“Apple peel” atresia of the small bowel: Prenatal diagnosis of the obstruction by ultrasound

35-year-old woman developed polyhydramnios during her first pregnancy. Ultrasound confirmed this and showed high bowel obstruction. Delivery was uneventful, the infant was asymtomatic. Abdominal films, however, demonstrated a high jejunal obstruction. At surgery apple peel atresia of the small bowel was found. Good outcome.     

Modified plication technique for the treatment of intestinal atresia

Three patients with intestinal atresias were treated by seromuscular stripping and mucosal plication to eliminate the risk of anastomotic leakage through the plication line and reduce the bulk of the plicated mass. The patients all had uncomplicated postoperative courses and tolerated oral feedings within 10 days. Offprint requests to: F. C. Tanyel     

Late presentation of a duodenal web in a patient with situs inversus and apple peel jejunal atresia

A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.     

Significance of antenatal diagnosis of omphalocele

A comparison has been made between 16 infants with omphalocele treated between 1962 and 1975 (group A) and 22 treated between 1976 and 1989. In the latter group 11 antenatally detected cases (group C) were considered separately from the 11 cases diagnosed at birth (group B). Recent advances have reduced the mortality for neonates with omphalocele from 37.5% in group A to 18.2% in group B. However, despite antenatal diagnosis, maternal transport, and early perinatal care, a surprising increase in mortality (45%) was observed among neonates with omphalocele detected antenatally (group C). Seven cases in group C (60%) also had other lethal or potentially lethal multiple anomalies including trisomy 13 or 18, and it is probable that the high mortality in this group was a result to these associated anomalies. Before the introduction of antenatal diagnosis, most of these infants would have died before referral to a pediatric surgeon for treatment. In general, if omphalocele is an isolated lesion the prognosis for survival following postnatal surgical correction exceeds 90%; in such cases, antenatal diagnosis may potentially contribute to improved survival. However, the presence of other multiple and/or untreatable malformations in the group diagnosed antenatally is associated with a poor overall prognosis.     

Concurrent jejunal atresia with “apple peel” deformity in premature twins

Jejunal atresia with apple peel deformity is a rare congenital malformation that is generally believed to result from in utero vascular accidents. We report this case due to the rare occurrence of jejunal atresia with apple peel deformity affecting both members of a set of twins in whom no apparent cause for the atresia exists.     

Gastroschisis with jejunal and colonic atresia,and isolated colonic atresia in dichorionic,diamniotic twins

Despite the increasing incidence of gastroschisis, the cause remains unknown. Genetic factors may contribute to bowel anomalies as demonstrated by cases of gastroschisis in twins and siblings, and other types of bowel anomalies in twins. Atresia of the colon represents one of the rarest causes of neonatal intestinal obstruction. We present the first case of dichorionic, diamniotic male twins in which there was gastroschisis with jejunal and colonic atresia in Twin A and isolated colonic atresia in Twin B.     

Acquired ileal atresia in infancy: report of two cases

Two infants, aged 3 months and 4 months, with acquired ileal atresia in the distal ileum are described. Both of them presented with features of intestinal obstruction following a diarrhoeal episode. In both patients the time interval between the onset of symptoms and detection of atresia at surgery was very short, i.e. 2 weeks. In our opinion, strangulation with subsequent resorption of gangrenous gut could have been the underlying mechanism in both patients. However, a short interval between initiation of symptomatology and detection of atresia prompted us to postulate a thromboembolic phenomenon as the cause of these lesions. While in one patient intussusception was noted on sonography, in the other there was no clear-cut aetiology present except for a high-lying caecum with a long mesentery that might have led to volvulus. We have reviewed the pertinent literature of this very rare sequela of gut inflammation.     

Early one-stage orthotopic jejunal pedicle-graft interposition in long-gap esophageal atresia

Since 1988, four children with long-gap esophageal atresia have undergone one-stage orthotopic jejunal pedicle-graft interposition at the age of 2 to 3 months. Obtaining enough jejunal length was no problem and major early complications did not occur. In one patient stenosis of the distal anastomosis was problematic and required corrective surgery. None of the patients demonstrated jejunitis as a result of gastroesophageal reflux. With follow-up periods of 12, 27, 46, and 60 months, all patients are doing well. It is concluded that the jejunum is a better esophageal substitute than is generally appreciated.     

A rare type of ileal atresia due to intrauterine intussusception

Intrauterine intussusception is an extremely rare cause of intestinal atresia. We report on a full-term neonate with clinical manifestations of intestinal obstruction two days after birth. The prenatal sonography at the late stage of pregnancy did not show any abnormality. The barium enema suggested distal intestinal obstruction. At surgery, a visible ileo-ileal intussusception resulting in ileal atresia was found. According to our knowledge, this presentation of ileal atresia did not fit into the present classification and have not yet been reported on. We present this rare type of ileal atresia due to intrauterine intussusception with operative evidence, which is the first case reported in the medical literature so far.     

