STUDY ON CLINICAL PROFILE , MANAGEMENT & OUTCOME OF GASTROINTESTINAL DUPLICATION IN CHILDREN

Duplication of the gastrointestinal tract occur any part of the alimentary tract from the tongue to the anus. Male have slightly more predominance than females. Gastrointestinal duplication will have a varied presentation. Ileum and the oesophagus are most commonly involved. Colonic duplication is rare and can present with diagnostic difficulties. Gastrointestinal duplication has presence of well developed coat of smooth muscle, intimately attached to the gastrointestinal tract in the mesentric region and show a common blood supply with the native bowel. Sometime it will have an epithelial lining representing some portion of the alimentary tract.

Duplication of the gastrointestinal tract occur any part of the alimentary tract from the tongue to the anus. Male have slightly more predominance than females. Gastrointestinal duplication will have a varied presentation. Ileum and the oesophagus are most commonly involved. Colonic duplication is rare and can present with diagnostic difficulties. Gastrointestinal duplication has presence of well developed coat of smooth muscle, intimately attached to the gastrointestinal tract in the mesentric region and show a common blood supply with the native bowel. Sometime it will have an epithelial lining representing some portion of the alimentary tract.

…………………………………………………………………………………………………….... Introduction:-
In 1733 Calder reported first case of gastrointestinal duplication. William E. Ladd coined the term gastrointestinal duplication in 1930, that included congenital anomalies of the foregut , midgut and hindgut. The incidence is of 1 in every 4500 autopsies. Enteric duplications are multiple in 10% to 20% cases. Duplication of the gastrointestinal intestinal tract occur any part of the alimentary tract from the tongue to the anus. Male have slightly more predominance than females. Gastrointestinal duplication will have a varied presentation. Ileum and the oesophagus are most commonly involved. Colonic duplication is rare and can present with diagnostic difficulties. Gastrointestinal duplication has presence of well developed coat of smooth muscle, intimately attached to the gastrointestinal tract in the mesentric region and show a common blood supply with the native bowel. Sometime it will have an epithelial lining representing some portion of the alimentary tract.

Clinical features
Prenatal ultrasonography will pick up gastrointestinal duplication 11,12 in majority of cases. Within two yrs of age most of the gastrointestinal duplication are diagnosed 14 . Depending on the location ( foregut, midgut, hindgut ) the size of the duplication , the presence of hetertopic mucosa will have varied presentation. Some of the duplication will be diagnosed incidentally on evaluation of nonspecific symptoms like abdominal pain , constipation and cough. Some of them will present as emergency with symptoms like ulceration, bleeding and perforation from the heterotopic gastric mucosa in the gastrointestinal duplication. Most of the cases of ileal duplication are taken as emergency as acute appendicitis.
Gastrointestinal duplication can be either cystic or tubular, seen on the mesenteric border. Presence of gastrointestinal duplication in the thoracic cavity can have symptoms like respiratory distress, failure to thrive, dysphagia and vomiting . Hemorrhage and infection of the gastrointestinal duplication in the thoracic cavity can cause sudden increase in the size of the swelling and may present as chest pain and respiratory distress.

Diagnosis
Van Dam in 1984 diagnosed enteric duplication in an antenatal mother at 20 weeks 15 . Hughes 11 suspected gastric duplication noticed as cyst at the gestational age of 16 weeks in antenatal ultrasound. Correia Pinto et al diagnosed antenatally 14 cases of enteric duplication diagnosed by ultrasonogram (USG) 13. . Postnatal evaluation of the antenatally diagnosed cases will show an anechoic cysts. The two important ultrasound signs are the presence of peristalsis and the "double wall" sign. The "double wall" signs 16,17 means the inner hyperechoic rim indicating 262 mucosa and submucosa and the outer hypoechoic indicating muscularis propria. The duplication cyst wall will show both smooth muscle and mucosa.Thoracic duplication presented as hydrops fetalis and in utero thoracoamniotic shunting done. Technetium TC 99m pertechnetate scan done in cases . patients presenting with bleeding per rectum and ultrasound picks up a cystic lesion in the abdomen Foregut duplication better diagnosed by the endoultrasonography. X-ray of the spine will show segmentation errors. Gastrointestinal contrast study will show the communication of the tubular duplication to the native bowel and compression by the cystic duplication in the adjacent bowel. Enteric duplication will have enhancing rim in computed tomography.
Magnetic resonance imaging (MRI) will help in the diagnosis and it is more accurate than ultrasound in delenating fetal anatomy. In fetal MRI Gastrointestinal duplications appear hyperintense in T1 -weighted images and hypointense in T2 -weighted images.

