消化道畸形伴发多发性或消化道其他畸形

目的 了解消化道畸形伴发多发性畸形或消化道其他畸形的发病情况。 方法 对我院近30余年来(60年代～90年代)手术记录完整的376例消化道畸形病例,其中肠闭锁168例,肠狭窄79例,肠旋转不良109例及环状胰腺20例,进行回顾性研究其伴发多发性畸形及消化道其他畸形所占比例。结果168例肠闭锁中多发性肠闭锁24例(14.3％)。伴发消化道其他畸形者57例(33.9％),其中胎粪性腹膜炎42例(25.0％),且其发病与年份有密切关系,在1985年以前的92例中伴胎粪性腹膜炎者34例占37％,而1996年以后的62例中仅有8例,占12.9％,其伴发率明显减少(P<0.005)。肠狭窄79例,多发性肠狭窄2例(2.5％),消化道其他畸形者14例(17.7％)。肠旋转不良109例中伴消化道其他畸形者21例,占19.3％,以十二指肠或空肠闭锁或狭窄最多,共13例,占伴发畸形的62％。在20例环状胰腺中伴发消化道其他畸形者4例,占20.0％。 结论 消化道畸形呈多发畸形或伴发消化道其他畸形的发病率很高,故术中应仔细检查以免遗漏。     

Contrast enema depiction of small-bowel volvulus in complicated neonatal bowel obstruction

About one-half of patients with meconium ileus (MI) present with a complication such as volvulus, atresia, meconium peritonitis or giant cystic meconium peritonitis. The treatment of these complications requires surgery. However, the preoperative diagnosis of complicated MI is difficult. We describe two neonates with complicated small-bowel obstruction, one with MI related to cystic fibrosis and the other not related to cystic fibrosis. In both, contrast enema depicted a spiral appearance of the distal small bowel, which at surgery proved to be the result of volvulus associated with antenatal bowel perforation. This appearance of the small bowel on contrast enema in this clinical setting has not been previously described. The recognition of this spiral appearance of the distal small bowel suggests the need for surgery.This paper was presented at the 41st Congress of the European Society of Paediatric Radiology, Heidelberg, Germany, June 2004     

The impact of cystic fibrosis on neonatal intestinal obstruction: the need for prenatal/neonatal screening

To determine the incidence of cystic fibrosis (CF) in neonates with intestinal obstruction (NIO) secondary to meconium ileus (MI), jejunoileal atresia (JA), meconium plug syndrome (MPS), volvulus (V), and meconium peritonitis (MP) and analyze the correlation of ultrasonographic (US) signs with CF in NIO with a prenatal diagnosis of intestinal anomaly, a prospective analysis of different types of NIO from 1990 to 1998 was undertaken. Immunoreactive trypsin measurement, genetic studies, and sweat tests were performed to confirm or rule out CF. Cases with prenatal diagnosis were analyzed for gestational age, dilated bowel, ascites, hyperechoic bowel, and calcifications. Of 80 neonates, 19 (24%) had CF: 2/33 (6%) JA, 6/14 (43%) MPS, 1/14 (7.1%) MP, 10/10 (100%) MI, and 0/9 V. Thirty (37.5%) had a prenatal diagnosis of an intestinal anomaly. The overall incidence of CF in NIO with a prenatal diagnosis of intestinal anomaly was 4/30 (13%), or 333 times the estimated risk of CF in the general population. A hyperechoic pattern with dilated bowel was associated with higher specificity for CF: 3/3 cases (100%), followed by hyperechoic bowel with ascites: 3/4 cases (75%). All babies with any type of NIO should thus be screened for CF. Prenatal screening for CF should be indicated in all pregnancies with US patterns of specific intestinal disorders.     

Idiopathic origin of meconium peritonitis

A 26-year-old, gravida 3 presented at 31 weeks of gestation with polyhydramnios. On ultrasound there was marked foetal ascitis with unilateral hydrocele. Patient delivered a 3.15 kg, large-for-date baby at 33 weeks and 3 days of gestation. On basis of clinical, radiological and sonographic features, diagnosis of meconium peritonitis was made. Ascitic tapping was done. Surgery was with held, as there were no signs of intestinal obstruction. DNA testing for cystic fibrosis was negative. Baby did not deteriorate so he was discharged. Baby was doing well on 2 months follow up. Hydrocele and ascitis were resolving. Rarely meconium peritonitis may occur without an underlying cause when peritonitis may be innocuous and intervention may not be required.     

