Sonographic findings in a fetus with megacystis-microcolon–intestinal hypoperistalsis syndrome

Chen, Chih‐Ping; Wang, Tao‐Yeuan; Chuang, Chun‐Yu

doi:10.1002/(sici)1097-0096(199805)26:4<217::aid-jcu8>3.0.co;2-g

Cited by 32 publications

(10 citation statements)

References 17 publications

(19 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our patient's heterogeneous presentation strengthens a previous argument of overlap between MMIH and PBS syndromes [10,20,21]. Moreover, we believe that there is a common developmental pathologic pathway at the base of PBS, MMIH, and OEIS.…”

Section: Discussionsupporting

confidence: 84%

Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant

Giuliani

Vendryes

Malhotra

et al. 2010

Journal of Pediatric Surgery

View full text Add to dashboard Cite

Section: Discussionsupporting

confidence: 84%

Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant

Giuliani

Vendryes

Malhotra

et al. 2010

Journal of Pediatric Surgery

View full text Add to dashboard Cite

“…The knowledge of the length of the remaining intestine may be essential for appropriate planning of postoperative management, for more accurately defining the short-gut syndrome, and for prognosis [1,6]. Moreover, assessment of fetal gut length according to reliable reference standards is necessary for the correct interpretation of ultrasonographic findings in syndromes associated with gastrointestinal abnormalities and short gut, such as congenital short bowel syndrome [7] and campomelic dysplasia Cumming-type [8] and microcolon megacystis syndrome [9]. In laboratory practice, reference ranges are usually derived from a reasonably large number of specimens, donated by healthy or presumably healthy subjects; this is virtually impossible when determining reference ranges for fetal or neonatal organ weights [4] or linear measurements.…”

Section: Discussionmentioning

confidence: 99%

“…Measurement of fetal intestinal length might also be of importance in the context of the fetal gastrointestinal abnormalities associated with short gut, such as congenital short bowel syndrome [7] and campomelia Cumming-type [8] or microcolon megacystis syndrome [9].…”

Section: Introductionmentioning

confidence: 99%

Development of Fetal Intestinal Length during 2nd-Trimester in Normal and Pathologic Pregnancies

et al. 2012

View full text Add to dashboard Cite

Linear growth of the human fetal gastrointestinal tract is not often discussed in the literature, and little is known about the effects of chromosomal abnormalities and intrauterine growth restriction (IUGR) on intestinal length, especially during the 2nd trimester. Accurate evaluation of intestinal length and knowledge of normal and reference values are of clinical importance. For example, intestinal resection may be necessary in preterm infants with necrotizing enterocolitis or mid-gut volvulus, and the surgeon should use data to be judicious in the amount removed. Linear measurements are essential in evaluating fetal development ultrasonographically and are an integral part of the postmortem examination. The intestinal lengths of 203 2nd-trimester fetuses and premature infants were measured. Small intestine length (SIL), colon length (CL), total bowel length (TBL; TBL = SIL + CL), and the length of the appendix (AL) increased with gestational age. No differences between the genders were observed. Colon length increased secondary to maceration, but no such effects were shown on SIL, TBL, or AL. No differences were shown in relation to IUGR. Small intestine length, CL, and TBL, but not AL, were shorter in fetuses with trisomy 21. Appendix length was not affected by any of the studied factors. We propose that the measurement of the length of the appendix may be used as an additional parameter for the postmortem evaluation of gestational age. Furthermore, its assessment may have potential as an ultrasonographic indicator of gestational age, particularly for the 2nd trimester.

show abstract

“…MMIHS is usually lethal during the first postnatal year, and can be suspected on the prenatal sonogram when a megabladder is demonstrated. Associated sonographic features may include dilated bowel loops8, enlarged stomach and hydronephrosis9. Low amniotic fluid digestive enzyme levels may support the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of intestinal pseudo‐obstruction

Shen

Schimmel

Eitan

et al. 2007

Ultrasound in Obstet & Gyne

View full text Add to dashboard Cite

CASE REPORTS Case 1A 28-year-old gravida 3, para 2 woman, was referred to our unit at 30 weeks' gestation with suspected fetal bowel obstruction. The patient and her husband were first cousins. Their medical histories were unremarkable and the woman had two healthy girls. Ultrasonography showed severe polyhydramnios with a deepest amniotic fluid pocket of 19 cm and multiple loops of dilated small bowel with visible peristalsis and no ascites or bowel-wall thickening. The scan was otherwise normal. Small bowel obstruction was suspected and the patient was counseled accordingly. She was offered and declined screening for cystic fibrosis. At 36 weeks' gestation she was admitted in labor, and a Cesarean section was performed on maternal request; the female neonate weighed 3145 g.The neonate had a severely distended abdomen with multiple dilated loops of small bowel on plain abdominal X-ray. An explorative laparotomy was performed with a preoperative diagnosis of small bowel obstruction. On laparotomy, multiple dilated loops of small bowel were observed with no mechanical obstruction. Appendectomy and rectal biopsy were performed. These were both normal, with normal ganglion cells on pathological examination. During the immediate postoperative period the patient remained obstructed, the urinary bladder was persistently enlarged and a neurogenic bladder was suspected. No other urinary tract abnormalities were observed on postnatal investigation. With a diagnosis of primary congenital intestinal pseudo-obstruction (CIPO) and possibly megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) the patient was put on total parenteral nutrition and gastrointestinal prokinetic medication. The obstruction gradually subsided, as did the bladder dysfunction. Within a month the infant was discharged requiring no medication and is currently 7 years old and in excellent health. Case 2A 31-year-old gravida 5, para 4 woman -sister to the first patient -was referred to our unit at 30 weeks' gestation with polyhydramnios. Like her sister, she was married to a first cousin. She had previously given birth to four healthy children. On admission the ultrasound scan showed moderate polyhydramnios, the deepest amniotic fluid pocket being 13 cm. A detailed anatomy sonogram performed at this time revealed a normal stomach bubble and multiple hypoechogenic dilated small bowel loops (Figure 1). The dilated loops had a maximal diameter of 18 mm and visible peristaltic waves were recorded. Additionally, right pyelectasis was observed

show abstract

Sonographic findings in a fetus with megacystis-microcolon–intestinal hypoperistalsis syndrome

Cited by 32 publications

References 17 publications

Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant

Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant

Development of Fetal Intestinal Length during 2nd-Trimester in Normal and Pathologic Pregnancies

Prenatal diagnosis of intestinal pseudo‐obstruction

Contact Info

Product

Resources

About