Cervical esophageal duplications
This is a rarest type of esophageal duplication. Blasius in 1971 reported first case of cervical esophageal duplication cyst 18 . The incidence is 1 in 8200. Duplication cyst of esophagus are benign, cystic lesion derived from primitive foregut that occur in neck, mediastinum or both. Two thirds of the duplication occur on the right side of the mediastinum.
About 80% of the cervical esophagus will not communicate with the esophageal lumen. Foregut duplication cyst are of three types bronchogenic, esophageal and neuroenteric. Among the duplication, esophagus is the second most commonest site of duplication.
In the mediastinum bronchogenic cyst is the most commonest cystic lesion. If the esophageal duplication cyst extends into spinal canal then it is called as neuroenteric cyst. About 60% occur in the lower one third of the esophagus . Most of them are incidentally noted. They usually present with respiratory distress that will need emergency intubation and the rest of them may present with the symptoms of either dysphagia, epigastric discomfort and retrosternal pain. Treatment is complete excision of the duplication or excision of the mucosa if excision of the duplication not possible . The approach for the excision of the cyst are axillary thoracotomy, cervical excision, median sternotomy or by thoracoscopy. Review of literature showed 18 cases reported as of cervical esophageal duplication cyst 19 . An early detection of cervical oesophageal duplication was done in an antenatal mother at 30 weeks and operated at 35 days of life due to respiratory failure. The differential diagnosis for the duplication cyst in the neck are thyroglossal cyst and lymphatic cyst.

Thoracic and thoracoadbominal duplications
Majority of the duplication present in the posterior mediastinum lower half. As these duplications are connected to the spinal canal with vertebral anomalies they are referred as neuroenteric cyst. About 20% of the duplication arise in the thorax or thoracoabdominal 20 . In thoracic duplication diagnosed antenatally in utero therapy is done if there is fetal distress.
Some of the duplication are noticed incidentally in chest x ray. Computed tomography (CT) of the thorax and the abdomen, done in cases of thoracic duplication, because of the association of the abdominal duplication in more than one fourth of the cases. To know the relation of the duplication to the spinal canal, magnetic resonance imaging (MRI) of the spine done A oral contrast study will define the relation of the duplication cyst to the esophagus. The differential diagnosis of the mediastinal duplication are neurogenic tumors and the pericardial cyst.Technetium Tc 99m pertechnetate scan picks up presence of ectopic gastric mucosa in the thoracic duplication cyst. The excision of the duplication can be done either thoractomy or by thoracoscopically. If excision of the duplication not possible then excision of the mucosa of the duplication done to avoid recurrence and malignancy. Thoracoscopic aspiration of the cyst can be done in case of a large thoracic duplication before excision. In cases of thoracic duplication with intraspinal extension, intraspinous portion of the cyst should be done. Excision of large thoracoabdominal duplication may be done in staged procedure.

Abdominal foregut duplications
Along the greater curvature most of the gastric duplication occur. About 82% are cystic and non communicating but they will have a common blood supply and muscular layer 21 ,18% are tubular and communicating.
There are four case reports of isolated duplication cyst (IDC) 21 which shows no connection with the alimentary tract but two of them showed gastric mucosa 21 .They present at younger age with the symptoms of abdominal distension, vomiting, pain abdomen, gastroesophageal reflux and with anemia due to the bleeding from the ectopic gastric mucosa. They may even present with pneumoperitoneum or hemoperitoneum. Ultrasound will pick up a large cystic mass in relation to the stomach. Oral contrast study will help to know the relationship to the stomach and the extent of the lesion. The differential diagnosis of a large right hypochondrial gastric duplication is choledochal cyst.
The treatment of gastric duplication is complete excision if possible. If complete excision not possible, then partial cyst excision or segmental gastrectomy or excision of the mucosa can be done.

