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N-acetylcysteine&#44; polyethylene glycol &#91;PEG&#93;&#44; or meglumine diatrizoate &#91;MD&#44; Gastrografin<span class="elsevierStyleSup">&#174;</span>&#44; Schering&#44; Berlin&#93;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> In this article&#44; we present the cases of three patients with CF that suffered several episodes of DIOS&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case 1</span><p id="par0010" class="elsevierStylePara elsevierViewall">Male patient aged 11 years with CF DF508&#44; afebrile&#44; presenting with abdominal pain and distension&#44; constipation and bilious vomiting of 48 hours&#8217; duration&#46; Abdominal radiography showed abundant faecal material in the right iliac fossa&#44; while the ultrasound findings were normal&#46; Treatment with N-acetylcysteine via enema was initiated due to suspected DIOS&#44; leading to partial relief of symptoms and the production of a small volume of soft stools&#46; At 24<span class="elsevierStyleHsp" style=""></span>h&#44; a barium enema with Gastrografin<span class="elsevierStyleSup">&#174;</span> was performed under radiographic control&#44; and the episode resolved&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case 2</span><p id="par0015" class="elsevierStylePara elsevierViewall">Female patient aged 6 years with CF DF508 presenting with constipation&#44; bilious vomiting and abdominal pain of two days&#8217; duration&#46; Treatment with phosphate enemas was initiated for suspected DIOS&#44; followed by an N-acetylcysteine enema that was repeated at 24<span class="elsevierStyleHsp" style=""></span>h&#44; resulting in the production of soft stools and the resolution of symptoms&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case 3</span><p id="par0020" class="elsevierStylePara elsevierViewall">Female patient aged 5 years with CF DF508&#44; presenting with DIOS of 4 days&#8217; duration with a sudden worsening of symptoms after conservative treatment&#46; A laparotomy was performed&#44; revealing plastic peritonitis and faecal impaction in the distal ileum and colon&#46; Manual disimpaction was performed&#44; followed by caecostomy &#40;Chait procedure&#41; with placement of a Kher tube used in subsequent days to start treatment with antegrade lavage with N-acetylcysteine and PEG&#46; The Kher tube was replaced by a Chait trapdoor &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; for continued delivery of enemas&#44; which was removed at seven months&#46; In the four years that followed&#44; the patient had two new episodes of DIOS &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; that were treated early with rectal Gastrografin<span class="elsevierStyleSup">&#174;</span> gravity enemas &#40;in the ward&#41;&#44; with resolution of symptoms in both&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The conservative management of DIOS by means of laxatives or surfactants&#44; administered either orally &#40;in cases of low-grade obstruction&#41; or via enema&#44; is effective in 89&#8211;96&#37; of cases&#44;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> especially if it is initiated early&#46; The administration of PEG at doses of 2<span class="elsevierStyleHsp" style=""></span>g&#47;kg&#47;day &#40;or 20&#8211;40<span class="elsevierStyleHsp" style=""></span>mL&#47;kg&#47;h of solution&#44; administered orally or with a nasogastric tube over 6&#8211;8<span class="elsevierStyleHsp" style=""></span>h&#41; is recommended&#44; or alternatively a solution of 50<span class="elsevierStyleHsp" style=""></span>mL of Gastrografin<span class="elsevierStyleSup">&#174;</span> in 200<span class="elsevierStyleHsp" style=""></span>mL of water in children up to 6 years of age and of double this amount for older patients&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The treatment can be repeated on subsequent days with the administration of half doses&#46; 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Scientific Letter
Treatment options of distal intestinal obstruction syndrome: And if the enemas fail?
Opciones de tratamiento del síndrome de obstrucción intestinal distal: ¿y si los enemas fallan?
M. Fernández-Ibietaa,
Corresponding author
mfndezibieta@hotmail.com

Corresponding author.
