Jejunoileal Atresia Research Articles

APPLE PEEL JEJUNAL ATRESIA IN A NEONATE: A RARE CAUSE OF INTESTINAL OBSTRUCTION

A preterm baby was born with antenatal diagnosis of upper gut obstruction. Postnatal abdominal x-ray showed triple gas shadows and ultrasound scan showed few dilated bowel loops. Laparotomy revealed apple peel deformity type of jejuno ileal atresia. Tapering duodeno-jejunoplasty with end to side anastomosis and transgastric feeding jejunostomy was carried out. Gradually feeds were introduced. Apple peel jejunal atresia classified as type iii-b jejunoileal atresia, is a rare malformation with a high incidence of mortality and morbidity. We are reporting a case of apple peel jejunal atresia in a preterm baby with survival.

Epidemiology of small intestinal atresia in Europe: a register-based study

BackgroundThe epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age...

Jejunoileal atresia: Factors affecting the outcome and long-term sequelae

Context:Jejunoileal atresia (JIA) is a common abnormality. The outcome is conditioned by several variables. Nutritional problems, and long-term sequelae are described among those who survive.Aim:To correlate the type of JIA and its management to the outcome and long-term quality of life.Settings and Design:Forty-three cases over a 17-year period (1992–2009). Perinatal data, management, and outcome were extracted from the clinical notes. The cases that had survived were contacted to get information about their present condition.Materials and Methods:Morbidity and mortality were matched to maturity, birth weight, mode of diagnosis, type of JIA, associated anomalies, and management and duration of parenteral nutrition. Growth and quality of life in 34 cases were evaluated via a telephone interview at a minimum of one year from surgery.Statistical analysis:Fisher test, Linear regression test, Kruskal-Wallis test, Dunn's comparison test.Results:Male/Female ratio was 25/18 and median birth weight was 2.644 g. Prenatal diagnosis was recorded in 34%. Six patients (14%) had associated anomalies. Primary surgery was resection and anastomosis in 88% and temporary stoma in 12%. Length of the resected bowel ranged from 3 to 65 cm. Whenever multiple atresia was found, the bowel length was saved by multiple anastomosis. Three dehiscences and three adhesive obstructions required a reoperation. Two patients (4%) died due to a central catheter–related sepsis. Prenatal diagnosis did not influence the outcome and was associated with a higher rate of Cesarean deliveries. Interview, at a median of nine years, showed normal growth in 85%. One case of short bowel syndrome is still on parenteral support at 22 months.Conclusions:Preserving bowel length and reducing the recourse to stoma is the key to good outcome and growth. Sequelae are correlated with the type of atresia and length of residual bowel; however, they decrease their severity with time.

Jejuno-ileal atresia: A 2-year preliminary study on presentation and outcome

Intestinal atresia is a common cause of neonatal intestinal obstruction. Jejuno-ileum is the commonest site of intestinal atresia. Reports on Jejunoileal atresia in developing countries are still few. The purpose of this study is to determine the presentation and management outcomes of neonates with Jejunoileal atresia treated in our hospital. Detailed data on all babies that presented and were treated within the study period (November2008-November, 2010) were kept and analyzed. A management protocol was put up and maintained. A total of 9 babies (7males and 2 females) were treated. They were aged 2 hours to 13 days. Their weight ranged from 1.7kg to 3.3kg. Apart from one baby which presented within 2hrs with prenatal ultrasound diagnosis, others had bilious vomiting from the first day of birth, abdominal distension and delayed or absent passage of meconium. Even though symptoms developed on the first day of birth, presentation to the surgical unit was delayed 72hours and beyond in most patients.Type I atresia is commonest (no=4).There is associated gut malrotation in 2 babies. Five babies had complications which included surgical site infection, sepsis, prolonged vomiting post operatively, aspiration, rupture of dilated proximal segment after membrane excision, entero-cutaneous fistula and malnutrition. Three babies died giving a mortality of 33.3%. Mortality is commoner in types IIIb and IV. Mortality is higher in complex atresia which most times will require neonatal intensive care and parenteral nutrition facilities. These are still lacking in our institution. Providing these facilities will further improve outcome.

