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Second Trimester Prenatal Diagnosis of Congenital Gastric Outlet Obstruction

Brigitte Bonin, MD, FRCSC, Andre Gruslin, MD, FRCSC, Nigel A.B. Simpson, MRCOG, Steven Z. Rubin, MD, FRCSC, Lawrence W. Oppenheimer, MB, FRCSC

Prenatal diagnosis of gastric outlet obstruction is made infrequently owing to variations in gastric dimension, and reports from the literature have been confined to diagnosis in the third trimester. We report a case of second trimester diagnosis of congenital pyloric atresia. Early diagnosis during pregnancy may be valuable in facilitating both prenatal and postnatal management. CASE REPORT
A 34 year old woman was referred in preterm labor at 24 weeks 2 days of gestation. Her first pregnancy had been complicated by idiopathic preterm labor, culminating in the delivery of a healthy 1.9 kg male infant at 30 weeks gestation. The present pregnancy had been uncomplicated, with confirmation of menstrual dates by ultrasonographic estimation at 16 weeks. The fetal anatomy at that time was noted to be normal. A genetic amniocentesis also was performed and revealed a normal male karyotype.

Received December 2, 1997, from Ottawa General Hospital (B.B, A.G., L.W.O.), Ottawa, Ontario, Canada; the Division of Obstetrics and Gynaecology (N.A.B.S.), St. Jamess University Hospital, Leeds, United Kingdom; and the Childrens Hospital of Eastern Ontario (S.Z.R.), Ottawa, Ontario, Canada. Revised manuscript accepted for publication February 20, 1998. Address correspondence and reprint requests to Brigitte Bonin, MD, FRCSC, Ottawa General Hospital, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6.

When she first was examined no clinically obvious hydramnios was noted. The cervix was 2 cm dilated and 50% effaced, with bulging membranes. A random blood glucose determination was normal and the maternal blood group was O negative with anti-Leb antibodies present. Ultrasonographic examination confirmed an increase in amniotic fluid volume, with the deepest vertical pocket measuring 98 mm. The fetal stomach was dilated (Fig. 1), with a measurement in the longitudinal axis of 33 mm, which was well above the normal range.1 No other abnormalities were seen and fetal growth was otherwise appropriate for gestational age. The patient was managed conservatively with tocolysis and bed rest. During the course of the next 3 weeks, serial measurements of the longitudinal axis of the stomach varied between 21 and 40 mm (Fig. 2). The combination of gastric dilation and polyhydramnios suggested the diagnosis of gastric outlet obstruction. We consulted with surgical and neonatal specialist colleagues as appropriate. Labor supervened at 27 weeks of gestation. The patient had a sudden spontaneous rupture of membranes, and the cervix was found to be fully dilated. A live male infant weighing 860 g was delivered vaginally as a breech birth. He was electively intubated and ventilated. A plain abdominal radiograph revealed a dilated stomach with no air distally (Fig. 3). Contrast studies from above and below confirmed that atresia was present only at the level of the stomach (Fig. 4). At laparotomy the day after delivery, no external abnormality of the duodenum or stomach was noted, but a nasogastric tube could not be passed beyond the pylorus. A longitudinal incision at the level of the pylorus revealed

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complete fusion of the anterior and posterior walls. A transverse pyloroplasty was performed. The baby made an uneventful recovery and remains well on follow-up studies.

