Meconium per vaginam secondary to ileal atresia and meconium peritonitis

A neonate was referred for an abdominal mass and passing meconium per vaginam. The anorectum was normal. Roentgenograms revealed a small-bowel obstruction and hypogastric peritoneal calcifications. Findings at celiotomy were: ileal atresia, meconium peritonitis, and two meconium-filled pseudocysts draining through the female genital tract. Excision of the inflammatory masses, resection of the atretic segment, and primary ileoileostomy were followed by an uneventful recovery. Only two similar cases, both fatal, are reported in the English literature. Meconium peritonitis should be considered in the neonate passing meconium per vaginam.     

Intrauterine intussusception—A cause for ileal atresia

A case of ileal atresia consequent to intrauterine intussusception is reported. The baby presented with features of neonatal intestinal obstruction but signs of peritonitis were absent. The intussusception was discovered on gross examination of distal atretic ileal segment. The case was managed successfully by resection and end to back anastomosis. This case is reported to highlight intrauterine intussusception as one of the causes of ileal atresia.     

Clinical experience of complex jejunal atresia

Purpose

Small intestinal atresia is relatively common anomaly that causes intestinal obstruction in neonates. Although surgical interventions are usually successful, critical problems could raise in certain cases. This study aimed to identify the distinct clinical characteristics of complex cases of jejunal atresia by retrospective analysis.

Methods

Overall, 91 cases of small intestinal atresia, which occured in infants between 2001 and 2010 at Pusan National University Children’s Hospital, were reviewed retrospectively. The clinical characteristics of complex jejunal atresia were analyzed.

Results

Of the 91 small intestinal atresias, 11 cases of complex jejunal atresia were found: high jejunal atresia with distal deletion, 3; high jejunal atresia with distal multiple atresias, 4; jejunal atresia with distal apple peel appearance, 1; jejunal atresia with colonic atresia, 1; jejunoileal atresia with distal volvulus, 2. Short bowel syndrome was found in four patients and bowel-lengthening procedure was performed in all. Three patients presented with an adhesive intestinal obstruction during the early postoperative period. Postoperative mortality occurred in one patient with distal volvulus.

Conclusions

From a surgical perspective, complex jejunal atresia can cause many critical problems after the correction operation. An aggressive and multidisciplinary approach is necessary for managing this condition.     

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.     

T-tubes in the management of meconium ileus

Fifteen cases of meconium ileus (MI) were treated between 1986 and 1995; 7 responded to conservative treatment. Eight were operated upon, and comprise the study group. Six of the operated babies had no complications; 1 had meconium peritonitis with a pseudocyst and small-bowel atresia, and 1 had a volvulus of a small-bowel segment with necrosis. In all 8 cases a T-tube (TT) was left via an enterotomy; in the complicated cases the enterotomy was pre-anastomosic. The obstruction was relieved in all the babies, without any stoma or bowel resection in the uncomplicated cases. Two complications occurred: 1 patient died of r failure 1 month following surgery and another required a relaparotomy for lysis of adhesions. We conclude that TT ileostomy is an effective and safe procedure for uncomplicated cases of MI that do not respond to conservative therapy, as well as for complicated cases that need an anastomosis.     

Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung’s disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.     

