Fetal Cyst Reveling Retroperitoneal Enteric Duplication.

Retroperitoneum is a very uncommon site of enteric duplication (ED). We report a new case of retroperitoneal ED cyst suspected in utero. Prenatal ultrasound showed an abdominal cystic mass. Noncommunicating retroperitoneal ED cyst measuring 70 mm × 30 mm was resected. Histopathologic examination confirmed the diagnosis.

INTRODUCTION

Enteric duplications (EDs) are uncommon anomalies that can occur at any point of the gastrointestinal tract. Their reported incidence is about 1 in 4500 live births.[1] The small intestine is the most common location; retroperitoneum is an extremely rare site. In general, diagnosed in the neonatal period or during infancy, they are increasingly diagnosed prenatally; early prenatal detection is possible. We report a new case of retroperitoneal ED cyst prenatally suspected. To our knowledge, there have been seven reported cases of retroperitoneal ED cyst in the English literature.

CASE REPORT

A female newborn, vaginally born at 39 weeks of gestation from a 32-year-old mother, gravid 3, para 3. Pregnancy was uneventful. Prenatal ultrasound at 22 weeks of gestation objectified an abdominal cystic mass located in the left upper abdominal quadrant, associated with fetal pyelectasis. Ultrasound follow-up showed the persistence of the cyst.

At birth, the newborn was asymptomatic. Birth weight was 4000 g, length was 51 cm, and head circumference was 35 cm. She passed meconium on day 1 of life. She was totally enterally fed, without incident. Postnatal ultrasound found a retroperitoneal para-aortic liquid-filled mass measuring 60 mm × 33 mm × 22 mm. Magnetic resonance imaging (MRI) confirmed the presence of a retroperitoneal cyst occupying the upper left retroperitoneal space; with mass effect displacing the left kidney down [Figure 1]. No associated congenital abnormality was found. The newborn had exploratory laparotomy at 3 months of life. Peroperative finding was a retroperitoneal cyst above the left adrenal, displacing the left kidney down, measuring 70 mm × 30 mm; with no communication with any portion of the alimentary tract. Histopathologic examination revealed an ED cyst lined by small intestinal epithelium, with no ectopic gastric mucosa. The postoperative period was uneventful; the patient was discharged on the 5th postoperative day. Postoperative ultrasound showed regression of transient pyelectasis.

Figure 1

Magnetic resonance imaging showing retroperitoneal cyst occupying the upper left retroperitoneal space; with mass effect displacing the left kidney

DISCUSSION

EDs represent a group of uncommon congenital anomalies. Intraabdominal duplications account for two-thirds of localization, among them, jejunoileal duplications account for 65%. EDs occur early in intrauterine life; their pathogenesis remains unclear; several hypotheses have been proposed.[2] Retroperitoneal duplications are extremely rare with few case reports in English literature. Jehangir et al. reported 38 cases of ED cysts; only one was retroperitoneal communicating with a nonfunctioning right kidney.[3]

EDs are a part of fetal intraabdominal cysts which evoke several differential diagnoses including ovarian cysts, renal cysts, choledochal cysts, hepatic cysts, and mesenteric cysts.[4] Accurate early prenatal diagnosis of ED is possible.[5] Marchitelli et al. found high concordance between prenatal and postnatal findings in fetal intra-abdominal cystic lesions (90.4%).[6] Specific signs could be detected in utero. The “double-wall” sign and peristaltism are suggestive of ED and help to differentiate it of differential diagnosis.[7] High incidence of associated anomalies to EDs was reported.[8] Jehangir et al. reported that vertebral and spinal anomalies were the most common.[3] Associated anomalies determine prognosis of EDs. Hence, when ED is suspected, associated anomalies should be checked.

Postnatal ultrasound helps diagnosis showing an anechoic fluid-filled mass, rarely an echogenic mass is found due to complication (hemorrhage, necrosis). Computed tomography and MRI allow specifying the type and the size of the anomaly. Clinical presentation of jejunoileal duplications includes hemorrhage, obstruction, and intussusceptions. The patient could be totally asymptomatic.[2] More serious complications may occur: Ulceration, perforation, severe hemorrhage, and malignant changes associated with the presence of ectopic gastric mucosa. Thus, once an ED is diagnosed, it should be resected. Surgical intervention is generally performed at the age of diagnosis. Elective surgery is proposed to asymptomatic patients prenatally diagnosed to ovoid potential complications.[9] There is no consensus about the surgical approach of EDs in infants. Bothe laparoscopic and open resections are possible. Laparoscopic approach offers a low rate of complications.[10]

FINANCIAL SUPPORT AND SPONSORSHIP

Nil.

CONFLICTS OF INTEREST

There are no conflicts of interest.