Management and Outcomes of Intestinal Atresia - A Single Institution Experience From 1947 to 2019.

AIM OF THE STUDY: We aimed to evaluate disease characteristics, associated malformations and surgical outcomes of congenital intestinal atresia. METHODS: We identified all patients with jejunoileal (JIA) or colonic atresia (CA) treated at the Helsinki University Children's hospital during 1947-2019 and collected clinical data retrospectively from archived and electronic medical records. RESULTS: Of the 180 included patients, 156 had JIA and 24 CA. Overall survival improved markedly from 34% during 1947-1977 to 93% during 1978-2019. Rate of primary anastomosis (81% in JIA, 21% in CA) remained unchanged while early surgical complications decreased (32% vs 18%, P = 0.04) and prematurity rate increased (21% vs 45%, P = 0.002) among JIA patients over time. Around half of patients had associated, mostly gastrointestinal malformations which occurred comparably in JIA and CA. During 1978-2019, 20 (21%) JIA patients, but none of CA patients, developed short bowel syndrome. Presence of type 3b atresia (n = 16) or gastroschisis (n = 14), but not the extent of intestinal resection or surgical complications, was associated with development of short bowel syndrome without decreasing survival. Shorter primary bowel resection without tapering surgery in JIA predisposed to repeated resections due to obstructive symptoms, and prolonged duration of parenteral nutrition. CONCLUSION: Survival of patients with JIA and CA has improved remarkably over time and is currently high despite frequent prematurity and associated malformations. In patients with JIA, apple peel atresia and gastroschisis predisposed to short bowel syndrome without jeopardizing high survival rates. LEVEL OF EVIDENCE: IV.

Jejunal Atresia With a Rare Association: A Case Report and Literature Review.

Jejunoileal atresia, a common cause of neonatal intestinal obstruction, typically manifests shortly after birth. This case report highlights a rare instance of a late preterm female neonate presenting with type 4 jejunoileal atresia along with proximal rectal atresia, an exceedingly uncommon combination. Initial symptoms included bilious emesis and failure to pass meconium, leading to surgical correction of jejunoileal atresia. However, postoperative complications, including vomiting and jaundice, prompted further investigation, revealing rectal atresia during a fluoroscopic study on day 29. Subsequent surgery was required to address the rectal atresia, resulting in additional challenges such as short bowel syndrome and infection. The complexity of diagnosis and management underscores the importance of thorough evaluation of the lower gastrointestinal tract in neonates with jejunoileal atresia to prevent misdiagnosis and reduce the need for multiple surgeries. Rectal atresia, which is a very rare anorectal abnormality, in combination with jejunoileal atresia is considered an incredibly unusual, exceptionally unique case; as to our knowledge, no similar presentation had previously occurred. Prompt identification and simultaneous treatment of both conditions can help mitigate complications, minimize the risk of necrosis and perforation, and improve overall outcomes. Comprehensive management strategies that encompass thorough diagnostic evaluation and coordinated surgical interventions are crucial for optimizing the care of neonates with complex intestinal malformations, ensuring timely resolution of symptoms, and reducing long-term morbidity.

Antimesenteric sleeve tapering enteroplasty with end-to-end anastomosis versus primary end-to-side anastomosis for the management of jejunal/ileal atresia.

BACKGROUND/OBJECTIVE: Small bowel atresia commonly causes neonatal intestinal obstructions. Technical problems are associated with the surgical management of atresia using primary end-to-end anastomosis. Furthermore, the significantly dilated proximal loop may be associated with the stasis of intestinal fluid, thus increasing pressure on the anastomosis and prolonging fasting time before initiation of oral intake. This study aimed to perform antimesenteric tapering of the proximal loop using a linear stapler to reduce its diameter and facilitate anastomosis with the distal loop. METHODS: This retrospective study included 57 neonates diagnosed with jejunoileal atresia. They were categorised into two groups: Group A (n = 29), which included neonates treated using antimesenteric sleeve enteroplasty tapering using a linear stapler and Group B (n = 28), which included neonates treated at the primary end of the proximal loop to the side of the distal loop anastomosis. RESULTS: The mean operative time was 122.5 min in group A vs. 118 min in group B, and the mean duration to reach full oral intake was 17 days in group A vs. 20.2 days in group B (p = 0.03). The mean length of hospital stay was 25 and 35 days in groups A and B, respectively (p = 0.042). CONCLUSION: Tapering the proximal dilated bowel loop to achieve anastomosis with the distal loop improved the transient time, reduced stasis and its associated translocation and colonisation, and allowed for early initiation and maintenance of oral intake. All these parameters shortened the overall length of hospital stay.

