausea, vomiting, abdominal distension are non-specific symptoms, which often delay the diagnosis. The physical examination and the direct abdominal x-ray signs of intestinal obstruction. The abdominal ultrasound is very useful for children. For the adult the results depend on the examiner’s experience and are hampered by obesity and the presence of gas in the intestine. The most sensitive diagnostic method remains the computed-tomography examination. The magnetic resonance and the exploration with the endoscopic capsule can also be useful.
In the intestinal intussusception are trained the mesenteric vessels. The venous compression generates local edema, which further reduces the blood flow which is already low in the area, leading to the necrosis of the affected intestine segment, perforation and peritonitis. Another complication of the intestinal intussusception is the acute or chronic intestinal occlusion.
The treatment of choice in the adult is surgery, because the substrate in most cases is neoplastic, preceded by sedation, muscle relaxants, hydration. In children it is effective the pneumatic or barium reduction under x-ray or ultrasound control.
Gastrointestinal stromal tumours (GIST) come from the interstitial cells of Cajal, part of the autonomic nervous system, named pacemakers of the intestine whose motility they coordinate. They appear from a mutation of a gene called c-kit that encodes a receptor for a growth factor expressed in the interstitial cells of Cajal. They can be found anywhere in the gastrointestinal tract and are part of the sarcoma family. GIST can be completely asymptomatic or determine the gastrointestinal bleeding, nausea, vomiting, intestinal obstruction. It often mimics the irritable bowel and become quite large before being diagnosed.
The treatment is surgical for localized tumours. Small tumours, with low mitotic index do not recur and after surgery they do not require adjuvant therapy. Tumours larger than 5 cm can spread or recur. C-kit tyrosine kinase inhibitor, imatinib (GLIVEC) 400mg/day, originally used in chronic myeloid leukaemia has been used in the GIST treatment, either pre-surgery (decreases the tumour size), or post-surgery (decreases the recurrence rate) or for patients with no surgical indication.
The patients refractory to imatinib respond to a tyrosine kinase multiple inhibitor – sunitinib.
Conclusion
The intestinal intussusception in the adult is a rare cause of intestinal obstruction and it is almost always secondary to other pathological conditions, in our case a GIST tumour. Both give suggestive symptoms of irritable bowel and the usual, often normal, paraclinical explorations, delay the diagnosis.
Causes of intestinal intussusception in the adult (90% intussusceptions have organic cause, 65% neoplasms):
• Tumours
• Intestinal polyps
• Mekel’s diverticulum
• Cecal duplication
• Intramural haematoma
• Inflammatory bowel diseases
• Lymphomas
• Parasitic infections
• Post-surgery adhesions
• Intestinal motility disorders: irritable bowel, chronic diarrhea, Hirshprung disease
Figure no. 4. Histopathological of gastrointestinal stromal tumour
Figure no. 3. Intraoperative appearance
2 intussuscepted loops
Tumor in the intestinal wall
Bibliography
1. Crowther KS ,Wyld L, Yamany Q, Jacob G. Gastroduodenal intussusception of a gastrointestinal stromal tumour. British Journal of Radiology 2002;75:987-989
2. Marinis A , Yiallourou A , Samanides L, Dafnios N, Anastasopoulos G Vassiliou I, TheodosopoulosT. Intussusception of the bowel in adults: A review. World J Gastroenterol. 2009 ; 15(4): 407–411.
3. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW. Diagnosis of gastrointestinal stromal tumours: A consensus approach. Hum Pathol. 2002; 33(5): 459-6
4. Gollub MJ. Colonic intussusception: clinical and radiographic features. AJR Am J Roentgenol. 2011; 196(5):W580-5.
5. Soni S, Moss P, Jaiganesh T. Idiopathic adult intussusception. Int J Emerg Med. 2011; 16;4:8
6. Mendez D, Caviness AC, Ma L, Macias CC. The diagnostic accuracy of an abdominal radiograph with signs and symptoms of intussusception. Am J Emerg Med. 2011; 29:10-17
7. Arolfo S, Teggia PM, Nano M. Gastrointestinal stromal tumours: Thirty years’ experience of an Institution. World J Gastroenterol. 2011;17(14):1836-9
Nr.4 din luna 2011
Mihaela Enache, M. Iriciuc, Iulian Copaci, T. Artenie, A. Dima, F. Vasilescu, M. Şotcan, Ciprian Jurcuţ, C. Rusu
Central Military Emergency University Hospital „Dr. Carol Davila”, Bucharest
We present the case of a 57-year old patient, who committed in our department for abdominal pain, nausea and vomiting occurring for 4 days. In the last 6 months she has been experiencing repeated similar episodes, of high intensity, which resolved spontaneously or with symptomatic medication. Every time the paraclinic data (biological, ultrasound, endoscopic) were normal.
Upon commitment, the patient had an altered general condition, was spilling food and bilious secretion, had skin and mucosal dehydration signs and the abdomen was diffusely painful upon palpation. This time as well, the biological parameters were normal, the EKG and the pulmonary x-ray, performed for routine control were, also, normal. The direct abdominal x-ray indicated diffuse gaseous intestinal distention. The abdominal ultrasound, performed immediately, identifies in the right parumbilical mesogastrium an image in target (concentric rings with increasing and decreasing echogenicity and thick intestinal wall), which easily alters its form by moving the intestinal liquid once the progression of the peristaltic waves, with proximal intestine dilatation of the same structure, suggesting intestinal intussusception (figure no. 1). This was subsequently confirmed by the CT scan, which shows the presence of the intestinal intussusception cylinders on a length of 15 cm (figure no. 2).
The intestinal intussusception and intestinal obstruction diagnosis was established and an emergency intervention took place (figure no. 3). At about 300 cm from the duodenojejunal (distal jejunum) angle the enteral intussusception segment with a length of about 15-20 cm and the perilesional adenopathies with a maximum diameter of about 1,5-2 cm stand out. After the dessintussusception of the enteral segments, without difficulty, a firm, round-oval shaped tumour developed in the intestinal wall, with a diameter of around 4/3 cm and partial stenosis, without obvious distinction in the enteral serosa (the cause for telescoping the proximal intestine by the latter in the distal segment) is discovered; proximal obstruction dilated enteral loops. A segmentary enterectomy is made, by lifting the intussusception segment and the loco-regional adenopathies (extemporaneously resulting from a neighbourhood node - without elements de malignancy), with the restoration of the digestive continuity by entero-enteroanastomosis latero-lateral suture in double layer.
The histopathology exam of the tumour from the intestinal wall established the gastrointestinal stromal tumour diagnosis (GIST) with low mitotic index (figure no. 4), which required no additional treatment. 6 months post-surgery the patient was completely asymptomatic.
Comments
The intussusception is intestinal the telescoping of one portion of the intestine in the immediately adjacent segment, altering the passage of the intestinal content and compromising the vascular flow. It can occur at the junction of the segments moving freely and the fixed retroperitoneal segments.
It is common with children (the clinical triad: cramps, vomiting, bloody diarrhea), in whose case it is usually primary and benign and rare in adults (5% of the causes digestive obstruction), in whose case it is almost always secondary to another pathological conditions, which alters the bowel movements (table 1).
The case history provides no reliable diagnostic elements in the intestinal intussusception. The abdominal colicative pains, nausea, vomiting, abdominal distension are non-specific symptoms, which often delay the diagnosis. The physical examination and the direct abdominal x-ray signs of intestinal obstruction. The abdominal ultrasound is very useful for children. For the adult the results depend on the examiner’s experience and are hampered by obesity and the presence of gas in the intestine. The most sensitive diagnostic method remains the computed-tomography examination. The magnetic resonance and the exploration with the endoscopic capsule can also be useful.