Surgical treatment of neonatal intestinal malformations

Oesophageal atresia (OA) is a congenital anomaly with an interruption of the oesophageal continuity with or without tracheo-oesophageal fistula (TOF). Type IIIB is the most common (85%) and shows hypersalivation post partum followed by recurrent attacks of suffocation as a consequence of recurrent aspirations. A nasogastric tube cannot be passed and radiodiagnostic investigations visualize the blind-ending upper oesophageal pouch. Surgical management includes the closure of the TOF and primary anastomoses of the blind ends. In case of a long gap, narrowing and delayed primary repair can be performed. Prognosis and survival are related to birthweight and to associated major cardiac defects. In patients with congenital duodenal atresia (DA), the complete interruption of the bowel continuity can be diagnosed antenatally by sonography and post partum by plain abdominal radiographs, showing the classic ‘double bubble’ sign. The obstruction can be due to an intraluminal web, complete separation of the duodenal ends, or caused by an extraluminal pancreas annulare or Ladd bands seen in the case of malrotation. Infants show bilious vomiting after birth and consecutive metabolic alkalosis. The surgical method to bypass the obstruction is a duodenoduodenostomy. Mortality rate is < 5%. Jejunoileal atresia (JIA) is a common cause of neonatal intestinal obstruction. More than 90% of patients have a single atresia. In the case of multiple atresias, most of the children are born prematurely with low birthweight. JIAs can be suspected prenatally via sonography. Main symptoms are bilious vomiting, a distended abdomen and signs of life-threatening sepsis in the case of bowel perforation. Surgical treatment has the aim of reconstructing bowel continuity and saving as much bowel length as possible to avoid the complications of short bowel syndrome and malabsorptive syndromes. Anorectal malformations (ARMs) comprise not only defects of the lowest part of the intestinal tract but also of the urinary and genital tract. In minor low types, the rectum and its fistula open into the perineal region and primary surgical repair by posterior sagittal approach is performed. In higher, more complex types, meconium passes into the male urethra or bladder or into the female vagina. In these cases, a colostomy and staged repair has to be performed. Complex types of ARM have a poorer outcome regarding bowel and urinary control.

Unusual Case Of Jejunal Atresia: A Mixture Of Type I And Type IIIa

Intestinal atresias are a most common cause of bowel obstruction in the newborn. Jejunoileal obstruction was found in 46% of the intestinal atresia. The classification of jejunoileal atresias defined 4 types of lesions. Our case does not conform these types. A mixture of type I and type IIIa. This atresia included fenestrated mucosal web with minimal mesenteric defect. Being a life threatening surgical emergency, prompt diagnosis and opportune treatment are required to prevent mortality and other complications in intestinal atresias. In conclusion, intestinal atresias should be remembered in bilious vomiting of newborn. And intestinal atresia type may be unusual.

Apple Peel Small Bowel, A Review of Four Cases: Surgical and Radiographic Aspects

Background: Although apple peel intestinal atresia is rare and is associated with a high mortality and morbidity, there is a dearth of its report in African literature. This study reviews four of the cases seen in a state teaching hospital in Lagos, considering the radiographic findings, surgical management and outcome. A brief review of literature is also undertaken. Aims and Objectives: To correle the plain radiographic findings with the surgical findings of neonates gathered over a six year period and to evaluate their surgical management, hoping to further help improve management of such neonates’ in future in resource limited regions such as ours. Methodology: It was a retrospective case series of four neonates who were brought in over a period of six years and operated after an initial plain abdominal X-ray. They were done as emergency cases, consent was obtained from their parents and the study was approved by the research and ethics committee. Operative findings were subsequently correlated with their radiographic findings and the surgical outcomes and follow up were documented. Conclusion: This study revealed that “the triple bubble sign” is a common radiographic finding in Apple Peel deformities, as well as gangrene of the jejunum and ileum at surgery. Key words: Apple peel atresia; Jejunoileal atresia; Plain Abdominal Radiography; Parenteral nutrition; Short Bowel Syndrome. DOI: http://dx.doi.org/10.3126/jnps.v31i3.5037 J Nep Paedtr Soc 2011;31(3): 227-234

Small intestinal atresia in europe: prevalence, associated anomalies and pregnancy outcomes

BackgroundSmall intestinal atresia (SIA) is a congenital anomaly characterised by the abnormal closure, discontinuity or narrowing of the duodenum, jejunum or ileum. This study aimed to describe the epidemiology of...