新生儿外科性疾病产前诊断的临床观察与分析

目的分析新生儿外科性疾病的产前诊断现状，探讨产前诊断对新生儿外科疾病转归的影响。方法2004年2月～2006年7月，产前多科会诊465例，进行胎儿及新生儿随访。分析产前会诊疾病分类，比较产前诊断新生儿外科性疾病与生后诊断的符合率，探讨产前诊断后胎儿处理对新生儿外科造成的影响作用。结果产前会诊465例中胎儿因素占395例（84．9％），外科性疾病315例，占79．7％（315／395）。395例中125例选择人工流产，占31．6％；32例新生儿生后2个月内接受手术，16例生后新生儿死亡。胎儿会诊疾病中占据前5名的为泌尿系异常73例（18．5％）、脑部异常包括脑室扩张和脉络膜囊肿60例（15．2％）、多发畸形42例（10．6％）、宫内感染包括宫内发育迟缓39例（9．9％）和心脏畸形32例（8．1％）。腹裂、脐膨出、膈疝、胸部占位性病变（肺纤维囊性变）和颈部巨大淋巴管瘤的人工流产终止妊娠率均高于50％。结论产前会诊中新生儿外科疾病占多数，产前诊断后部分新生儿外科疾病人工流产率较高。产前诊断的胎儿肾盂扩张或脑室扩张，随访中有较高的消退率和〈15％的新生儿手术率。     

Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s

Oesophageal atresia management has evolved alongside the development of paediatric surgery. An analysis of a 30-year prospective collection of oesophageal atresia cases treated at the Royal Alexandra Hospital for Children, NSW, Australia is presented. There has been a dramatic change in the surgical approach. Fewer infants are undergoing operations of cervical oesophagostomy, gastrostomy and oesophageal replacement. More, including fragile infants, are surviving with a repaired native oesophagus. Fewer infants are suffering morbidity secondary to anastomotic leak. The progression in treatment has been enabled by improved neonatal support due to advances in neonatology, neonatal anaesthesia, nutritional support and antimicrobial therapy. Oesophageal atresia treatment and outcome has changed markedly over three decades. Cooperative multi-centre database development is now required to provide data in order to further refine treatment for clinical challenges that remain.     

影响胆道闭锁早期诊治的原因分析

目的：分析影响胆道闭锁早期诊断和治疗的因素并探讨解决对策。方法：回顾性总结与分析70例胆道闭锁患儿就诊年龄与临床表现、主诊医生和家长对本病的认识、影像学检查结果等方面对早期诊断的影响，探讨延误诊治的各种原因。结果：影像学检查中，肝胆核素检查27例，6例误诊。B超检查在未掌握观察肝门纤维块前，23例中仅有14例诊断正确，而在掌握该方法后，检查38例，35例诊断正确。结论：先天性胆道闭锁延误诊治的原因有：临床表现不典型、观察病情不细致、主诊医生对本病的认识不足、影像学检查的误诊等。因此建议：仔细体检,医生亲自观察大便颜色；B超检查内容包括观察肝门纤维块和胆囊进食前后的变化。肝胆核素检查， 应防止放射物污染，减少同位素在体内的分解水化。     

Rapid enlargement of a choledochal cyst: antenatal diagnosis and delayed primary excision

A case of choledochal cyst (CC) antenatally diagnosed at 29 weeks' gestation is reported. Rapid enlargement of the cyst soon after delivery resulted in complete gastric outlet obstruction (GOO). The lesion was treated by external drainage as a temporary maneuver, with delayed cyst excision and hepaticoduodenostomy at the hepatic hilum performed at 81 days of age. Surgical treatment of CC in early infancy has been reported to be safe and effective. However, delayed primary excision would be an alternative procedure, especially in rare cases showing rapid enlargement resulting in GOO, since this choice has the potential advantage of allowing weight gain and improved nutritional status without risking interim complications due to the drainage procedure. Accepted: 24 March 1997     

A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel's diverticulum

Intrauterine intussusception is one of the rare causes of intestinal atresia. Conclusion: A case of ileal atresia due to intrauterine intussusception caused by Meckel's diverticulum is reported.     