DISCUSSION Pyloric atresia accounts for 1% of all gastrointestinal obstructions due to atresia.2 Its pathogenesis may entail incomplete recanalization of the foregut, as in duodenal atresia, or the defect may be secondary to a vascular accident in intrauterine life, as has been suggested in ileal and jejunal atresias.3 Its familial occurrence has suggested that it may be an inherited disorder, with a postulated autosomal recessive transmission.4 It has also been documented in association with epidermolysis bullosa, whereby replacement of damaged luminal mucosa by scar tissue causes pyloric outlet obstruction.4,5 Because pyloric atresia is usually accompanied by hydramnios, the antenatal presentation is commonly that of preterm labor. An increase in formation of amniotic fluid in the third trimester coupled with a decreased intestinal absorption accounts for the development of polyhydramnios. In a previous study by Barkin and coworkers6 that quantified the amount of amniotic fluid, 21% of cases of marked polyhydramnios were associated with primary gastrointestinal abnormalities. These authors also linked the timing of the appearance of polyhydramnios to the type of congenital anomaly and demonstrated that when it is secondary to a gastrointestinal obstruction, polyhydramnios typically appears in
Figure 1 Measurements of the fetal stomach at presentation (24 weeks gestation).

the third trimester. The diagnostic accuracy of sonography was high in detecting these abnormalities. Visualization of the fetal stomach on ultrasonograms has been documented as early as 9 weeks gestation, although its characteristic anatomy does not emerge until week 14.7 Nomograms for stomach measurements have been constructed by other investigators1,7 and in this case were helpful in establishing the diagnosis of gastric dilatation. Fetal stomach volume is determined by the interaction of at least four processes: swallowing, stomach secretion, stomach absorption, and stomach emptying.8 Changes in gastric dimensions over a period of time can be observed in normal pregnancies mainly because stomach filling and emptying are dynamic processes.9 In the normal stomach, measurements made 20 min apart differed by between 17% for the longitudinal and 25% for the transverse axes.8 These changes can be observed as early as 14 weeks of pregnancy.8 Swallowing activity has been observed as early as 16 weeks in the human fetus.10 It remains episodic and minimal, however, until 30 weeks of pregnancy.11 Pritchard found that the volume of amniotic fluid swallowed by a normal fetus increases with gestational age.10 Although rapid changes in stomach size may not occur in the presence of a pathologic condition, it is important to note that, in our case, the gastric measurements varied considerably from one ultrasonogram to the other and were not consistently in the abnormal range. In a similar case report, the stomach measurements, although variable, were above normal on every occasion.12 Early in pregnancy the volume of amniotic fluid swallowed by the fetus might be low enough to
Figure 2 Serial longitudinal measurements of the fetal stomach (nomogram adapted from Nagata and coworkers1).

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Figure 3 Absence of air distal to the stomach on postnatal radiograph.

Figure 4 Postnatal contrast study demonstrates gastric dilation with absence of contrast material distally, confirming the existence of obstruction.

allow absorption by the fetal gut. Later in pregnancy, the volume swallowed increases and, in the presence of an obstruction, seems to exceed the resorptive capacity of the stomach, resulting in its dilation.13 This is thought to occur as early as 29 weeks. Because of all the different processes influencing stomach size and the small contribution of swallowing in early pregnancy, it is therefore not surprising that the stomach appeared normal on some second trimester scans in our case even in the presence of an obstruction. This emphasizes the need for repeated measurements in cases in which gastrointestinal obstruction is a consideration. Other documented gastric abnormalities that have been manifested as dilation and hydramnios include preantral web,14 pyloric stenosis,15 and nonhypertrophic pyloric stenosis associated with intestinal malrotation.16 In one additional case, isolated gastric dilation was the only abnormal finding to accompany a postnatal diagnosis of pyloric stenosis.17 Gastric dilation also may be seen in association with other gastrointestinal defects, such as duodenal and proximal jejunal atresias, 50% of which are associated with hydramnios. It is, however, rarely seen in the presence of obstruction of the distal ileum and colon.2