Intestinal atresia and necrotizing enterocolitis: Embryology and anatomy.

The primitive gut originates at week 3 of gestation from the endoderm, with posterior incorporation of the remaining embryo layers. Wnt, Notch and TLR4 pathways have been shown to play central roles in the correct development of the intestine. The classical hypothesis for intestinal atresia development consists of failure in bowel recanalization or a vascular accident with secondary bowel reabsorption. These have been challenged due to the high frequency of associated malformations, and furthermore, with the discovery of molecular pathways and genes involved in bowel formation and correlated defects producing atresia. Necrotizing enterocolitis (NEC) has a multifactorial pathogenesis with prematurity being the most important risk factor; therefore, bowel immaturity plays a central role in NEC. Some of the same molecular pathways involved in gut maturation have been found to correlate with the predisposition of the immature bowel to develop the pathological findings seen in NEC.

Type 4 Ileal Atresia and Anorectal Malformation in a Neonate: A Rare Association.

Anorectal malformations and jejunoileal atresias are common causes of intestinal obstruction in neonates. Both have their own set of associated anomalies but it is extremely rare for the two to co-occur in the same patient. In this case report, we detail and describe this unusual incidence in a three-day-old neonate who was provisionally diagnosed with a case of simple imperforate anus. Per-op findings showed a type 4 Ileal atresia and an ileostomy was then created. Our experience stresses the importance of timely antenatal diagnosis and the presence of a high index of suspicion when encountering such patients. Both factors are key and crucial in determining the outcome and post-op course of the patient.

Outcome of Tapering Enteroplasty in Managing Jejunoileal Atresia.

Background: Jejunoileal atresia (JIA) represents a common cause of neonatal bowel obstruction. There is a discrepancy between the diameters of the proximal and the distal bowel loops and this is managed with excision or tapering of the dilated proximal bowel loop. We aim primarily to evaluate the outcome of tapering enteroplasty (TE) in managing JIA and secondarily to compare the outcome of TE to non-TE. Materials and Methods: A retrospective analysis of records of all neonatal admissions with JIA from January 2017 to December 2018 at a tertiary university children's hospital. Type and location of atresia, time to full enteral feeds, length of stay (LOS), complications, and outcome were assessed in TE and non-TE groups. Results: Forty-one patients were included in the study; 29 (70.7%) cases had jejunal atresia and 12 (29.3%) had ileal atresia. Seventeen (41.4%) patients had TE. The median days to full feeds was 19; 28 in the TE group versus 16 in the non-TE group (P = 0.022). Four (9.7%) cases needed a re-exploration because of failure to start feeds; all in the non-TE group. The median LOS was 33 days for TE versus 22 days for non-TE (P = 0.101). Twenty-one cases (51.2%) developed a wound infection and showed a significantly longer median LOS of 29.5 versus 19 days (P = 0.019). Mortality was 7 (17.1%). Conclusions: TE did not show a superior outcome when compared to resection of the dilated bowel. It was associated with longer time to reach full enteral nutrition and longer LOS.

Jejunoileal Atresia: A National Cohort Study.