In the intestinal intussusception are trained the mesenteric vessels. The venous compression generates local edema, which further reduces the blood flow which is already low in the area, leading to the necrosis of the affected intestine segment, perforation and peritonitis. Another complication of the intestinal intussusception is the acute or chronic intestinal occlusion.
The treatment of choice in the adult is surgery, because the substrate in most cases is neoplastic, preceded by sedation, muscle relaxants, hydration. In children it is effective the pneumatic or barium reduction under x-ray or ultrasound control.
Gastrointestinal stromal tumours (GIST) come from the interstitial cells of Cajal, part of the autonomic nervous system, named pacemakers of the intestine whose motility they coordinate. They appear from a mutation of a gene called c-kit that encodes a receptor for a growth factor expressed in the interstitial cells of Cajal. They can be found anywhere in the gastrointestinal tract and are part of the sarcoma family. GIST can be completely asymptomatic or determine the gastrointestinal bleeding, nausea, vomiting, intestinal obstruction. It often mimics the irritable bowel and become quite large before being diagnosed.
The treatment is surgical for localized tumours. Small tumours, with low mitotic index do not recur and after surgery they do not require adjuvant therapy. Tumours larger than 5 cm can spread or recur. C-kit tyrosine kinase inhibitor, imatinib (GLIVEC) 400mg/day, originally used in chronic myeloid leukaemia has been used in the GIST treatment, either pre-surgery (decreases the tumour size), or post-surgery (decreases the recurrence rate) or for patients with no surgical indication.
The patients refractory to imatinib respond to a tyrosine kinase multiple inhibitor – sunitinib.
Conclusion
The intestinal intussusception in the adult is a rare cause of intestinal obstruction and it is almost always secondary to other pathological conditions, in our case a GIST tumour. Both give suggestive symptoms of irritable bowel and the usual, often normal, paraclinical explorations, delay the diagnosis.
Causes of intestinal intussusception in the adult (90% intussusceptions have organic cause, 65% neoplasms):
• Tumours
• Intestinal polyps
• Mekel’s diverticulum
• Cecal duplication
• Intramural haematoma
• Inflammatory bowel diseases
• Lymphomas
• Parasitic infections
• Post-surgery adhesions
• Intestinal motility disorders: irritable bowel, chronic diarrhea, Hirshprung disease
Figure no. 4. Histopathological of gastrointestinal stromal tumour
Figure no. 3. Intraoperative appearance
2 intussuscepted loops
Tumor in the intestinal wall
Bibliography
1. Crowther KS ,Wyld L, Yamany Q, Jacob G. Gastroduodenal intussusception of a gastrointestinal stromal tumour. British Journal of Radiology 2002;75:987-989
2. Marinis A , Yiallourou A , Samanides L, Dafnios N, Anastasopoulos G Vassiliou I, TheodosopoulosT. Intussusception of the bowel in adults: A review. World J Gastroenterol. 2009 ; 15(4): 407–411.
3. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, Miettinen M, O'Leary TJ, Remotti H, Rubin BP, Shmookler B, Sobin LH, Weiss SW. Diagnosis of gastrointestinal stromal tumours: A consensus approach. Hum Pathol. 2002; 33(5): 459-6
4. Gollub MJ. Colonic intussusception: clinical and radiographic features. AJR Am J Roentgenol. 2011; 196(5):W580-5.
5. Soni S, Moss P, Jaiganesh T. Idiopathic adult intussusception. Int J Emerg Med. 2011; 16;4:8
6. Mendez D, Caviness AC, Ma L, Macias CC. The diagnostic accuracy of an abdominal radiograph with signs and symptoms of intussusception. Am J Emerg Med. 2011; 29:10-17
7. Arolfo S, Teggia PM, Nano M. Gastrointestinal stromal tumours: Thirty years’ experience of an Institution. World J Gastroenterol. 2011;17(14):1836-9
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