PRENATAL DIAGNOSIS
Prenat Diagn 2004; 24: 312–314. Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/pd.864
SHORT COMMUNICATION
Complete large bowel duplication with paraduodenal cyst: prenatal sonographic features Paritosh C. Khanna*, Vinit Gawand, Ajita J. Nawale, Tejaswini Deshmukh and Suleman A. Merchant Department of Radiology and Imaging, LTM General Hospital and LTM Medical College, Sion, Mumbai, India Objectives To illustrate diagnostic dilemmas while considering a diagnosis of complete large bowel duplication and duodenal duplication cysts. Methods A case of large bowel dilatation with a peripancreatic cyst was identified sonographically in a 27-year-old gravida 3 at 35 weeks of gestation, suffering from gestational diabetes and pregnancy-induced hypertension. Results On the basis of sonographic findings of dilated, hyperperistaltic large bowel loops, and a large cyst with echo-free contents near the pancreas, a diagnosis of large bowel obstruction with a paraduodenal cyst was considered. The findings were confirmed after postnatal ultrasonography. At the time of surgery, it proved to be a completely duplicated large bowel with a paraduodenal cyst. Conclusion Prenatal findings of a hugely dilated, hyperperistaltic large bowel should lead one to suspect/consider large bowel duplication. Associated cysts are likely to be bowel duplication cysts. Timely intervention can thus obviate potentially serious complications. Copyright 2004 John Wiley & Sons, Ltd. KEY WORDS:
colonic duplication; duodenal duplication; cyst; ultrasonography
INTRODUCTION Colonic duplication is one of the unusual reasons for large bowel dilatation and an apparent large bowel ‘obstruction’ at sonography. It is therefore necessary to have a high index of suspicion for this diagnosis, although rare.
CASE REPORT Our patient, aged 27 years had a bad obstetric history (gravida 3, aborted 2) with one abortion at 16 weeks and the second at 8 weeks of gestation. In the current pregnancy, she was diagnosed with gestational diabetes and pregnancy-induced hypertension (PIH) both controlled by dietary modifications alone. The first routine prenatal ultrasonography that the patient underwent at 35 weeks depicted an abdomen filled with large, hyperperistaltic, dilated bowel loops with haustrae and a large ahaustral cyst near the pancreas (Figure 1). A subsequent focused anomaly scan did not reveal any associated fetal malformations. An obstetric Doppler scan at 38 weeks of gestation was normal. A 2.5 kg baby, with an Apgar score of 8, was delivered at 40 weeks of gestation, by an emergency lower segment cesarean section in view *Correspondence to: Paritosh C. Khanna, 1211 B Everest Apartments, JPN Road, 7 Bungalows, Mumbai 400 061, Maharashtra, India. E-mail:
[email protected]
Copyright 2004 John Wiley & Sons, Ltd.
of nonprogression of labor and a bad obstetric history. The baby had gross abdominal distension with visible peristalsis, and the passing of meconium was delayed. A follow-up ultrasonographic examination performed at this time confirmed our prenatal findings (Figure 2). At the time of surgery, a completely duplicated large bowel was found along the antemesenteric border of the compressed normal large bowel and had a mesentery of its own. When traced, it was found to have no communication with the normal large bowel, resulting in closed-loop obstruction. As this segment had a separate vascular supply, it could be completely excised (Figure 3). The cyst, which was found to be in close proximity to the duodenum and pancreas, was avascular, and was removed as well (Figure 3). It was found to contain approximately 30 cc of mucinous material. No other associated fetal anomalies were noted intraoperatively. The baby was passing stool normally by the second postoperative day.
DISCUSSION Duplication cysts or enterogenous cysts are rare congenital anomalies and may be found in any part of the gastrointestinal tract (Bhargava et al., 1976). The different locations and sizes of these duplications require a specific diagnostic and surgical approach (Schalamon et al., 2000). Enteric duplications are most commonly jejunoileal, the incidence at this site being 53%, with only 13% of cases occurring in the colon and 6% in Received: 2 December 2003 Revised: 22 January 2004 Accepted: 31 January 2004
PRENATAL SONOGRAPHIC DIAGNOSIS OF BOWEL DUPLICATION
Figure 1—Prenatal ultrasonography at 35 weeks’ gestation depicting a large dilated bowel loop measuring 2.3 cm in diameter with haustrae (lower arrow), and an ahaustral cystic lesion representing the paraduodenal cyst (upper arrow)
Figure 2—Postnatal follow-up ultrasonography reveals a hugely dilated colon (white arrows) and a well-circumscribed cyst (black arrow)
Figure 3—Surgical specimen showing the dilated duplicated colon (left) and the avascular cystic lesion (right); the latter was paraduodenal in location Copyright 2004 John Wiley & Sons, Ltd.