Prenatal diagnosis is valuable because it allows appropriate counseling for the parents, and obstetric and neonatal interventions may be planned. In other reported cases, expectant management alone was effective in deferring delivery,12 although tocolysis and therapeutic amniocentesis may be helpful.18,19 At delivery, decompression of the stomach is necessary, and elective intubation may be useful. Intravenous feeding and correction of electrolyte disturbances may be required. A plain abdominal radiograph will help to confirm the diagnosis, although appropriate contrast studies will be necessary in determining the exact site of the obstruction and in detecting the presence of distal atresia. A transverse pyloroplasty or gastroduodenostomy is the traditional procedure at laparotomy, but endoscopic methods are now emerging. The prognosis is excellent, although gastroesophageal reflux is common in the short term.2 Genetic counseling is appropriate if a familial incidence is present. This case illustrates the potential for early diagnosis of gastric outlet obstruction and underlines the importance of documenting gastric dimensions, particularly in association with polyhydramnios. Early prenatal diagnosis is valuable in assessing the need

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for therapeutic amniocentesis and observing for signs of preterm labor as well as for planning appropriate interventions. This case also illustrates, however, the difficulties in diagnosing fetal gastrointestinal tract obstructions prior to the third trimester. REFERENCES
1. Nagata S, Koyanagi T, Horimoto N, et al: Chronological development of the fetal stomach assessed using realtime ultrasound. Early Hum Dev 22:15, 1990 Dudgeon DL: Lesions of the stomach. In Ashcraft KW, Holder TM (Eds): Pediatric Surgery. 2nd Ed. Philadelphia, WB Saunders, 1993, p 295 Bronsther B, Nadeau MR, Abrams MW: Congenital pyloric atresia: A report of three cases and a review of the literature. Surgery 69:130, 1971 Adashi EY, Louis FJ, Vasquez M: An unusual case of epidermolysis bullosa hereditaria letalis with cutaneous scarring and pyloric atresia. J Pediatr 96:443, 1980 Orense M, Garcia-Hernndez JB, Celorio C, et al: Pyloric atresia associated with epidermolysis bullosa. Pediatr Radiol 17:435, 1987 Barkin SZ, Pretorius DH, Beckett MK, et al: Severe polyhydramnios: Incidence of anomalies. AJR 148:155, 1987 Goldstein I, Reece EA, Yarkoni S, et al: Growth of the fetal stomach in normal pregnancies. Obstet Gynecol 70:641, 1987 Zimmer EZ, Chao CR, Abramovich G, et al: Fetal stomach measurements: Not reproducible by the same observer. J Ultrasound Med 11:663, 1992

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Devane S, Soothill P, Candy D: Temporal changes in gastric volume in the human fetus in late pregnancy. Early Hum Dev 33:109, 1993

10. Pritchard JA: Fetal swallowing and amniotic fluid volume. Obstet Gynecol 28:606, 1966 11. McLain CR: Amniographic studies of the gastrointestinal motility of the human fetus. Am J Obstet Gynecol 86:1079, 1963 12. Hasegawa T, Kubota A, Imura K, et al: Prenatal diagnosis of congenital pyloric atresia. J Clin Ultrasound 21:278, 1993 13. Nelson LH, Clark CE, Fishburne JI, et al: Value of serial sonography in the utero detection of duodenal atresia. Obstet Gynecol 59:657, 1981 14. Zimmerman HB: Prenatal demonstration of gastric and duodenal obstruction by ultrasound. J Assoc Can Radiol 29:138, 1978 15. Jouppila P, Kirkinen P: Ultrasonic and clinical aspects in the diagnosis and prognosis of congenital gastrointestinal anomalies. Ultrasound Med Biol 10:465, 1984 16. Hershkovitz E, Steiner Z, Shinwell E, et al: Prenatal ultrasonic diagnosis of nonhypertrophic pyloric stenosis associated with intestinal malrotation. J Clin Ultrasound 22:52, 1994 17. Katz S, Basel D, Branski D: Prenatal gastric dilatation and infantile hypertrophic pyloric stenosis. J Pediatr Surg 23:1021, 1988 18. Peled Y, Hod M, Friedman S, et al: Prenatal diagnosis of familial congenital pyloric atresia. Prenat Diagn 12:151, 1992 19. Sharony R, Sinow R, Asch M, et al: Prenatal ultrasound diagnosis of gastric outlet obstruction due to a pyloric web. Prenat Diagn 15:56, 1995

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