Purpose: Jejunoileal atresia (JIA) is a rare disease. We aimed to determine the overall incidence of this malformation and associated malformations in a national cohort. Furthermore, we compared the treatment results of this cohort with the current literature. Methods: Data from the major health insurance company, which covers ~30% of the German population, were analyzed. All patients with ICD-10-Code Q41.1-9 (atresia of jejunum, ileum, other parts and not designated parts of the small bowel) who underwent any surgical procedure for small bowel were analyzed in a 10-year period between 2007 and 2016. Results: A total of 435 patients were included in the study. The incidence was 2.1 per 10,000 live births. The male:female ratio was 1:2. Sixty-four percent were premature, 21% had associated cardiac anomalies, 16% had abdominal wall defects, 7% had urogenital malformations, and 7% had cystic fibrosis. Sixty percent of all patients with jejunoileal atresia, 57% of patients with accompanying abdominal wall defects and 72% of patients with associated cystic fibrosis required ostomy as the initial procedure. In 25% of all patients, only one intestinal operation was coded. In 39% of patients, two operations were coded. Twelve percent of all patients required feeding gastrostomy or jejunostomy. Sixteen percent of all patients presented with liver-related complications, i.e., cholestasis or liver insufficiency. Six patients underwent an intestinal lengthening procedure (2 Bianchi, 4 STEP). In five patients, initial lengthening was performed within 1 year after the first intestinal operation. Mortality until 1 year after initial surgery was 5%. Of those who died, 88% were premature, 34% had cardiac anomalies and 16% had abdominal wall defects. None had cystic fibrosis. Patients with ostomy significantly more often needed operative central venous line or operative feeding tube. Short bowel was coded significantly more often in these patients. Conclusion: Patients with JIA present with low mortality. The rate of ostomies is higher than in literature. To give clinical recommendations for the initial surgical approach, further clinical research is needed.

Immune function and infectious complications in children with jejunoileal atresia.

OBJECTIVE: Little is known about differences in immune function among children with multiple intestinal atresia (MIA) and those with isolated intestinal atresia (IA), and how such differences may manifest as infectious complications and patient outcomes. This study aimed to investigate the immune function and its impact on patient outcomes in IA and MIA children. METHODS: A single-center retrospective cohort study included children aged 0-19 years with intestinal atresia who were referred to a multidisciplinary intestinal rehabilitation program from 1/2000 to 12/2016. Data were collected for patient characteristics, surgical history, immunologic work-up, and infection-related hospitalizations. Groups of IA and MIA children were compared using chi-square test or Fisher's exact test for categorical variables and using Mann-Whitney test for continuous variables, as appropriate. RESULTS: Twenty-seven children (18 IA, 9 MIA) were included. More than half of the patients had low CD counts for age in IA and MIA groups: CD3 58.3% vs. 66.7% (p = 1.0), CD4 50.0% vs. 66.7% (p = 0.7), CD8 67.7% vs. 88.9% (p = 0.3), respectively. Six out of 12 IA children and 3 out of 8 MIA children had hypogammaglobulinemia (p = 0.7). Three out of 10 IA patients and 3 out of 5 MIA children had frequent bacteremia (≥5/year). Eight children (6 IA and 2 MIA) underwent intestinal and/or liver transplant; MIA children had a worse posttransplant outcome. CONCLUSIONS: IA children may have an immunodeficiency and associated infectious complications requiring hospitalization. We suggest performing immunologic evaluation not only in MIA but also in IA children presenting to an intestinal rehabilitation program to identify immunodeficiency. Early immunodeficiency screening may help initiate appropriate intervention and improve patient outcomes. LEVEL OF EVIDENCE: Level III.

Novel insights into the histology of jejunoileal atresia and its therapeutic implications.