Congenital pyloric atresia and associated anomalies

Congenital pyloric atresia (CPA) is a very rare condition that was first described by Calder in 1749. Commonly, CPA occurs as an isolated lesion, which has an excellent prognosis, but it can also be seen in association with other malformations, which can have a negative impact on the final outcome. The medical records of all patients with the diagnosis of CPA treated at our hospital were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, history of polyhydramnios, diagnosis, associated anomalies, operative findings, treatment and outcome. Eleven cases with the diagnosis of CPA were treated at our hospital. There were five males and six females. Seven were products of full term normal vaginal delivery and the remaining four were prematures. Their birth weights ranged from 1.2 to 3.9 kg (mean 2.2 kg). Polyhydramnios was seen in seven (63.6%). Associated anomalies were seen in six (54.5%). These included epidermolysis bullosa (EB) in three, hereditary multiple intestinal atresias (HMIA) in two including a duplication cyst in one of them, esophageal atresia in one, Down’s syndrome in one, congenital heart disease in one, cleft palate in one and aplasia cutis congenital (ACC) in one. Intraoperatively, five had pyloric diaphragms, in two of them there were double diaphragms, three had pyloric atresia with a gap between the two ends and two had pyloric atresia with no gap. One of them had duodenal perforation as well as ileal perforation. The patients with pyloric diaphragms had excision of diaphragms and Heineke–Mickulicz pyloroplasty. Four of the other five patients had gastro-duodenostomy. One of them also had duodeno–jejunostomy due to associated distal duodenal atresia and excision of duplication cyst. The patient with duodenal and ileal perforation and because of the marked distension of the duodenum had reduction duodenoplasty, gastroduodenostomy, and duodeno–jejunostomy. The area of ileal perforation was resected and end-to-end anastomosis was done after correction of the associated malrotation. One patient with associated esophageal atresia had gastrostomy and gastrojejunostomy. Post-operatively, all did well initially, but subsequently, six of them died giving an overall survival of 45.5%. Sepsis was the cause of death in all of them. CPA is very rare and when it occurs in isolation it has a good prognosis. The association of CPA with HMIA is universally fatal. CPA in association with EB has a high mortality but there are encouraging results with the use of steroids and phenytoin. Sepsis continues to be the main cause of death and an associated combined immunodeficiency should be excluded.     

Multiple intestinal atresias and encephalomalacia in the survivor after in-utero death of a monozygous co-twin

A twin infant who survived the death of his monochorionic co-twin in utero and was born with multiple jejunoileal atresias and severe cerebral damage is reported. The pathogenesis of these defects is discussed in the light of previously reported similar cases. The incidence of severe brain damage in twins born alive after the intrauterine death of a monochorionic co-twin is high, suggesting brain destruction by embolization of thromboplastin-rich material from the dead fetus via placental vascular anastomoses. A similar mechanism could be evoked in the present case to account for the intestinal atresias, which likely occurred after the 22nd gestational week, i.e. after the normal permeabilization of the embryonic gut. In the light of this observation and similar ones made by others, it is clear that surgical management of congenital defects during the neonatal period should be influenced by the knowledge of in-utero death of a monochorionic co-twin during the second half of gestation.     

Long-term results of delayed primary anastomosis for pure oesophageal atresia: a 27-year follow up

The management of a newborn with pure oesophageal atresia continues to be challenging. We started treating babies with pure oesophageal atresia by delayed primary anastomosis in 1977. The purpose of this study was to review the long-term outcome in infants with pure oesophageal atresia (EA) treated by delayed primary anastomosis with special emphasis on gastroesophageal reflux (GOR) related morbidity. The medical charts of all patients treated by delayed primary anastomosis between 1977 and 2004 were retrospectively reviewed. All survivors were followed up with completion of a questionnaire and personal/phone interviews. There were 26 patients in total admitted during the 27-year study period with the diagnosis of pure oesophageal atresia. Three died prior to surgery due to associated anomalies; two had almost no distal oesophageal segment and underwent oesophageal replacement surgery. The remaining 21 children were treated with delayed primary anastomosis and made up our study group. There were four deaths (19%) in this group, and all were prior to 1980. The median gestational age was 35.5 weeks and the median birth weight was 2.6 kg; median initial gap was 3.7 cm and median preoperative gap was 1.5 cm; median age at operation was 80 days and the median hospital stay was 5.5 months. The median follow-up period was 13.5 years. Fourteen children (66%) developed symptomatic gastroesophageal reflux and nine of these needed fundoplication (43%). Sixteen children developed strictures at the anastomotic site; ten responded to repeated dilatations while six needed resection and reanastomosis. At the time of this study, 15 out of the 17 survivors (88%) were on normal diet with no respiratory problems and 2 (12%) were dependent on gastrostomy feeds. Our long-term follow-up data shows that the delayed primary anastomosis provides excellent functional results in patients born with pure oesophageal atresia. The high incidence of gastroesophageal reflux and associated morbidity requires early intervention to prevent ongoing feeding problems due to oesophagitis and stricture formation.     