Apple-peel atresia presenting as foetal intestinal obstruction

Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

Evaluation of epidemiologic indices of neonateʼs diseases in the Pediatric Surgery Ward of the Ahvaz Jundishapur University hospitals during the period 1993–1996 and 2002–2005

Introduction and aim The neonatal period is a critical time for infants, and 2/3 of first-year mortality occurs during this period. In developing countries, surgical diseases are the most important cause of neonatal mortality. Mortality was reduced in the past decade due to the availability of well-trained pediatric surgeons and the development of sophisticated instruments. The aim of this study was to show neonatal mortality and other indices in the period when there were no pediatric surgeons compared with the period when pediatric surgeons were available. Patients and methods This retrospective study was conducted in the Imam Khomeini and Abuzar Hospital during the period 1993–1996 and 2002–2005. All neonates admitted in the surgical wards of the Imam Khomeini and Abuzar Hospital were included in this study. Only cases of those patients who died before data accumulation was completed or discharged against medical advice were excluded from our study. In the first period, no pediatric surgeon was available and all surgeries were performed by general surgeons. In the second period, pediatric surgeons were available. Data were statistically analyzed and χ 2 -test was used to compare noncategorical data. A P value of less than 0.05 was considered to be significant. This study was approved by the ethics committee of the hospital, and no intervention was used. Results In our study, 88 cases (males = 60.2%, female =39.8%) in the first period and 452 cases (males =61.7%, females= 38.3%) in the second period were included. The mean age at admission time in the first and second period was 6 days for both groups. The mean weights in the first and second period were 2886 ±766 g and 2915± 658 g, respectively. The mean lengths of hospital stay in the first and second period were 5 days and 10.5 days, respectively. In the first period, 68 cases (37.3%) were admitted in the first 7 days of life and 54 cases (61.4%) had a birth weight of 2500–3500 g. Of the 88 cases, 40 cases died (45.5%). In the second period, 353 cases (78.1%) were admitted in the first 7 days of life and 278 cases (61.5%) had a birth weight of 2500–3500 g. Of the 452 cases, 101 cases died (22.3%). Conclusion In our study, the most common surgical diseases in both the periods were imperforate anus, esophageal atresia with or without tracheoesophageal fistula (TEF), Hirschsprung’s disease, and jejunoileal atresia; these were similar to results found in other studies. Keywords: Hirschsprung’s disease, imperforate anus, inguinal hernia, pediatric, surgery

Exploratory laparotomy for acute intestinal conditions in children: A review of 10 years of experience with 334 cases

The aim of this study was to review 10 years of experience in the management of children with acute intestinal conditions requiring exploratory laparotomy. This retrospective study included 334 children (244 boys and 90 girls) who underwent laparotomy for acute intestinal conditions between Jan 1, 2000 to Dec 31, 2009. Patients were grouped into two categories: group A (n = 44) included patients who needed laparotomy with terminal ileostomy and group B (n = 290) included patients who needed laparotomy without terminal ileostomy. We excluded neonates and patients with jejunoileal and colonic atresias, anorectal malformations, congenital pouch colon, neonatal necrotising enterocolitis, Hirschsprung's disease, appendicitis, abdominal trauma and gastrointestinal tumours. During the last 10 years, 334 laparotomies were performed in children under 12 years: 59.88% for intestinal obstruction and 40.11% for perforation peritonitis. Causes in order of frequency were: ileal perforations 34.13%; intussusceptions 26.34%; Meckel's obstruction 10.17%; congenital bands and malrotation 6.88%; postoperative adhesions 5.98%; miscellaneous peritonitis 5.68%; miscellaneous intestinal obstructions 4.79%; abdominal tuberculosis 4.19% and roundworm intestinal obstruction 1.79%. Ileostomy closures (n = 39) was tolerated well by all except one. The mortalities were 28 (8.38%) in group B and 6 (1.79%) in group A. The need for re-exploration not only increases the morbidity but also increases mortality as well. Diverting temporary ileostomy adds little cumulative morbidity to the primary operation and is a safe option for diversion in selected cases. The best way to further reduce the mortality is to create ileostomy at first operation.