AIM: To study the histology of resected specimens of jejunoileal atresia (JIA) and to explore its probable therapeutic implications. METHODS: Biopsies were taken from the resected specimens of 32 patients of JIA. Sections were taken at the atretic ends and successively at every 5 cm in resected proximal bowel. They were stained for light microscopy and immunohistochemistry for CD117 (Interstitial cell of Cajal), α smooth muscle antibody (SMA), neuron filament protein (NFP) and calretinin. Histological findings on light microscopy and immune reactivity intensity for NFP, calretinin and α-SMA were recorded. Time taken to achieve full enteral nutrition (FEN; defined as attainment of oral feeds at 100 ml/kg/day without any intravenous fluid supplementation) was recorded and correlated with CD117 count by non-parametric Spearman rank correlation coefficient. RESULTS: Light microscopy failed to detect any abnormality in majority (n = 27, 84%) of JIA specimens. Mucosal ulceration (7.8%), submucosal inflammatory cells (8%), focal muscle thinning (2.5%), decreased ganglion cells and nerve fibers (3.8%) were noted on light microscopy. However all of them had normal α SMA, calretinin and NFP immunoreactivity. Mean ICC counts at proximal and distal atretic segment were 6.56 ±â€¯3.79 and 8.37 ±â€¯3.21 per HPF respectively. Serial sections in proximal dilated segment did not reveal any definite or well demarcated increase in ICC counts. CD117 counts at the proximal cut end of atresia was less than 6 per high power field (hpf) in 15 patients (46.8%) while at the site of anastomosis a count of more than 6/hpf was observed in 73% patients. There was no direct correlation of ICC counts with attainment of FEN. CONCLUSION: Absence of major morphological abnormalities in the proximal dilated bowel contradicts the findings of earlier studies on histology of JIA in smaller cohorts. Our observations reiterate that bowel plication rather than massive bowel resection should be the preferred management in babies with JIA. LEVEL OF EVIDENCE: Prognosis study (Level II).

Comparison of outcomes following three surgical techniques for patients with severe jejunoileal atresia.

BACKGROUND: Severe jejunoileal atresia is associated with prolonged parenteral nutrition, higher mortality and secondary surgery. However, the ideal surgical management of this condition remains controversial. This study aimed to compare the outcomes of patients with severe jejunoileal atresia treated by three different procedures. METHODS: From January 2007 to December 2016, 105 neonates with severe jejunoileal atresia were retrospectively reviewed. Of these, 42 patients (40.0%) underwent the Bishop-Koop procedure (BK group), 49 (46.7%) underwent primary anastomosis (PA group) and 14 (13.3%) underwent Mikulicz double-barreled ileostomy (DB group). Demographics, treatment and outcomes including mortality, morbidity and nutrition status were reviewed and were compared among the three groups. RESULTS: The total mortality rate was 6.7%, showing no statistical difference among the three groups (P = 0.164). The BK group had the lowest post-operative complication rate (33.3% vs 65.3% for the PA group and 71.4% for the DB group, P = 0.003) and re-operation rate (4.8% vs 38.8% for the PA group and 14.3% for the DB group, P < 0.001). Compared with the BK group, the PA group showed a positive correlation with the complication rate and re-operation rate, with an odds ratio of 4.15 [95% confidence interval (CI): 1.57, 10.96] and 12.78 (95% CI: 2.58, 63.29), respectively. The DB group showed a positive correlation with the complication rate when compared with the BK group, with an odds ratio of 7.73 (95% CI: 1.67, 35.72). The weight-for-age Z-score at stoma closure was -1.22 (95% CI: -1.91, -0.54) in the BK group and -2.84 (95% CI: -4.28, -1.40) in the DB group (P = 0.039). CONCLUSIONS: The Bishop-Koop procedure for severe jejunoileal atresia had a low complication rate and re-operation rate, and the nutrition status at stoma closure was superior to double-barreled enterostomy. The Bishop-Koop procedure seems to be an appropriate choice for severe jejunoileal atresia.

An Atypical Variant of Apple Peel Atresia: Reporting a Rare Case.