Pyloric duplication
Pyloric duplication can present as early as six days of life. They usually present with symptoms that mimic hypertrophic pyloric stenosis. Ultrasonogram (USG) will be of little benefit in diagnosing pyloric duplication and very difficult in differenciating from hypertrophic pyloric stenosis. There are case reports of pyloric duplication with aberrant pancreatic duct 22 . Complete excision of the pyloric duplication is the treatment of choice. Duodenal duplication incidence is 5% -7%. Normally located in the medial border of duodenum and will be noncommunicating. They are more common in the first and second part of duodenum. It will have varied presentation like nausea, weight loss, vomiting, jaundice and gastrointestinal bleeding.
There are case reports of recurrent pancreatitis, due to the involvement of ampulla of vater in the wall of duodenal duplication. Adenocarcinoma arising from the Duodenal duplication reported.
The differential diagnosis of duodenal duplication in the right hypochondrium is choledochol cyst. Endoscopic retrograde cholangiopancreatography (ERCP) and Magnetic resonance cholangiography (MRCP ) will help in the diagnosis ,whether duodenal duplication is communicating with ampulla of vater or not. Treatment is resection of the duodenal duplication and reconstruction with intraoperative cholangiography to know whether biliary communication is there or not. If resection not possible then either marsupialization of the cyst or Roux-en-Y loop of jejunum anastomosed to the cyst can be done. Cystic duplication can be treated by endoscopic incision if the ampulla of vater not involved. Short hospital stay and procedure time are the advantages of endoscopic excision.

Duplication of the small intestine
Most commonest gastrointestinal duplication. Majority occur in the ileum. Duplication of the small intestine can be either tubular (18%) or cystic (82%). Duplication may be communicating or non communicating.
Child can present with abdominal pain, distension and vomiting. In small bowel intussusceptions these cystic duplication may act as a lead point. They may present as bleeding and perforation from the ectopic gastric mucosa in the duplication cyst which can be diagnosed by the technetium-99m pertechnetate scan. Depending upon the blood supply there are two type of duplicatons Type I: (Parallel) the artery supplying the duplication will be on one side and the artery supplying the native bowel will be on the other side Type II : (Intramesentric) the mesenteric vessel on both sides will pass through the duplication and will enter the native bowel 268 Li and colleagues found 90% of the type II lesion had thoracic vertebral anomaly. The treatment for type I duplicaton is dividing the mesentry with straight artery and contralateral artery safe guarded. In type II the small branches of the straight arteries are divided and the duplication is enucleated 23 . If duplication is not resectable then Roux-en-Y loop of jejunum anastomosed to the cyst.

Hindgut duplications
Colonic and rectal duplications constitute 17% of gastrointestinal duplication. They have been found in the conjoined twins and may be associated with duplication of the genitalia and urinary tract. Rectal duplication more often present as a cystic type in the presacral region behind the rectum. Sometimes the tubular duplication will be extensive involving the entire colon and present as second opening in the perineum or in the posterior wall of the vagina. Hindgut duplications are of three classes. The first class is the short tubular mass or cystic type seen in the mesentry of the colon and they are rare. The second class is the midline cystic mass behind the rectum Fig 14:-Large rectal duplication in front of the sacrum pushing rectum anteriorly and in front of the sacrum or coccyx and sometimes they will extend into the peritoneal cavity. This can be removed without damaging the blood supply of the rectum. The third class is more common in females and they are side to side duplication of the rectum 270 and colon. They are more commonly associated with spina bifida and genitourinary anomalies. The treatment is fenestration of the duplication lumen and or partial resection.
Hindgut duplication can present as rectovaginal fistula, abdominal distension and constipation or passing flatus and feces through the vagina. Other presentations are rectal bleeding, perirectal abscesses or fistula. Computed tomography (CT) , magnetic resonance imaging (MRI) and retrograde injection of the contrast media will show the relation of the duplication to the rectum and the extent of the duplication. An autosomal dominant condition called Curarino triad is the presacral mass along with the rectal stenosis and sacral anomalies.
Short cystic duplication can be treated with limited colon resection or excision of the duplication from the mesentery. Presacral duplication can be removed by posterior sagittal approach . In case of infected presacral duplication it is better to drain externally than into the rectum. Sometimes a combined abdominal and perineal approach may be needed for the excision of the duplication 24 . During excision of the presacral lesion, the chance of injuring the bladder neck, sphincter and ureters are more ,so preoperative stenting of the ureters may help in decreasing the injury. In case of non resectability of the lesion temporary diverting colostomy can be done. The commonest complication is recurrence if not properly In long-side -by-side duplication fenestrating the two lumina both proximally and distally can be done. In case of rectal duplication with two perineal openings excision of the duplication that does not pass through the sphincter is done and may also need sphincter muscle complex reconstruction 23 .   The most commonest presentation is the abdominal distension.