Management of jejunoileal atresias: an experience at eastern Nepal

BackgroundIntestinal atresia is a common cause of neonatal intestinal obstruction, and management of this disease in limited setup of a developing country is very difficult.MethodsThis study is a retrospective study of patients with jejunoileal atresias and their postoperative outcome in a teaching hospital in eastern Nepal over a 5-year period.ResultsThere were 28 children (19 boys and 9 girls). 11 children (39.28%) had jejunal atresia and 17 (60.71%) had ileal atresia. Eight (28.5%) patients died, 6 were jejunal atresia (54.5%) and 2 were ileal atresia (11.7%). The most common cause of death was sepsis which occurred in 7 out of 8 cases (87.5%). The risk factors for mortality identified were leucopenia, neutropenia, delay in surgery, location of atresia and type of atresia. Jejunal atresia tended to have a higher mortality than ileal atresia, and severe types of atresia (type IIIb and IV) were more often associated with mortality than other types of atresia. The significant differences between jejunal and ileal atresia were the increased duration between presentation and surgery, longer postoperative and total hospital stay, presence of more severe atresias and an increased risk of mortality in case of jejunal atresias.ConclusionThe prognosis for this disease have definitely changed in the last few decades in developed countries but in our environment, problems like late presentation and diagnosis, lack of availability of good neonatal intensive care units and parenteral nutritional support still prevail.

Jejunoileal Atresia A study of 60 cases in children welfare teaching hospital.

Jejunoileal Atresia A study of 60 cases in children welfare teaching hospital.

Should We Be Concerned About Jejunoileal Atresia During Repair of Duodenal Atresia?

During repair for duodenal atresia, it has been emphasized that inspection of the small bowel to identify a second atresia is required. The laparoscopic approach for repair of duodenal atresia has been criticized for its limitation to perform this step. Given that duodenal atresia and jejunoileal atresias do not share common embryologic origins, we question the validity of this concern. Therefore, we conducted a multicenter retrospective review of duodenal atresia patients to quantify the incidence of jejunoileal atresia in this population. After institutional review board approval (IRB #07-12-187X), a retrospective review was conducted on all patients who have undergone duodenal atresia repair at seven institutions over the past 7-12 years. Demographics and the presence or absence of a jejunoileal atresia were recorded. Four hundred eight patients with duodenal atresia were identified. The mean gestaational age was 36.3 ± 2.9 weeks, and the mean weight was 2.5 ± 0.8 kg. Mean age at operation was 19 days (range, 1-1314). There was a 28% incidence of trisomy 21. Two patients (0.5%) were identified as having a second intestinal atresia, and both were type IIIb. One patient was diagnosed at the time of duodenal atresia repair; the other was a delayed diagnosis. Both patients did well after repair. In this, the largest series of duodenal atresia patients compiled to date, the rate of a concomitant jejunoileal atresia is less than 1%. This low incidence is not high enough to mandate extensive inspection of the entire bowel in these patients, and a second atresia should not be a concern during laparoscopic repair of duodenal atresia.

Jejunoileal atresia and cystic fibrosis: Don't miss it!

Jejunoileal atresia and cystic fibrosis: Don't miss it!

Clinical Course of Obstructive Jaundice Associated with Operated Meconium Peritonitis in Neonates

Meconium peritonitis (MP) may induce prolonged cholestasis after laparotomy. In this study, we investigated the postoperative clinical course of MP retrospectively and discuss the relationship between MP and the development of obstructive jaundice, including biliary atresia (BA). Between 1979 and 2008, 23 infants with MP underwent laparotomy at our institution. Eleven of the 23 infants (47.8%) developed obstructive jaundice postoperatively. The medical charts of these 11 infants were reviewed. The causative disease underlying MP included jejunoileal atresia in 10 and cloacal anomaly in 1. Of these 11 infants, 4 had acholic stools. Nine of the 11 improved with conservative management including an expectant approach, choleretic agents, and exchange blood transfusion. To differentiate the diagnosis from BA, open cholangiography was required in 2 cases following negative HIDA scintigraphy and a small gallbladder on ultrasonography. One of these 2 cases was diagnosed as BA and underwent hepatic portoeneterostomy simultaneously, after which the infant became jaundice free. Postoperative cholestasis after MP was a transient condition in most cases. However, ultrasonography and HIDA scintigraphy should be performed to differentiate BA in infants with MP who show prolonged jaundice with acholic stools.