Hyaluronic acid: Additional biochemical marker in the diagnosis of biliary atresia

BACKGROUND: The purpose of the present paper was to evaluate the value of biochemical markers, including conventional liver function tests, gamma-glutamyl transferase (GGT), and hyaluronic acid (HA), in the diagnosis of neonatal cholestasis. METHODS: Infants with neonatal jaundice were consecutively enrolled during 1 year period. The patients were diagnosed as having biliary atresia (BA) if there was either bile ductular proliferation in the portal tracts, atretic common bile duct/gallbladder, or evidence of bile duct obstruction demonstrated by liver pathology or intraoperative cholangiography, respectively. Serum HA was measured using an enzyme-linked immunosorbent assay-based test. RESULTS: A total of 25 patients diagnosed as having BA (n = 10), neonatal hepatitis (NH; n = 9), choledochal cyst (n = 3) and parenteral nutrition-induced cholestasis (n = 3), were studied. The age at diagnosis was not significantly different between groups. Only GGT and HA were significantly elevated in the patients with BA when compared to NH (P = 0.02, P = 0.03, respectively). In BA, the median value of serum HA was 514 ng/mL (range 19-4476 ng/mL), compared to 50 ng/mL (range 19-315 ng/mL) in NH. Additionally, the serum HA level was much higher in children with choledochal cyst. CONCLUSION: HA could be considered as a complementary biochemical marker for evaluating infants with prolonged jaundice.     

Prenatal diagnosis of isolated congenital pyloric atresia in a sibling

Although familial occurrence of congenital pyloric atresia (CPA) has been frequently reported in the past, many of these cases were associated with epidermolysis bullosa (EB), and familial isolated CPA was a relatively rare condition. We prenatally diagnosed and successfully treated a sibling of a subject with isolated CPA, who was diagnosed prenatally by fetal ultrasonography based on the findings of a distended stomach combined with polyhydramnios. The first case was a 2398‐g female infant born at 36 weeks of gestation, who had been prenatally diagnosed as CPA. The second case, a younger sister of the first case, was a female infant weighing 2434 g, who had been also diagnosed as CPA by fetal ultrasonography at the check‐up for the polyhydramnios of the same mother. Neither of the infants showed dermal lesions such as EB, and both underwent pyloroplasty with an excision of the pyloric membrane successfully after birth.     

Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis

This retrospective study reviews the medical records of 77 fetuses and babies with congenital diaphragmatic hernia (CDH) referred to two hospitals in Detroit from 1986 through 2000. The aims were to examine the effects on outcome of multiple variables, especially the type of CDH, associated anomalies, and ultrasound prognostic parameters. Ultrasound measurements of head (HC), chest (CC), and abdominal circumferences (AC) were obtained from videotapes. ANOVA and chi-square analysis were used to determine statistical significance between groups and proportions. Eighty-nine percent (65/73) of pregnancies resulted in live births, and 54% (35/65) of patients survived past 30 days. Liveborn patients with low APGAR scores were less likely to survive. Forty-three percent (30/70) had major associated anomalies, with cardiac anomalies constituting about 52% (33/64) of the major associated anomalies. Seventy percent of patients with isolated CDH survived versus 36% of patients with both CDH and cardiac anomalies. Sixty-seven percent (8/12) of fetuses antenatally diagnosed before 25 weeks of gestation survived past 30 days of birth. The survival rate of right-sided CDH with liver herniation was 80% (8/10), compared with 29% (4/14) for left-sided CDH with liver herniation (p=0.088). There was a significant linear relationship (r=0.603, p =0.029) between CC/AC and CC/HC among patients with CDH; survivors had higher CC/AC and CC/HC values than nonsurvivors. These results support the utility of CC/AC and CC/HC measurements and the presence of liver herniation as important prognostic factors that can be used in antenatal counseling and in planning clinical trials.     

Ovarian torsion cyst presenting as a wandering tumor in a newborn: antenatal diagnosis and post natal assessment

A case of an in-utero torsion ovarian cyst is reported. The diagnosis was made antenatally and the 4-cm cystic mass was followed by US from birth until the age of 3 months. Surgery was delayed until the baby reached a satisfactory weight. In the mean-while, the follow-up studies showed the mass to wander in the abdomen. Surgery revealed an ovarian cyst. Ovarian torsion cysts should be considered in the differential diagnosis of wandering tumors.Deprtment of Radiology (Dr. H.J.Daled)     

Bezoar formation and malabsorption secondary to persistent dilatation and dysmotility of the duodenum after repair of proximal jejunal atresia

Bezoar formation is a rare but well-recognised complication of previous gastric surgery and persimmon ingestion. We present a case of bezoar formation in the duodenum of a child with a grossly dilated, dysmotile duodenum following surgery for jejunal atresia. The presenting symptoms were vomiting, diarrhoea, and growth failure, with evidence of malabsorption secondary to bacterial overgrowth in the bezoar. Plication of the duodenum to reduce its size and improve its motility may reduce the likelihood of future bezoar formation.     

A rare association of diphallus,colonic duplications,ileal atresia,and an anorectal malformation

Diphallus is a rare congenital anomaly that is often associated with various other anomalies of the midline structures of the posterior portion of the body. We report a unique case of a 1-day-old male with complete diphallus, incomplete bilateral tubular duplications of the colon, ileal atresia, and an anorectal malformation.