Intestinal cystic duplication is rare vital abnormality which can be found in any location along the digestive tract from the mouth to the anus. The incidence rate is 1 per 4,500 newborns. Most often it affects the small intestine, namely ileum . The first case of intestinal duplication was described by Calder in 1733 . Ladd was the first who systematized various classifications of disease and invented the term “alimentary canal duplication” .
Duplication of the intestine may be cystic or tubular. There are also localized forms of intestinal duplication associated with abnormalities of the vertebral column and spinal cord. Localized forms of duplication mostly occur in the area of jejunum and ileum . Typical signs of cystic duplication include intimate adhesion to intestine; smooth-muscle layer in the cyst wall; mucosal layer is histologically similar to one of the gastrointestinal tract segments . Blood supply can be common with the unaffected intestine or independent. Cystic duplications having connections with normal intestine either proximal or distal to the site of injury are the most common. Ununited duplication is quite rare .
According to one of the theories, the origin of intestinal duplication is explained by the violation of the digestive tract recanalization during solid phase of embryogenesis. Theory of primary notochords splitting suggests that stretching of neural tube leads to the intestine duplication and abnormalities of the vertebral column and spinal cord (hemi-vertebrae, spina bifida, bands between thoracic and cervical spinal cord) [5, 6].
Clinical manifestations of intestinal duplications vary. Patients may experience abdominal pain, vomiting, tumor formation or acute gastrointestinal bleeding. Duplication in the chest cavity simulates respiratory symptoms . In cases of the small intestine cystic duplication clinical signs depend on the type, size, location and characteristics of the mucosal lining of the cyst. Thus, small cystic duplication can act as an anchor in intussusceptions or volvulus, while large cystic duplications cause clinical picture of intestinal obstruction .
The diagnostic algorithm for suspected cystic intestinal duplication includes an X-ray examination with contrast of the intestine. Contrast passage can detect filling defect and, rarely, connection of cyst and intestine cavity. Ultrasound is quite important in the diagnosis of cystic duplication. The latter, in its turn, is visualized as echo positive inner edge represented by the mucous membrane and outer hypoechoic rim of smooth muscle layer. This sign is called as “symptom of muscular embankment”. On computed tomography cystic duplications manifest as round, fluid-filled cysts with a smooth membrane [1, 7].
Treatment of cystic intestinal duplication is surgical. The optimum amount of the operation is excision of cystic duplication with adhered segment of intestine after ligation of vessels that provide blood supply to these structures. Only cyst removal is not radical, because of the common intestinal wall and, often, the blood supply .
The newborn F, a boy, was born on 02.03.14 in obstetric clinics of SE “IPOG NAMS Ukraine” from the III pregnancy, II childbearing, by c-section, in term of 39-40 weeks. Weight at birth constituted 3,640 g, Apgar score – 5 points on the first and fifth minutes of life, body length – 53 cm, head circumference – 36 cm, chest circumference – 36 cm.
Low intestinal obstruction was diagnosed on prenatal ultrasound in fetal medicine department of SI “IPOG NAMS” on the 25th week of pregnancy when echo negative formation was detected in the lower abdomen with size: 28x16 mm 11x8, 6x8 mm moderate increase in amniotic fluid. In prenatal ultrasound follow-up on the 38th week of pregnancy dilated bowel loops in the lower abdomen were detected with the size of 66x54 mm, 47x42 mm, 29x23 mm, and significant increase in amniotic fluid.
Child was born in the presence of pediatric surgeon. In the delivery the enlarged abdomen with distended bowel loops were noted. In the process of gastric intubation 10 ml of transparent content was obtained, anus was with a through-passage, meconium was on the tip of the rectal tube. For further assessment and treatment the newborn after initial resuscitation was transferred to the pediatric ICU in transport incubator.
On admission to the intensive care unit the child’s condition was difficult. Breathing was voluntary, abdomen was enlarged in size, symmetrical, did not participate in breathing, with visible peristalsis, soft, tender, with palpable bowel loops. Meconium passed only after the enema. Due to severe bowel distention influencing the effectiveness of voluntary breathing, the child was transferred to the artificial pulmonary ventilation.
Under the conditions of intensive care unit the child was examined: plain radiograph of the abdomen showed reduction in pneumatization of intestinal loops. Ultrasound showed distended non-peristaltic intestinal loops, and in the lower abdomen weak peristalsis and more dense intestinal contents were detected. I stage bilateral hydronephrosis was also revealed. Neurosonography detected signs of I stage ventricle ditations. Patent ductus arteriosus and patent foramen ovale were noted using the echocardiography.
The child underwent preoperative preparation during one day. It included normal saline infusion, transfusion of fresh frozen plasma, solutions of amino acids, vitamin K, antibiotics, and the great saphenous vein catheterization on the thigh.
On the second day of life surgical treatment was performed: laparotomy, resection of ileum with duplication, creating ileo-ileoanastomosis, end to end. Features of operation: in the proximal segment of the ileum and within 30 cm, there was intimate bowel adhesions with tense cystic formation sized 30 x 6 x 4 cm (Fig. 1), the latter was aspirated with needle and 500,0 ml of light yellow discharge was received. The ileum was spread on the cyst in the middle third of it with common mesenterium. Ileum vitality in this region was debatable. Cyst was mobilized with adhered bowel. The distal ileum portion was thin. Distal resection extended to 15 cm of colon after ileocecal angle. A single-row ileo-ileoanastomsis end to end was formed.
The postoperative course was without surgical complications. Patient underwent intense infusion and antibiotic therapy, parenteral nutrition, transfusion of fresh frozen plasma, syndromatic treatment. First voluntrary bowel movement happened 3 days after the surgery. Enteric nutrition was started on the postoperative day 8. Gradually, the volume per single feeding increased to 80.0 ml with the transition to breastfeeding. The child was discharged on the 23rd day of life in good condition, weighing 3,800 g.
Thus, such clinical case is rare and therefore has great academic and practical value for pediatric surgeons, emergency physicians, neonatologists and other specialists. Prenatal diagnosis has created optimal conditions for the delivery and resuscitation in the first minutes of life. Conducted treatment of this severe malformation has been proved effective.