The Experience of Operative Management in Jejunoileal Atresia

Purpose: Intestinal atresia is a common cause of neonatal intestinal obstruction. Recently, the survival rate has been increasing from development of prenatal diagnosis, total parenteral nutrition (TPN) and neonatal intensive care. We evaluated the complication rate and cause of mortality after operative management for jejunoileal atresia. Methods: We reviewed 62 patients (36 males, 26 females) with jejuno-ileal atresia who underwent operation from 1998 to 2007. Results: There were 37 patients with jejunal atresia and 25 with ileal atresia. The average gestational age was 256±16.6 days and birth weight was 2,824±620 g. Prenatal diagnosis was performed in 45 patients (72.6%) around gestational age 27 weeks. Within 2nd day after birth, 44 patients (71%) underwent operation. Half of the jejunoileal atresia was type Ⅲa and type Ⅰ was in 8, type Ⅱ was in 3, type Ⅲb was in 12, and type Ⅳ was in 8. The operative treatment was resection & anastomosis in 59 patients and enterotomy & web excision in 3. They started feeding at 12.4±11.5 days after operation on average. The average duration of TPN was 26.7±23.5 days, and the incidence of cholestasis was 30.6%. Hospital days averaged 36.8±26 days. Early complication occurred in 14 patients (intestinal obstruction in 5, sepsis in 4, wound problem in 3, anastomosis leakage in 1, and intraabdominal abscess in 1). Late complication occurred in 7 patients (anastomosis stricture in 4 and intestinal obstruction in 3). There was only one case of mortality due to short bowel syndrome after re-operation for adhesive ileus. Conclusion: The operation for intestinal atresia was successful and aggressive management contributed to a low mortality rate.

Individualized management of upper rectal atresia

Background/Purpose Congenital colonic atresia (CA) or stenosis is an infrequent cause of low intestinal obstruction in the neonate. Atresias can occur at any level, and the management of CA is determined by the atretic site and by the presence or absence of associated anomalies. We report our experience dealing with upper rectal atresia during a 5-year period. Methods Between January 2004 and December 2008, 3 female newborns with upper rectal atresia with or without associated anomalies were treated. Modes of clinical presentation, methods of diagnosis, associated anomalies, alternative management techniques, and clinical outcome were retrospectively analyzed. Results All 3 patients had progressive abdominal distension, bilious vomiting, and failure to pass meconium. Contrast enema showed an atresia at the upper rectum in 2 patients. At laparotomy, case 1 was found to have type III atresia of the upper rectum. Resection of the dilated portion of the proximal colon with end sigmoid colostomy was accomplished in the neonatal period followed by a transanal mucosectomy with takedown of the colostomy and a pull-through procedure at age 3 months. Case 3 had multiple jejunoileal atresias and an upper rectal atresia. The initial management was multiple resections of atretic bowel and anastomoses and an end sigmoid colostomy. The secondary procedure was a takedown of the colostomy and transanal mucosectomy with a pull-through procedure. Case 2 had type I upper rectal atresia in association with imperforate anus complicated by colon perforation during performance of a distal colostogram leading to a complicated and protracted clinical course. All the patients are currently well with voluntary bowel movements, and one has occasional soiling with follow-up of 9 months to 3 years. Conclusions Colon atresia, especially at the level of the upper rectum, is uncommon. Whether to proceed with an ostomy or to individualize the operative procedure according to the location of the atresia is still controversial. Transanal mucosectomy was a useful technique at the time of the definitive pull-through for the treatment of upper rectal atresia. In cases of upper CA associated with imperforate anus, delay in diagnosis and potential complications may result if the diagnosis of upper rectal atresia is missed.