Prenatal diagnosis of the fetus with cystic fibrosis and meconium ileus

The sonographic finding of hyperechoic or dilated fetal bowel raises suspicion of a number of prenatal disorders including meconium ileus (MI), meconium peritonitis, congenital infection, neoplasm, or chromosomal trisomy. These findings may also represent transient normal variants. The following case report details the evaluation of one pregnancy with abnormal intestinal echogenic findings on serial sonograms (US), to demonstrate inherent diagnostic difficulties in such a case. A diagnostic algorithm is presented to aid in the proper use of US and DNA mutation analysis for cystic fibrosis (CF), so that the cause of an abnormal abdominal US can be established earlier and more accurately than suggested by previous management schemes. Earlier fetal diagnosis may help to anticipate postnatal problems associated with CF/MI, and therefore provide more optimal clinical management of the affected fetus.     

Meconium peritonitis in stillbirths

Meconium peritonitis is a sterile, chemical peritonitis resulting from perforation of the bowel in perinatal life. In stillbirths meconium peritonitis is extremely rare. We report the autopsy findings in three fetuses ranging from 21-39 weeks gestation in which meconium peritonitis was identified. Maternal history in two cases was suggestive of possible fetal hypoxia. No family history of cystic fibrosis was discerned. One fetus was hydropic and abdominal calcifications were noted on postmortem radiograph. Gross evidence of meconium in the peritoneal cavity, visceral adhesions, and serosal nodules were noted in two fetuses. Nodules of calcified meconium seen by microscopy were the only clues to diagnosis in the third fetus. The discovery of meconium peritonitis at autopsy may be the only residual evidence of antecedent bowel perforation. We suggest that intrauterine hypoxia may play a role in the development of meconium peritonitis in some cases.     

Meconium Peritonitis in Stillbirths

Meconium peritonitis is a sterile, chemical peritonitis resulting from perforation of the bowel in perinatal life. In stillbirths meconium peritonitis is extremely rare. We report the autopsy findings in three fetuses ranging from 21-39 weeks gestation in which meconium peritonitis was identified. Maternal history in two cases was suggestive of possible fetal hypoxia. No family history of cystic fibrosis was discerned. One fetus was hydropic and abdominal calcifications were noted on postmortem radiograph. Gross evidence of meconium in the peritoneal cavity, visceral adhesions, and serosal nodules were noted in two fetuses. Nodules of calcified meconium seen by microscopy were the only clues to diagnosis in the third fetus. The discovery of meconium peritonitis at autopsy may be the only residual evidence of antecedent bowel perforation. We suggest that intrauterine hypoxia may play a role in the development of meconium peritonitis in some cases.     

Meconium peritonitis,intraperitoneal calcifications and cystic fibrosis

Twelve cases of meconium peritonitis were retrospectively reviewed in this study. Five of these patients had cystic fibrosis. Of the patients with cystic fibrosis, intraperitoneal calcifications were present in three (60%) and two (40%) had none. All seven patients (100%) who had abconium peritonitis without cystic fibrosis had abdominal calcifications. There results indicate that the presence of intraperitoneal calcification does not exclude the diagnosis of cystic fibrosis and that the absence of calcification favors cystic fibrosis. However, a review of the literature indicates the absence of calcification is merely of clue and not definitive as to the etiology of the meconium peritonitis.     

Fetal meconium peritonitis without sequelae

Meconium peritonitis is a chemical peritonitis usually resulting from antenatal bowel rupture. Prenatal ultrasound findings include ascites, intraabdominal masses, bowel dilatation and the development of intraabdominal calcifications [1–5]. The most common bowel disorders which lead to meconium peritonitis in utero are those resulting in bowel obstruction and perforation, such as small bowel atresias, volvulus and meconium ileus [1–5]. Meconium ileus is associated with cystic fibrosis in most cases, although extraluminal abdominal calcifications are usually scarce in cases of cystic fibrosis [1, 6]. Postnatal outcome for infants with meconium peritonitis depends on the etiology for bowel rupture and underlying disease.     