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the duodenum (Heis). Ours was a case of combined complete colonic duplication and a duodenal duplication cyst. The cause of duplication is unknown, but various theories have been postulated (Heis, 1997; Behrman et al., 2000). Normally, there is intense epithelial proliferation in the bowel, between five and eight weeks of gestation, followed by recanalization by progressively coalescing vacuoles, which form the definitive lumen. Errors of epithelial recanalization resulting especially in the foregut may form intramurally located cysts, which give rise to small submucosal duplications. Partial twinning results in hindgut (distal ileal and colonic) duplications in association with a split in the primitive streak, resulting in caudally separated, duplicated notochords that later fuse during cranial elongation of the embryo. These may be associated with bladder and urethral duplications. ‘Early’ twinning may result in duplication of genital and other pelvic structures, whereas ‘late’ twinning generally results in only a colorectal duplication. Median septum formation implies the adherence and fusion of adjacent bowel walls, resulting in luminal duplication and explaining the occurrence of side-by-side tubular duplications. Embryonic diverticula, a common entity in the developing gut, support the occurrence of small cystic duplications around the circumference of bowel wall and enteric cysts in the presacral space. Finally, the split notochord theory postulates a neural tube traction mechanism wherein an embryologic error prior to mesoderm formation may permit a segment of GI endoderm to herniate through the notochord forming an entrapped diverticulum and resulting in a concomitant persistent vertebral anomaly. The resulting tubular diverticulum may separate and form an intramesenteric abdominal duplication cyst (Heis; Jimenez et al., 1999; Goyert et al., 1991). Colonic duplications most frequently present with obstruction of the adjacent bowel by the dilated duplication—as was the case with our patient and with the report by Jimenez et al. 1999—or complications from the obstruction (Heis). Bowel compression in utero may lead to neonatal microcolon (Leonidas, 1989). Colonic duplications may be associated with duplications of the genitourinary system (Heis; Jimenez et al., 1999). Those resulting from partial twinning may have other paired midline structures such as paired bladder and uteri, duplicated external genitalia and renal anomalies, and wide pelvis with abdominal wall herniae. The caudal pelvic cavity may also be divided into halves by an antero-posterior peritoneal septum (Heis). Duplication of the intestine may also occur in association with vertebral and spinal cord anomalies (hemivertebra, anterior meningomyelocele, duplication of an otherwise normal cord and a band connection between the lesion and cervical or thoracic spine) and is thought to arise from splitting of the notochord in the developing embryo (Heis; Behrman et al.). Vertebral anomalies and respiratory complaints from transdiaphragmatic herniation of the duplicated segment are rare with duodenal duplications (Heis). In the case by Richards et al., a duplicated gastric antrum was found to be associated with an extralobar pulmonary sequestration (Richards et al., 1996). Prenat Diagn 2004; 24: 312–314.
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In patients with colonic or presacral duplications who remain undiagnosed into adulthood, malignant changes resulting in adenocarcinoma can occur, making it imperative to diagnose even small colonic duplications. Constipation, obstruction, and volvulus are the common presenting symptoms. The incidence of neoplastic change in hindgut duplications is much higher than in any other location (Heis). Unusual sites of alimentary tract duplications detected by prenatal ultrasound include the cecum (Haratz-Rubinstein and Sherer, 2003) and the retroperitoneum (Duncan et al., 1992). Morphologically, duodenal duplications may be either tubular or cystic, although most are cystic (Heis). These are usually lined with duodenal mucosa, but if lined with gastric mucosa, peptic ulceration and hemorrhage can occur in addition to bowel obstruction (Heis). In our patient, the paraduodenal cyst, on histology, proved to be lined by duodenal mucosa, thus proving its duodenal origin. In retrospect, we concluded that the viscid mucus recovered from the cyst was a product of the mucus-secreting Brunner’s glands normally found in the duodenum. In addition to a paraduodenal location, these cysts may also be found in an immediate subhepatic location (Yamataka and Pringle, 1998). In these cases, on prenatal ultrasonography, a differential of choledochal cyst, bowel duplication cyst, or omental cyst should be considered (Yamataka and Pringle, 1998). Prenatal diagnosis by sonographic screening of uncomplicated intestinal duplications will prompt early surgical management before the onset of complications (Goyert et al., 1991; Kangarloo et al., 1979; Bidwell and Nelson, 1986; Degani et al., 1995). On ultrasonography, uncomplicated bowel duplication may appear similar to fluid-filled bowel (Kangarloo et al., 1979) as in our case. An echogenic inner rim is highly suggestive of the diagnosis (Kangarloo et al., 1979; Bidwell and Nelson, 1986) of duplication. Duodenal duplication cysts present with an ultrasonographic spectrum ranging from a sonolucent cyst to an echogenic mass (Kangarloo et al., 1979). The ‘double bubble’ sign in prenatal diagnosis is most often associated with duodenal atresia. However, other cystic abdominal masses such as duodenal duplications can also give rise to this sonographic appearance (Malone et al., 1997). Resection of the duplication is the most effective method of treatment (Bhargava et al., 1976) as the blood supply to these duplicated colons is separate (Heis). During surgery, the rare occurrence of a common blood supply between the duplication and the native bowel must be carefully identified and protected to avoid undue sacrifice of normal bowel (Pinter et al., 1992). In one case, the duplication was found incidentally during an operation for an anorectal malformation and was treated by laparoscopic-assisted resection (Schalamon et al., 2000). Resection of the duplication with or without minimal resection of the adjacent normal
Copyright 2004 John Wiley & Sons, Ltd.
intestine should be mandatory (Schalamon et al., 2000). Balen et al. have described a 70-cm long jejunoileal duplication that was successfully dissected free from the normal small bowel and excised without intestinal resection-anastomosis (Balen et al., 1993). It should be kept in mind that presence of peristaltic activity within a cyst points to a duplication of gastrointestinal origin (Richards et al., 1996; Bidwell and Nelson, 1986). To conclude, the prenatal detection of bowel duplication, cystic or otherwise, allows close neonatal surveillance and timely surgical intervention prior to the onset of potentially serious complications.
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