Apple peel small bowel. Imaging case book

We have read with interest the article from Herman and Siegel related to imaging in the case of apple peel small bowel (APSB) or ‘Christmas tree deformity’. In the context of the diagnostic value of upper gastrointestinal series complemented by contrast enema for APSB, the authors described in detail the pathogenesis of this type of intestinal atresia. Although it is generally accepted that the majority of APSB cases result due to early in utero vascular accidents, APSB most likely represents a spectrum of disorders that includes hereditary phenotypes. However, the authors did not make mention of the pathogenetic role in APSB of intrauterine disruptive events such as volvulus, internal herniation and intussusceptions. In cases of jejuno-ileal atresia, such events are typically observed in single mid and low atresias. To demonstrate the pathogenetic role of late intrauterine events, we can add a unique case of APSB due to intrauterine midgut autoamputation caused by midgut volvulus. The baby was the first child of a 20-year-old mother with a history of ongoing heavy nicotine abuse. In the 26th week of gestation, small bowel obstruction due to volvulus was found by serial intrauterine ultrasounds. Owing to preterm labor, the baby was delivered by cesarean section in the 33rd week. Postnatally, the diagnosis of upper bowel obstruction was confirmed by ultrasound and plain X-ray. No air was seen distal to the proximal jejunum. At laparotomy, an apple peel-type jejunal atresia was noted 10 cm below the duodenojejunal flexure with largely dilated oral jejunum, mesenteric defect, concomitant volvulus of the distended oral jejunum and spiraled short segment terminal ileum with absence of the dorsal mesentery (Figure 1). Neither embryonic cords nor abnormal fixation of the narrow colon was observed. Surprisingly, a free-floating helical atretic bowel segment of browngreen color was found within the peritoneal fluid (Figure 2). Its intestinal origin was confirmed by histology, which revealed a

Neonatal intestinal obstruction in Zaria, Nigeria.

To study the aetiology, morbidity and mortality of neonatal intestinal obstruction. A retrospective study. Ahmadu Bello University Teaching Hospital, Zaria, Nigeria. One hundred and fifty one neonates (< or = 28 days) undergoing surgery for mechanical intestinal obstruction. The male/female ratio was 3:1 and median age at presentation was four days (range five hours-28 days). Anorectal malformation was the commonest cause, 104 (68.9%), 86.5% of which were high anomalies and 13.5% low; the median age at presentation was three days. Fifty two per cent of colostomies for the high anomalies were performed using general anaesthesia and 48% local anaesthetic, but there was an increasing use of local anaesthesia over the years. Hirschsprung's disease accounted for 11 (7.3%) of the cases, representing 20% of all patients presenting with Hirschsprung's disease to this hospital; the median age was six days and in two patients the caecum and sigmoid colon respectively had perforated; nine patients had colostomy, one caecostomy and one ileostomy (total colonic aganglionosis). Eleven (7.3%) patients had incarcerated or strangulated ingunial hernia (ten) and congenital ventral hernia (one); the hernias were repaired in all patients and three required intestinal resection for gangrene, two of which had ipsilateral testicular gangrene, necessitating orchidectomy. Intestinal atresia was the fourth common cause of obstruction ten (6.7%), eight of which were jejunoileal atresias and two duodenal and the median age was seven days; one atresia was associated with Hirschsprung's disease and had ileostomy, all other jejunoileal atresias were resected and duodenoduodenostomy was performed for the duodenal atresias. Other less common causes of neonatal intestinal obstruction were incarcerated exomphalos, malrotation, hypertrophic pyloric stenosis, annular pancreas, and idiopathic ileal volvulus and meconium ileus respectively. Postoperative complications occurred in sixteen of 95 patients (16.8%) including colostomy or ileostomy complications 11, wound infection three and anastomotic dehiscence (two). The overall mortality was 21.1%, 70% from overwhelming infection and 30% respiratory embarrassment; the mortality from the various conditions were Hirschsprung's disease 43%, intestinal atresia 40%, incarcerated exomphalos 40%, anorectal malformation 18.5% and the only patient with volvulus died. The morbidity and mortality of neonatal intestinal obstruction in this hospital has improved over previous years due largely to meticulous resuscitation before surgery but the problems of late presentation and poor neonatal intensive care facilities persist. The findings are at variance with those in developed countries.