An unusual case of meconium peritonitis associated with perforated hydrocolpos

Meconium peritonitis results from intestinal wall perforation with meconium spillage into the peritoneum during intrauterine life. Anal atresia is associated with meconium peritonitis and is also associated with cloacal malformation. A female neonate presented with a distended abdomen, anal atresia, and meconium peritonitis as diagnosed by calcification in the peritoneal cavity on abdominal radiograph. Sonography and cystography demonstrated a cloacal malformation with perforation of the hydrocolpos and resultant spillage of meconium into the peritoneum. There was no evidence of further perforation of bowel at surgery. This unusual case of meconium peritonitis is reported.     

Gastroschisis: Are prenatal ultrasonographic findings useful for assessing the prognosis?

Objective. The objective of this study was to assess various prenatal patterns in correlation with survival and the occurrence of complications of antenatally recognized gastroschisis (G).Materials and methods. We retrospectively studied 34 cases of G. Mortality and morbidity in the postnatal period were assessed and correlated with the prenatal presence or absence of: (1) bowel and/or stomach dilatation, (2) thickening and/or hyperechogenicity of the intestinal wall, (3) meconium peritonitis (in the abdomen) before 20 weeks of gestation, (4) asymmetrical bowel dilatation, and (5) associated malformations. Morbidity took into account the length of hospitalization and the number of surgical procedures.Results. The overall survival rate was 94 %. Neither bowel nor stomach dilatation was significantly correlated with mortality. However, evidence of intestinal dilatation greater than 17 mm had a positive predictive value of 67 % for atresia, with a negative predictive value of 86 %. Thickening and/or hyperechogenicity of the bowel wall were not significantly associated with mortality. Meconium peritonitis before 20 weeks and asymmetrical bowel dilatation were not statistically significant because of the small sample size. Twelve patients (35.3 %) had postnatal complications, with a mean hospital stay of 127 days. Outcome was not modified by the mode of delivery. Associated extradigestive anomalies were present in 20.6 % of cases. Chromosomal anomalies were not seen.Conclusion. The prognosis of prenatally detected G is excellent despite the frequency of small bowel atresia (67 %) in the group with postnatal complications. Meconium peritonitis before 20 weeks of gestation and/ or asymmetrical bowel dilatation also appear to be indicators of atresia (2/4, 50 %) or high morbidity (3/4,75%).     

胎粪性腹膜炎23例临床分析

目的：　探讨胎粪性腹膜炎的病因、临床特点及诊断治疗。方法：　采用回顾性研究,通过对23例患者的临床分析,结合X线检查,来阐明其特点,并对其治疗进行分析。结果：　胎粪性腹膜炎的病因以肠闭锁多见,占30.4% (7/23);其临床特征主要为:腹腔内钙化,大量纤维组织粘连,伴或不伴有假性囊肿形成;手术治疗是有效的治疗方法。结论：　肠闭锁是胎粪性腹膜炎的重要原因;早期诊断,正确的手术方式,认真的术后护理是提高胎粪性腹膜炎存活率的关键     

Meconium peritonitis—A leading cause of neonatal peritonitis in Kashmir

Meconium peritonitis is a chemical reaction of peritonium to meconium which occurs due to leakage of meconium into peritonal cavity as a result of perforation of intestines antinatally which gets subsequently sealed. In the present retrospective study, 39 cases of neonatal peritonitis were studied. Meconium peritonitis was diagnosed if a) abdominal X-ray showed diffuse calcifications (b) abdominal paracentesis showed meconium aspirate and c) leprotomy examination. Twenty (51.3%) out of 39 cases of neonatal peritonitis were found to have meconium peritonitis. These included 14 boys and 6 girls, birth weight ranged from 1500 gms to 3200 gms and mean age of presentation included abdominal distension in 100% cases, H/O not passing meconium in 50% cases, Ascites in 45% cases and vomiting in 40% cases, 30% presented with abdominal mass. Pseudocyst formation on X-ray was seen in 23% cases which was much higher than reported in world literature. Overall mortality in meconium peritonitis was 80%. The incidence of meconium peritonitis in this part of country in much higher than reported in Western and Indian literature. The high incidence may be due to underlying cystic fibrosis because Kashmir has a more homogenous population and consanguinity is very common. A prospective study including sweat chloride testing needs to be undertaken to look into the cause for proportionately higher occurrence of meconium peritonitis in Kashmir with particular reference to cystic fibrosis.     

Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia

A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a persisting urachus were also found. Extra-abdominal malformations such as Down's syndrome, congenital diaphragmatic hernia, aortic stenosis, and malformations of vertebral bodies were seen. Twin siblings of children with IND were either healthy (n=3) or died in utero (n=1). Seventeen children with IND developed severe intra-abdominal complications during the perinatal period such as necrotizing enterocolitis (NEC), meconium ileus, or bowel perforations. NEC was frequently associated with preterm birth. Bowel perforations were seen in mature and preterm newborns with IND. Taken together, IND is found in a variety of obstructive bowel diseases. This may support the hypothesis that IND is a secondary phenomenon or that congenital atresias and stenoses of the digestive tract have a pathogenesis similar to that of intestinal innervation disturbances. IND may also be a part of complex malformation patterns since it occurs with a number of extraintestinal and non-obstructive intestinal malformations.     

Prenatal detection of the cystic form of meconium peritonitis: no issues for delayed postnatal surgery

Prenatal ultrasound (US) diagnosis and postnatal outcome are reviewed in three babies with the complex form of meconium peritonitis (MP), the cystic type. Perinatal management is discussed. Large intra-abdominal cysts with signs of calcifications were detected during the second mid-trimester. Meconium ascites and polyhydramnios appeared between 32 and 35 weeks of gestation. Signs of anaemia were assessed on median cerebral artery peak systolic velocity. Sudden appearance of hydrops and anaemia required preterm delivery, neonatal resuscitation and urgent abdominal drainage. Postnatal US imaging confirmed prenatal sonographic evidence. Abdominal X-ray showed calcifications and no free abdominal air. Intestinal diversion was performed in two patients on their first day of life and evolution was uneventful. Hospital death occurred in one baby, who was submitted to delayed surgery due to unstable hemodynamic conditions. Distal ileal perforation walled off by pseudocysts was detected in all cases. One baby was found to be affected by cystic fibrosis. Ileal intussusception was described in the non-surviving infant. The cystic type of MP may have a potentially rapid lethal course and the onset of foetal anaemia and polyhydramnios is a bad prognostic factor. Severe evolution in hydrops and foetal distress may occur at any moment suggesting the persistence of a leakage or re-rupture of the cysts with new meconium spillage into the abdomen. Prenatal detection of ascites, polyhydramnios and pseudocysts requires a strict follow-up, and timing of delivery has to be planned in a tertiary centre. Postnatal radiological imaging does not offer further information over prenatal imaging and surgical decision should not be influenced by the absence of abdominal free air. Urgent abdominal drainage at birth, followed by intestinal diversion of persistent intestinal perforation on the first day of life, may prevent bacterial colonisation and improve prognosis.     

Abdominal dystocia in a case of undetected intrauterine meconium peritonitis due to cystic fibrosis

The case of a 36 year-old primigravida is presented. After a normal anomaly scan at 22 weeks and a normal pregnancy, she went into labor at term. Dystocia due to massive abdominal distension complicated the second stage. The newborn girl had meconium peritonitis with colonic perforation and required colonic resection with colostomy. Genetic testing detected cystic fibrosis. In this case complex meconium peritonitis developed silently (without any clinical sign) after a normal anomaly scan. This has not been reported since the start of the widespread use of obstetric ultrasound. Late meconium peritonitis can escape detection and should be thought of in cases of unexpected abdominal distension causing dystocia.     