, L. Ayuso-Gonzálezb, M.S. Fernández-Córdobac, Y. Argumosa-Salazarc, J. Gonzálvez-Piñerad
a Servicio de Cirugía Pediátrica, Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain
b Servicio de Cirugía Pediátrica, Hospital Virgen del Camino, Complejo Hospitalario de Navarra, Pamplona, Navarra, Spain
c Servicio de Cirugía Pediátrica, Hospital General Universitario de Albacete, Albacete, Spain
d Servicio de Cirugía Pediátrica, Hospital General de Alicante, Alicante, Spain
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along with obstruction of varying degrees &#40;vomiting of bilious or faecal material&#44; abdominal pain and distension&#41;&#46; It is essential to differentiate DIOS from constipation&#44; appendicitis&#44; intussusception&#44; adhesion-related disorders&#44; Crohn&#39;s disease and fibrosing colonopathy&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;4</span></a> The insufficient secretion of chloride and water&#44; a slow intestinal transit&#44; impaired ileal secretion and fat malabsorption are predisposing factors&#46; There is no evidence of a link between DIOS and excessive pancreatic enzyme supplementation&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Some of the risk factors for DIOS are&#58; severe genotypes &#40;DF508&#44; W1282X&#41;&#44; pancreatic insufficiency&#44; dehydration&#44; history of meconium ileus&#44; lung transplant and a previous episode of DIOS&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> The management of DIOS is usually conservative by means of antegrade or retrograde lavage with laxative or surfactant enemas &#40;phosphate&#44; N-acetylcysteine&#44; polyethylene glycol &#91;PEG&#93;&#44; or meglumine diatrizoate &#91;MD&#44; Gastrografin<span class="elsevierStyleSup">&#174;</span>&#44; Schering&#44; Berlin&#93;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> In this article&#44; we present the cases of three patients with CF that suffered several episodes of DIOS&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case 1</span><p id="par0010" class="elsevierStylePara elsevierViewall">Male patient aged 11 years with CF DF508&#44; afebrile&#44; presenting with abdominal pain and distension&#44; constipation and bilious vomiting of 48 hours&#8217; duration&#46; Abdominal radiography showed abundant faecal material in the right iliac fossa&#44; while the ultrasound findings were normal&#46; Treatment with N-acetylcysteine via enema was initiated due to suspected DIOS&#44; leading to partial relief of symptoms and the production of a small volume of soft stools&#46; At 24<span class="elsevierStyleHsp" style=""></span>h&#44; a barium enema with Gastrografin<span class="elsevierStyleSup">&#174;</span> was performed under radiographic control&#44; and the episode resolved&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case 2</span><p id="par0015" class="elsevierStylePara elsevierViewall">Female patient aged 6 years with CF DF508 presenting with constipation&#44; bilious vomiting and abdominal pain of two days&#8217; duration&#46; Treatment with phosphate enemas was initiated for suspected DIOS&#44; followed by an N-acetylcysteine enema that was repeated at 24<span class="elsevierStyleHsp" style=""></span>h&#44; resulting in the production of soft stools and the resolution of symptoms&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case 3</span><p id="par0020" class="elsevierStylePara elsevierViewall">Female patient aged 5 years with CF DF508&#44; presenting with DIOS of 4 days&#8217; duration with a sudden worsening of symptoms after conservative treatment&#46; A laparotomy was performed&#44; revealing plastic peritonitis and faecal impaction in the distal ileum and colon&#46; Manual disimpaction was performed&#44; followed by caecostomy &#40;Chait procedure&#41; with placement of a Kher tube used in subsequent days to start treatment with antegrade lavage with N-acetylcysteine and PEG&#46; The Kher tube was replaced by a Chait trapdoor &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; for continued delivery of enemas&#44; which was removed at seven months&#46; In the four years that followed&#44; the patient had two new episodes of DIOS &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; that were treated early with rectal Gastrografin<span class="elsevierStyleSup">&#174;</span> gravity enemas &#40;in the ward&#41;&#44; with resolution of symptoms in both&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The conservative management of DIOS by means of laxatives or surfactants&#44; administered either orally &#40;in cases of low-grade obstruction&#41; or via enema&#44; is effective in 89&#8211;96&#37; of cases&#44;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> especially if it is initiated early&#46; The administration of PEG at doses of 2<span class="elsevierStyleHsp" style=""></span>g&#47;kg&#47;day &#40;or 20&#8211;40<span class="elsevierStyleHsp" style=""></span>mL&#47;kg&#47;h of solution&#44; administered orally or with a nasogastric tube over 6&#8211;8<span class="elsevierStyleHsp" style=""></span>h&#41; is recommended&#44; or alternatively a solution of 50<span class="elsevierStyleHsp" style=""></span>mL of Gastrografin<span class="elsevierStyleSup">&#174;</span> in 200<span class="elsevierStyleHsp" style=""></span>mL of water in children up to 6 years of age and of double this amount for older patients&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The treatment can be repeated on subsequent days with the administration of half doses&#46; The use of rectal lavage with water and&#47;or Gastrografin<span class="elsevierStyleSup">&#174;</span> delivered directly to the caecum by colonoscopy has also been reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">5&#44;6</span></a> In case of treatment failure or poor response to treatment&#44; the surgical approach should be based on disimpaction with washout of the ileocaecal area&#44; and enterostomy or caecostomy are preferred over intestinal resection&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;5&#44;6</span></a> We chose to perform a caecostomy with placement of a temporary button in our patient because a complete disimpaction was not possible and due to the presence of peritonitis&#46; This option allowed the patient to continue undergoing antegrade lavage efficiently during the convalescence period&#46; Another&#44; more permanent alternative reported in adult CF patients with recurrent DIOS refractory to conservative treatment and with poor adherence to treatment &#40;as a last step in management&#44; after failed enemas and having decided against placement of a Chait caecostomy catheter&#41;&#44; is the performance of an antegrade continence enema &#40;ACE&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> bringing the appendix out onto the abdominal wall to create a permanent stoma through which the bowel can be washed out periodically&#46;</p></span></span>"
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