Eur opean Rev iew for Med ical and Pharmacol ogical Sci ences 2014; 18: 1507-1512 Gastrointestinal tract duplications in children

M.H. OKUR, M.S. ARSLAN, S. ARSLAN, B. AYDOGDU, G. TÜRKÇÜ 1, C. GOYA 2, I. UYGUN, M.K. CIGDEM, A. ÖNEN, S. OTCU

Department of Pediatric Surgery, Dicle University Faculty of Medicine, Diyarbakir, Turkey 1Department of Pathology, Dicle University Faculty of Medicine, Diyarbakir, Turkey 2Department of Radiology, Dicle University Faculty of Medicine, Diyarbakir, Turkey

Abstract. – AIM : Gastrointestinal tract dupli - tract (GI). They mostly occur in distal and cations (GTD) are rare congenital abnormalities have an incidence rate of 4,500 1-12,500 2. GTD that can occur anywhere along the gastrointesti - may be single, multiple, or complex and may nal tract. These anomalies may present as a sin - 3 gle, multiple, or a vague pathologies. Diagnosing present challenges for diagnosis and treatment . and treating these diseases may be difficult in The pathogenesis still remains vague though er - some patients. We aimed to present 32 patients rors in embryological canalization or a part of the who were followed and treated in our clinic. notochord syndrome have been previously sug - PATIENTS AND METHODS: This study included gested 1. GTD symptoms depend on the location the patients between 2000 and 2013. Evaluations of the lesion. Common diagnostic methods in - included clinical presentations, diagnostic strate - gies and algorithms, surgical procedures and as - clude ultrasonography (USG), computed tomog - sociated anomalies, and presence of ectopic tis - raphy (CT), magnetic resonance imaging (MRI), sue, complications, and prognosis. scintigraphy, and upper GI series. Several antena - RESULTS: Common clinical presentations in - tal cases have been reported to be diagnosed by cluded vomiting (n=8; 25%), palpable abdominal USG and MRI 4. mass (n=4; 13%). Twenty-eight patients (2 of them antenatally) were diagnosed preoperatively while Total excision is the method of choice in the four of them were diagnosed at surgery. Ileal du - treatment of GTD. Nevertheless, other methods plications constituted the most common type of therapy may be needed depending on the lo - (34%) while the least common ones were located calization and type of the GTD along with the in , thoracoabdomen and rectum. One of length of the segment involved. We aimed to pre - our patients was present with a gastric duplica - sent the procedures employed in the diagnosis tion which was closely interconnected to a tubu - lar duplication of esophagus, which had never and treatment of 32 GTD patients who had dif - been encountered in the literature before. ferent locations of duplications along GI tract. CONCLUSIONS: It is crucial to note that dupli - cations are likely to occur in various types and numbers and also may accompany other anom - Patients and Methods alies. Computed Tomography (CT) remains the method of choice since Magnetic Resonance (MR) is likely to cause the use of sedation and This retrospective study included 32 patients analgesia at very young ages and it may also be who were diagnosed with GTD and received relatively costly despite being more sensitive in treatment at Dicle University Medical School Pe - soft tissues. Mucosal stripping is an ideal diatric Surgery Clinic between 2000 and 2013. method for the patients requiring restricted Age, gender, clinical presentations, diagnostic surgery. The antenatal asymptomatic cases can be operated after their 6 th months of age. strategies and algorithms, surgical procedures and the patients’ age at surgery, location of GTD, Key Words: associated anomalies, presence of ectopic tissue, Child, Gastrointestinal duplication, Surgery . complications, and prognosis were evaluated. As per our clinical algorithm, the patients presenting with abdominal complaints were primarily diag - Introduction nosed with abdominal graphy + USG. Upper GI series was performed in the patients suspected Gastrointestinal tract duplications (GTD) are with passage problems such as vomiting and di - uncommon congenital abnormalities which can gestion. Depending on these examinations, CT occur anywhere throughout the gastrointestinal was used in most of the patients and MRI was