Intestinal atresia

Intestinal atresia accounts for about one third of all cases of neonatal intestinal obstruction. The survival rate has improved to 90% in most of the series with the operative mortality being <1%. The survival rate improves with distal atresias. An increased mortality is observed in multiple atresias (57%), apple peel atresia (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%) and gastroschisis (66%). Although appearance of echogenic bowel on prenatal ultrasonography is suggestive of GI, it is confirmed in only 27% cases. Prenatal ultrasonography is more reliable in detection of dudenal atresia than more distal lesions. Short bowel syndrome is the major impediment in the management of jejunoileal atresia. Although total parenteral nutrition (TPN) is the main adjunctive treatment, it delays intestinal adaptation and may cause cholestasia and subsequent liver damage. Graduated enteric feedings, use of growth hormone, glutamine and modifed diets containing low fat, complex carbohydrates and protein supplements have been used in a adults with short bowel syndrome to successfully diminish TPN requirements and enhance nutrient absorption in nearly half of the patients. Utilization of growth factors to facilitate intestinal adaptation and advances in small bowel transplant may improve the long-term outcomes in future.     

Meconium Ileus: Immediate and Long-term Survival

The results of surgery for meconium ileus obstruction (37 cases) at Queen Elizabeth Hospital for Children, London, during the years 1953 to 1970 are presented. The surgical survival was 30% in the years 1953 to 1961, and 70% in the years 1962 to December 1970, inclusive. The long-term survival was 57%. Complications, such as small bowel atresia, gangrene, and meconium peritonitis, were associated with a higher mortality. The adoption of the Bishop Koop operation in 1962 coincided with the increase in the survival rate. An association between meconium ileus and hypertrophic pyloric stenosis was noted.     

Meconium peritonitis in utero

To clarify the relationship between clinical features in utero and postnatal prognosis, 20 fetuses who underwent ultrasonic (US) evaluation for meconium peritonitis (MP) over a 17-year period were reviewed. According to final US findings in utero, patients were classified into three types. Type I (massive meconium ascites) was noted is 5 cases, type II (giant pseudocyst) in 4, and the other 11 were classified as type III (calcification and/or small pseudocyst). Abdominal calcifications were identified in only 5 cases (2 type I, 1 type II, 2 type III). Seven fetuses who had associated polyhydramnios (1 Type I, 1 Type II, 2 Type III) and fetal hydrops (3 Type II) were delivered before 36 weeks' gestation. Cardiopulmonary resuscitation at birth was required in 9 cases (5 type I, 4 type II) who underwent abdominal drainage before delivery and/or immediately after birth. Although dilatation of the intestine was identified in 10 fetuses (2 type II, 8 Type III), 18 had intestinal atresia and 2 had fecal obstruction of the distal ileum. Four infants (2 type I, 1 type II, 1 type III) died of respiratory failure and postoperative complications. These results indicated that careful fetal US may be useful for perinatal management of MP. Accepted: 15 September 1999     

Acute respiratory infections during pregnancy and congenital abnormalities: a population-based case-control study

Diseases of respiratory system caused by acute infections are among the most common maternal diseases during pregnancy. The objective of the study was to estimate the association between congenital abnormalities and acute respiratory infections during the first trimester of pregnancy. The data set of the Hungarian Case-Control Surveillance of Congenital Abnormalities including 22 843 cases with congenital abnormalities, 38 151 population controls without congenital abnormalities and 834 malformed controls with Down syndrome between 1980 and 1996 was evaluated. 2118 cases with congenital abnormalities (9.3%), 3455 population controls (9.1%) and 92 malformed controls with Down syndrome (11.0%) had mothers with acute respiratory infections. Of 25 different congenital abnormality groups, esophageal atresia/stenosis showed a high adjusted prevalence odds ratios (POR) with 95% confidence interval (CI) for acute respiratory infections during the first trimester of pregnancy in case mothers compared with population controls (3.6, 1.4-9.1) and malformed controls (1.9, 1.0-3.5), respectively. In addition there was an association between medically recorded acute respiratory infections during the first trimester of pregnancy and a higher risk for some other congenital abnormalities, such as posterior cleft palate and multiple congenital abnormalities. In conclusion a possible association between some congenital abnormalities, particularly esophageal atresia/stenosis and maternal acute respiratory infections cannot be excluded due to the interactions of the microbial agents, related drug treatments and last but not least the indirect effect of maternal diseases, such as fever-hyperthermia, hypoxia and dietary deficiency. However, periconceptional multivitamin/folic acid supplementation during the early pregnancy was able to reduce the acute respiratory infection related risk for congenital abnormalities.