Intestinal atresia: management problems in a developing country

Over a 13-year period, 24 children with intestinal atresia were managed at the Jos University Teaching Hospital, Jos, Nigeria. Intestinal atresia ranks as the second most common cause of neonatal intestinal obstruction (after anorectal malformation) in our hospital. Five children had duodenal obstruction (two atresia, two duodenal webs, one annular pancreas), 17 had jejunoileal atresia, and two had colonic atresia. Fourteen were boys, and 10 were girls (M:F: 1.17:1). The median age at presentation to the surgeon was 6 days (range: 1 day-12 years). The most common presenting features were bilious vomiting and abdominal distension. Six patients did not pass meconium within the first 24 h of birth. The median weight at presentation was 2.6 kg (range: 1.1 kg-5.0 kg). Seven patients (four with jejunoileal atresia and three with duodenal obstruction) had associated congenital anomalies. Diagnostic investigation was plain abdominal x-ray, showing double-bubble gas shadows in duodenal atresia and varying degrees of air-fluid levels in jejunoileal and colonic atresias. An upper gastrointestinal series was done in three patients and a barium enema in one. Retrocolic duodenojejunostomy was done for all patients with duodenal atresia and annular pancreas, duodenotomy and web excision for those with duodenal webs, and resection with end-to-end anastomosis for those with jejunoileal atresia. One child with atresia involving the whole ileum and the colon had a jejunorectal anastomosis, while the other child with colonic atresia had caecostomy followed later by ileorectal anastomosis. Ten neonates died, giving a mortality rate of 41.7%. Mortality from intestinal atresia is still high in our environment, due mainly to lack of neonatal intensive care facilities.     

Intestinal atresia: an end-to-end linear anastomotic technique

In a series of 19 neonates with small-bowel atresia, 16 were treated by end-to-end linear anastomosis (ELA) without resection and 3 by resection anastomosis (RA). Seven atresias were jejunal, 11 were ileal, and 1␣jejunoileal; 3 cases were type II, 12 type IIIa, 3 type IIIb, and 1 type IV. There were 4 deaths, 1 after ELA and 3 after RA. The overall mortality decreased from 68 to 20.80% and for linear anastamosis to 6.25% presumably, because the intestinal contents are propelled along the lumen in a linear fashion and not at an angle as in end-to-back anastamosis, avoiding shearing of the suture line. The additional plicating sutures reduce the radius and increase the propelling force. We recommend this technique because it is based on sound principles of physics and preserves the entire available length of intestine. Accepted: 8 January 2001     

Neonatal survival of prenatally diagnosed exomphalos

Purpose  Exomphalos is a midline defect, with a viable sac composed of amnion and peritoneum containing herniated abdominal contents with an incidence of about 1 in 4,000 live births. Associated major abnormalities can be karyotypic, syndromic or structural in up to 70% of cases. The aim of this study is to determine the factors that influence survival of antenatally diagnosed exomphalos. Methods  All antenatally diagnosed and postnatally confirmed exomphalos registered with our fetal medicine unit, during 2002–2007, were reviewed. Both prenatal and postnatal outcomes were analysed. Results  Of 88 cases identified with exomphalos, 85 were prenatally diagnosed. Fifty-five of them died in utero (45 terminations, 5 spontaneous abortions and 5 still births). There were 33 live births (37.5%), 7 of which were premature (30–35/40 gestation). Five babies died before coming to surgery (all with major exomphalos as well as abnormal karyotype) while 28 were operated upon. Fourteen cases with minor exomphalos, all isolated, were primarily closed and all survived to discharge. Of 14 babies with major exomphalos, 4 were closed primarily. Nine required silo formation and six successfully underwent secondary closure (one of which had a prenatal diagnosis of giant ruptured exomphalos). Three died before closure, two from sepsis and multi-organ failure, and one from an undiagnosed tracheo-oesophalgeal cleft. All three deaths had antenatally diagnosed giant ruptured exomphalos and were less than 34/40 weeks gestation. One baby was managed conservatively with antiseptic solution applied to the sac and left to heal by secondary intention. There were 17 cases of isolated exomphalos (with no other structural abnormalities), all of which survived. Conclusion  Antenatal diagnosis of exomphalos is 96% sensitive. Severe karyotypic and structural abnormalities were present in all intra-uterine and early postnatal deaths. Overall survival to discharge was 28%. Both minor and isolated exomphalos carried a good prognosis. Isolated exomphalos was a better prognostic factor than severity of the exomphalos itself. Ruptured giant exomphalos were associated with a poorer outcome especially in premature babies.     