Corresponding Author: Mehmet Hanifi Okur, MD; e-mail: [email protected] 1507 M.H. Okur, M.S. Arslan, S. Arslan, B. Aydogdu, G. Türkçü, C. Goya, et al. used only for a limited number of patients. this patient, a cystic formation in the greater cur - Scintigraphy was rarely used, only for studying vature of the stomach with the size of 10 × 6 cm the ectopic mucosa. was detected to be thoroughly linked to a tubular duplication of 4-5 cm stretching along the esoph - agus gut. The gastric cyst was managed with mu - Results cosal stripping from the common wall and the tubular esophagus segment was excised through The 32 patients consisted of 9 (28%) male total excision. Histopathological examination on and 23 (71%) female patients with a mean age the samples obtained from the gastroesophageal of 2.7 years (5 days-10 years). Eight of them junction revealed two distinct patterns of mucos - were infant and 5 of them were neonate at ad - al tissue (gastric and oesophageal) each of which mission. Common clinical presentations includ - complying with their source. ed vomiting (n=8; 25%), palpable abdominal Seven patients were present with associated mass (n=4; 13%), and abdominal distension anomalies. Common anomalies included di - (n=5; 16%). USG was performed in 28, CT in astematomyelia, BlockVertebra, malrotation, 21, MRI in 3, and scintigraphy in 5 patients. congenital dislocation of the hip, hydronephrosis, Twenty-eight patients (2 of them being antena - , and anal atresia with rectovestibular fis - tal) were diagnosed preoperatively, whereas 4 tula. Cystic GTD were present in 25 (78%) and patients were diagnosed incidentally during the tubular in 7 (22%) patients. Surgery was per - exploration of invagination, acute abdomen, formed in 30 patients following the diagnosis, cloacal malformation and anal atresia + RV fis - whereas 2 asymptomatic patients were diagnosed tula. Ileum (n=11; 34%) constituted the most antenatally and received surgery after the 6 th common site of GTD, followed by duodenum month of age. Twenty-seven patients received la - (n=5; 16%), jejunum (n=3), colon (n=3), stom - parotomy, 2 laparoscopy, 2 laparotomy + thora - ach (n=2), ileocecum (n=2), appendix (n=2), cotomy, and 1 thoracotomy. Nineteen patients thoracoabdomen (n=2), esophagus (n=1), and underwent resection anastomosis, 12 mucosal rectum (n=1) (Table I and Figures 1, 3 ). stripping, and 1 patient received both. Ectopic One of our patients was present with gastric gastric mucosa was observed in 6 (19%) patients duplication which was closely interconnected to (5 in the stomach and 1 in the pancreas). One pa - tubular duplication of esophagus (Figure 2). In tient developed wound area infection and another

Table I. Clinical characteristics of duplications.

Patient Ectopic tissue Additional n (%) Type (n) Complaint (n) (n) anomalies (n)

Esophagus 1 (3) Cystic (1) Respiratory distress (1) Gastric (1) Gastric 2 (6) Cystic (2) Vomiting (2) Duodenum 5 (16) Cystic (5) Vomiting 5) Jejunal 3 (9) Cystic (3) Vomiting (1) Mass (1) Ileal 11 (34) Tubular (1) Abdominal distension (4) Pancreas (1) Malrotation (1) Cystic (10) Mass (3) Gastric (1) Congenital hip Abdominal pain (2) dislocation (1) Bleeding (1) Thoracoabdominal 2 (6) Tubular (2) Respiratory distress (2) Gastric (1) (1) BlockVertebra (1)

Ileocecal 2 (6) Cystic (2) Abdominal distension (1) Hydronephrosis (1) Abdominal pain (1) Appendix 2 (6) Tubular (2) _ Cloaca (1) AA + RV (1) Colonic 3 (9) Tubular (2) Constipation (1) Gastric (1) Cystic (1) Bleeding (1) Abdominal pain (1) Rectum 1 (3) Cystic (1) Constipation (1) Total 32 57