Observations And Results:-
Eight cases presented only with abdominal distension.

33) The age and sex distribution
In our study out of the 20 cases,15 cases are male children(75%) and 5 cases are female children(25%). Many of the cases presented from within one day of life to 7 months and the oldest child was 8 years. This study was compared to the study conduted by Hacettepe university, Department of Pediatric Surgery, Ankara, Turkey by Karnak I, found 53% are male children and 69% presented within the age of 1 year 27 .

ISSN: 2320-5407
Int. J. Adv. Res. 9(05), 259-293 292 Five cases had antenatal scan, out of that two cases was diagnosed possibility of gastrointestinal duplication. Karnak I in his study found that 70% -80% of the cases are detected antenatally and out of that 30% will need emergency surgery 27 . Puligandala diagnosed 31% of the duplication antenatally 26 .

Mode of presentation and clinical examination
The commonest presentation observed is abdominal distension (40%) Two cases with respiratory failure and two cases with constipation noted. The commonest clinical finding is a mass palpable in the right iliac fossa and one case had two anal opening.
Karnak I in his study found that the mode of presentation depends upon the anatomic location of the cyst, pressure effect, complications like presence of ectopic gastric mucosa, torsion of duplication 27 . Karnak I observed the most commonest presentation abdominal mass, abdominal distension constipation , respiratory distress and in 33% he felt the mass clinically 27 .

Location, type of duplication and surgical procedure
The commonest site of duplication observed is ileum (55%) and cystic type of duplication is predominant (80%). 50% of the tubular duplication noticed in the midgut and 75% were communicating. Only two cases had multiple duplication (10%).
Four cases had emergency surgery out of the four cases two cases had perforation in the duplication and two had torsion with gangrene of the duplication. Karnak I observed 70% of the duplication observed in the ileum, 79% are cystic duplication, 21% are tubular duplication and 5.3% had multiple duplication 27 . H.K. Ramakrishna in his study found that most of the duplication are in ileum, 74% are cystic duplication and 26 % are tubular duplication 23 .
Excision of the duplication done in 8 cases(40%), resection of the duplication and end to end anastomosis done in 6 cases(30%) and excision of the mucosa done in one case. Karnak I observed many of the cases had excision of the duplication(53%) 27 . Li et al has done excision of the duplication without resection in 14 cases (6 -tubular,8cystic) 28 .

Associated anomaly
Six cases had hemivertebrae(30%), one case had rectovestibular fistula with complete colonic duplication. Karnak I observed hemivertebrae in 26% of the cases 27 . Blickman in his study found that association of colonic duplication with rectovestibular fistula 29 .

Cause of acute presentation
Two cases had volvulus and two cases had perforation. Puliganda PS in his study found that volvulus was noticed in 26.3% of the duplication and 20% had perforation of the gastrointestinal duplication 26 . None of our patients had intussusception and lower gastrointestinal bleeding.

Cause of death
Two cases had respiratory failure and two cases had septicaemia followed by death. Karnak I observed six cases died of unrelated cause 27 . Cases presented with respiratory distress due to the duplication cyst in the thoracic cavity caries a poor prognosis.

Histopathological finding
In our series 5 patients had heterotopic mucosa. One case was reported as lymphangioma and the others reported as duplication cyst. Karnak I diagnosed 33% had heterotopic gastic mucosa 27 .
Conclusion:-1. A good antenatal ultrasound can pick up gastrointestinal duplication, requiring further evaluation and elective surgery 2. Incidentally noted gastrointestinal duplication should be operated electively to avoid complications like bleeding and perforation from the ectopic gastric mucosa. 3. Most of the gastrointestinal duplication will present within 1 year of age 4. Gastrointestinal duplication is more common in males 5. Ileum is the most commonest site for gastrointestinal duplication 6. Cystic and communicating type of gastrointestinal duplication are most common types of duplication