Fetal exposure to cocaine and infections during the first 18 months of life in infants from a Mediterranean city

AIM: The incidence of infections during the first 18 months of life was investigated in 36 infants prenatally exposed to cocaine and in 72 non-exposed controls from Barcelona, Spain. METHODS: Fetal exposure to cocaine was ascertained by meconium analysis, infections by structured questionnaire. RESULTS: A higher incidence of infections, if excluding those acquired in utero, was not found in exposed infants versus non-exposed infants of similar demographical and socioeconomic characteristics. CONCLUSIONS: A possible role for life style factor in those cases where increased infections are associated with fetal exposure to cocaine is hypothesized.     

Pyloro-duodenal atresia

Seven patients born with pyloro-duodenal atresia are reported, one had an additional atresia of the small and large bowel, and another had a pneumoperitoneum probably secondary to rupture of the stomach. All patients had the same radiological findings and had no air distal to the pylorus. Consanguinity was elicited in four and a familial incidence of an identical malformation in siblings in six. 6 of the 7 patients were operated on and 4 are living and well 6 months to 5 years later. This report raises the total number of published cases of pyloro-duodenal atresia to 46, and provides further evidence for an autosomal recessive mode of inheritance.     

Morphological abnormalities in the innervation of the atretic segment of bowel in neonates with intestinal atresia

The aim of this study was to examine precisely the morphological abnormalities in the myenteric plexus at the atretic end of the bowel in jejunoileal atresia (JIA). Although changes in the myenteric plexus has been examined in the proximal and distal segments of bowel in JIA, a histochemical analysis of the atretic segment is lacking. Specimens from the atretic end of bowel were obtained from six neonates with JIA. Whole-mount preparations were made of the myenteric plexus, and the cholinergic and nitrergic components were studied by staining with acetylcholine esterase (Ach E) and NADPH diaphorase, respectively. Controls were obtained from two neonates undergoing small bowel resection for Meckel’s diverticulum. At the blind end of bowel in type 3a atresia (5 neonates), the intensity of NADPH staining was comparable with controls. However, there was distortion of polygonal architecture of the primary and secondary plexuses at the blind end arranged in concentric fashion parallel to the circular muscle fibres. The ganglia were large and irregularly shaped and contained round neuronal cells. In the sausage shaped segment of bowel in multiple atresia, there was total loss of polygonal architecture with abnormal ganglia, and whorls of nerve fibers. Neuronal cells could not be made out in the ganglia. In specimens stained with Ach E, the findings were similar except that the staining intensity was markedly reduced compared with controls. The morphological abnormalities in the atretic bowel in type 3a were restricted to the architecture of the plexuses and ganglia. The neuronal cells were normal. However, the total loss of polygonal architecture and absence of neuronal cells in the ganglia in multiple atresia probably indicate a different etiology for this type of atresia     