1508 Gastrointestinal tract duplications in children

Figure 1. A, Axial CT section; stomach ( yellow arrow ), gastric duplication cyst (red arrow), esophageal duplication cyst (green arrow). B, Macroscopic appearance. C, Microscopic appearance and transition zone patient developed treatable ileus. One patient acute abdomen invagination, Hirschsprung’s dis - died of sepsis. Mean hospital stay in our series ease, etc.) or may accompany these diseases as was 6 days. well 5,6 . These duplications are best explained by the development of fetal intestinal diverticulitis, vacuolization, caudal duplication and split noto - Discussion chord theory 7,8 . GTD cysts may be single or mul - tiple and they are twice more common in females GTD are rare abnormalities which are general - than males 9,10 . Similarly, the females in our study ly diagnosed at early ages. These anomalies may had an outstanding preponderance (71%) over occur anywhere along the gastrointestinal tract males. Jejunal and/or ileal duplications (44-53%) from the mouth down to the anus. GTD may be comprise the most common type, followed by asymptomatic or present with vague symptoms colonic (13-15%), gastric (7-9%), duodenal (4- mimicking some other common pathologies (e.g. 6%), and rectal (3-4%) duplications 4,10,11 . In a

Figure 2. A, Appendiceal. B, Colonic duplication.

1509 M.H. Okur, M.S. Arslan, S. Arslan, B. Aydogdu, G. Türkçü, C. Goya, et al.

Figure 3. A, Ileocecal. B, Duodenal duplication. similar vein, 47% of our patients had jejunal pain. Colonic and rectal duplications are mostly and/or ileal while 16% had colonic and 3% had accompanied by constipation and bleeding. We rectal duplications. consider that GTD must be suspected in the pa - Thoracoabdominal duplications are reported to tients suffering from any of the symptoms afore - constitute 2-4% of all GTD types 11-13 . These du - mentioned. plications were only present in two of our pa - USG, the graphy of upper GI, CT, MRI, and tients (6%). Complex duplications are also re - scintigraphy are commonly reported as useful vi - ported in the literature; however, in one of our sualization techniques for GTD 15-17 . Although usg patients, who was present with gastric duplica - is valuable for diagnosis, in some cases USG pre - tion, we observed something which had never sents a restrictive use particularly in rectal dupli - been reported before: the patient presented with a cations and also in detecting the relations of the cystic duplication in the stomach which was lesions with their surroundings and associated closely related to the tubular duplication in the anomalies. Instead, MRI or CT may be employed esophagus. Keeping this notion in mind, the to provide a clear imaging of these situations. physician should conduct a detailed exploration Moreover, MRI is reported to be quite sensitive in GTD patients since these anomalies are quite in soft tissues 18 . On the other hand, MRI has likely to occur in unexpected ways. some disadvantages such as causing sedation or The symptoms of GTD are often related to the anesthesia in the early years of age and being rel - location, size and mucosal lining of the duplica - atively costly. In our series, CT was the common tions. Oral and esophageal lesions may cause res - technique used for visualization. We consider piratory difficulties while gastrointestinal dupli - that CT must be the method of choice when MRI cations may lead to nausea, vomiting, obstruc - seems impractical. The series of upper gastroin - tion, hemorrhage, or perforation 5,14 . In our series, testinal may be useful for the patients presenting the patients with esophageal duplications pre - with nutritional problems and vomiting. sented with respiratory difficulties and the ones Scintigraphy has been reported useful in de - with gastro duodenal lesions were mostly suffer - tecting the ectopic gastric mucosa. Ectopic mu - ing from vomiting and nutritional problems. In cosa does not exist in 68-73% of the GTD pa - ileal and jejunal duplications most common tients. In the patients with ectopic mucosa, the symptoms were palpable mass, abdominal dis - mucosa is generally of gastric origin while pan - tension, pain, and bleeding, respectively. One pa - creatic origin is also possible 12,13 . Moreover, gas - tient with ileal duplication was present with in - tric mucosa can also originate from Meckel’s di - vagination. Another patient with ileocecal lesion verticulum 19 . Conducting several experimenta - was brought to our clinic with an abdominal tions using the gamma camera technique, Priebe

1510 Gastrointestinal tract duplications in children et al 20 reported that an ectopic tissue with a size for ileocecal duplications and for the cases with of at least 1.8 cm 2 is needed to provide a clear multiple lesions who have the risk of short bow - imaging for the ectopic gastric mucosa. Other el. One of our patients received mucosal strip - studies argue that the substance injected in the ping in a combination with resection anastomo - cases with peptic ulceration, inflammation, intus - sis. We believe that a detailed exploration is es - susception, hemangioma, arteriovenous malfor - sential in GTD since these anomalies may occur mations, bowel obstructions, and urethral anom - as multiple duplications. Reported mortality rate alies may lead to nonspecific uptake 21-23 . There is is 4-20% while the rate in our series was only no current consensus as to whether the use of 3%. One patient died of sepsis following the op - scintigraphy in the detection of ectopic tissue eration for esophageal duplication. We attribute makes any effect on determining the surgical our low rate of mortality to the recent advance - strategy. ments in the diagnostic and treatment techniques. In the cases where surgery is restricted due to the site of the lesion, the first thing to discover is to detect the presence of ectopic mucosa. In the Conclusions partial resection of ectopic gastric mucosa, the cyst should be excised since acid secreting mu - It is commonly accepted that GTD cysts may cosa may lead to complications such as haemor - occur in different shapes and numbers and also rhage, erosion, or malignant degeneration 6,24 . may accompany other anomalies. CT remains the Scintigraphy was used in our two patients who method of choice since MRI is likely to cause the presented with lower GI hemorrhage, and in an - use of sedation and analgesia at very young ages other patient the colonic duplication was excised and it may also be relatively costly despite being at surgery since it had been suggested by scintig - more sensitive in soft tissues. Scintigraphy is raphy to be the origin of bleeding in the ectopic rarely needed in GTD cases. While resection is gastric mucosa. The patient who did not present the method of treatment for duplications, mucos - with ectopic mucosa on scintigraphy was operat - al stripping is quite useful in the cases with re - ed under elective conditions since the hemor - stricted surgery. Surgical intervention is general - rhage stopped spontaneously. Depending on ly performed at the age of diagnosis, whereas the scintigraphic images, 4 patients had negative and surgery for the patients diagnosed at antenatal 1 patient had positive findings. These results period may be performed following the 6 th month were confirmed by the pathological analysis. We of age since they are generally asymptomatic. consider that scintigraphy could only be used in the patients undergoing restricted surgery, who –––––––––––––––– –-– –– present with unlocatable hemorrhage, to clarify Conflict of Interest whether the bleeding stems from the ectopic gas - The Authors declare that there are no conflicts of interest. tric mucosa. Associated anomalies are reported with an in - cidence rate of 16-26% 25 . The frequency of asso - References ciated anomalies in our series (22%) was similar to the rate reported in the literature, and vertebral 1) O’N EILL JA, R OWE MI, G ROSFELD JL, F ONKALSRUD E, CORAN A. Pediatric surgery. St. Louis, MO, Mosby anomalies constituted the 6% of our patients. Year-Book, 1998. Surgical intervention is generally performed at 2) COLLINS DC . A Study of 50,000 specimens of the the age of diagnosis; however, the surgery for an - human vermiform appendix. Surg Gynecol Obstet tenatal patients may be performed after 6 th month 1955; 101: 437-445. 4,5,14 of age since they are generally asymptomatic . 3) MAC PHERSON RI . Gastrointestinal tract duplica - Most of our patients underwent surgery shortly tions: clinical, pathologic, etiologic, and radio - after their diagnosis while only 2 antenatal pa - logic considerations. Radiographics 1993; 13: tients were operated on in the 6 th month of their 1063-1080. ages. Commonly reported surgical procedures 4) OFFIR BEN -I SHAY . Multiple duplication cysts diag - nosed prenatally: case report and review of the are thoracotomy, thoracotomy + laparotomy, la - literature. Pediatr Surg Int 2013; 29: 397-400. parotomy, or laparoscopy 4,11,13 . 5) SCHALAMON J, S CHLEEF J, H ÖLLWARTH ME. Experi - Mucosal stripping was used to excise the cysts ence with gastro-intestinal duplications in child - in the cases with limited resection anastomosis. hood Langenbeck’s Arch Surg 2000; 385: 402- Mucosal stripping is an ideal method specifically 405 .

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