延期食管端端吻合术治疗I型食管闭锁

目的本文介绍4例I型食管闭锁延期食管吻合的治疗经验,供临床参考。方法4例均为女性,出生体重分别为2．75kg、2．30kg、2．45kg、2．50kg,无早产及窒息缺氧史,3例产前均有羊水过多史,出生后口鼻大量泡沫,置入胃管困难。x线片见食管盲端位于T2～T3水平而腹部无充气。所有患儿均于出生后2～3d行第1次手术,采用单纯胃造瘘术,术中阻断胃远端,近端造影显示两盲端距离,证实两盲端距离为5～7个椎体,I期食管端端吻合不可能;术后予食管近端经鼻置管,每2～3h抽吸唾液,同时经胃造瘘进行肠道营养;第2次手术于12～16周后进行,经胸行食管食管吻合术。结果第1次手术时两盲端距离分别为7cm、5．5cm、5cm、4cm,术后经胃造瘘喂养;第2次手术时食管盲端均增粗明显,盲端间距均缩短,分别为5cm、4cm、3．5c、3cm,其中2例经松解周围粘连及肌层切开,完成一定张力下吻合。1例采用内牵引法,另1例行外牵引,5～7d后再次经胸行食管-食管端端吻合术。4例术后均以呼吸机辅助通气,其中3例发生吻合口漏,经保守治疗后痊愈。3例术后发生吻合口狭窄经胃镜下扩张好转,术后随访3个月至2年,2例存在胃食管返流,予保守治疗。结论对I型食管闭锁进行胃造瘘后延期手术可缩短食管盲端间距离,内牵引或外牵引法可在短期内诱导食管盲端迅速生长,为食管一食管吻合术提供可能。     

The role of fiberoptic endoscopy in the evaluation and management of long gap isolated esophageal atresia

Accurate measurement of gap length is useful for operative planning in cases of esophageal atresia (EA) without distal fistula. This paper demonstrates how fiberoptic endoscopy of the distal esophagus enables measurement of the gap in the case of isolated EA, and compares other commonly practiced techniques.     

Ileal atresia associated with an omphalomesenteric duct remnant

This paper describes an unusual case of ileal atresia associated with an omphalomesenteric duct (OMD) remnant. A 1-day-old male developed bilious vomiting and abdominal distension. At laparotomy, the distal portion of the ileum was connected to the umbilicus with a fibrous band. Around this omphalomesenteric band, the ileum was strangulated and necrotic; the end of the ileum was atretic. Apart from these structures, the cecum terminated without mesentery. This experience shows evidence of an OMD remnant as a possible cause of jejunoileal atresia. Accepted: 20 December 1996     

Unusual cases of neuronal intestinal dysplasia

Six children with isolated neuronal intestinal dysplasia (NID) and unusual complications are presented. Three had meconium peritonitis, one of them in association with a solid pyloric atresia, one midgut volvulus, one rectal stenosis, and one jejunojejunal intussusception. These cases show that patients with isolated NID may present with a heterologous pattern of symptoms very different from those generally reported, indicating that NID may not be a distinct clinical entity. The symptomatology of NID seems to be directly correlated with the degree of functional defect of gut motility. NID should be suspected in any case of functional small-bowel obstruction. Offprint requests to: P. Sacher     

Intrauterine intussusception causing intestinal atresia during pregnancy]

CASE REPORT: A case of intrauterine intussusception causing ileal atresia is reported. The neonate presented at the age of one day with bilious vomiting, failure to pass meconium and progressive abdominal distension. A clinical diagnosis of intestinal obstruction was made, and was confirmed by plain abdominal radiographs. At laparotomy, atresia of the terminal ileum was found. The lumen of the resected distal segment contained an intussusceptum. The blind ends were resected and an anastomosis was performed. Recovery was uneventful. COMMENTS: Intrauterine bowel intussusception is a rare clinical entity. When intussusception occurs in the foetus and the time elapsed is enough to allow for gangrene and resorption of the intussuscepted portions of the bowel, intestinal atresia may result. CONCLUSIONS: The careful examination of the distal blind end of the ileum aids to obtain a correct classification and etiologic diagnosis of the atresia.