Apple peel intestinal atresia is a rare congenital malformation. It consists of a proximal jejunum ending in a blind pouch and distal small bowel wrapped around its vascular supply in a spiral fashion. A combination of type IIIb jejunoileal atresia (apple peel atresia) and type IV (multiple intestinal atresias) is a rare entity. The diagnosis and management of such complicated cases is a challenge, especially in resource-limited settings. We report a case of a four-day-old female who presented to the neonatal intensive care unit with complaints of vomiting, yellow discoloration of the skin, and failure to pass meconium since birth. The baby was born preterm (34 weeks) via spontaneous vaginal delivery. The physical examination concluded a jaundiced and dehydrated child with a soft, non-tender abdomen and absent gut sounds. X-ray abdomen showed two air-fluid areas in the left hypochondrium. The upper gastrointestinal gastrografin study revealed that contrast opacified the third part of the duodenum and no contrast was observed beyond it. On exploratory laparotomy, proximal jejunal atresia with four distal atresias in apple peel fashion and a viable 20 cm of small bowel was observed. The apple peel segments were supplied by mesenteric vessels. Unfortunately, our patient expired despite all supportive measures. The case highlights the significance of the prenatal and early postnatal diagnosis of such a complex combination of intestinal atresias for adequate and timely management.

Predictive Factors for Postoperative Outcome in Children with Jejunoileal Atresia.

Background Jejunoileal atresia is a relatively rare congenital gastrointestinal requiring surgery and long postoperative care. The postoperative outcome is affected by many factors and this study focuses on finding predictors for time to full enteral feeding, length of hospital stay (LOH), and postoperative complications. Methods This was a retrospective study of all children operated for isolated jejunoileal atresia between 2001 and 2017 at a tertiary center of pediatric surgery. Independent variables regarding demographical-, operative-, and postoperative data were abstracted. Primary outcome was time to full enteral feeding, LOH, and postoperative complications in terms of reoperation or central line complication. Any significant variables from the univariate analysis were further analyzed with logistic regression and presented as odds ratio with 95% confidence interval. Results After exclusion because of concomitant gastroschisis ( n = 1), and death before discharge ( n = 2), 47 patients were further analyzed (49% boys, 53% premature). No significant differences could be seen in the univariate analysis between children with short and long time (median > 17 days) to full enteral nutrition. Patients with longer LOH (median >32 days) had significantly lower birth weight compared with those with shorter LOH; median 2,550 g versus 2,980 g ( p = 0.04). Patients with a central line complication had significantly longer median time to full enteral feeding (median 27 vs. 12 days, p = 0.03), and significantly longer median LOH (median 43 vs. 21 days, p = 0.03), but these parameters were not significant in a multivariate analysis. No significant results were found regarding reoperation. Conclusion Low birth weight seems associated with an increased LOH in children operated on for jejunoileal atresia, and central line complications seem related to the duration with central line in this group. The small cohort may constitute a power problem in this study and further research regarding the included variables may reveal more potential predictors for the postoperative outcome.

A Pilot Study on Histopathology of the Jejunoileal Atresia-Can it Be Used as a Guide to Determine the Length of Adequate Resection?

INTRODUCTION: Some studies reported that there is abnormality in the histopathology of atretic bowel in jejunoileal atresia (JIA). We have made an attempt to assess sequential histopathologic changes in the resected atretic segment. MATERIAL AND METHODS: The histopathology of the resected segment was evaluated at 1, 3, 5, 7, 9, and 11 cm from atretic end (Sections A to F, respectively). The ratio of inner and outer muscle layer (measured by NIS-Element D software) was calculated at every section. Immunohistochemistry for α-smooth muscle actin (α-SMA) was also done. The findings were compared with control. RESULTS: In control set (n = 5), the ratio of inner and outer muscle layer was 1.03. In patients with JIA, the ratio was 0.68 to 0.9 at section A. This ratio varied at various sections in all specimens. In section F, this ratio was 0.95 to 1.09, which is close to control ratio. There were no specific findings related to α-SMA staining. CONCLUSIONS: It appears that the bowel proximal to the atresia is abnormal for a varied length. It may be a possibility that this abnormality is present at least up to about 10 cm proximal to atresia. Adequate resection is important for optimal outcome.

A new variant of type III jejunoileal atresia.

Jejunoileal atresia (JIA) is a congenital defect that can result in significant loss of bowel length. The traditional classification of JIA was first proposed by Grosfeld and includes 4 subtypes. Among these, type IIIB, or apple-peel atresia, is characterized by a proximal atretic jejunum and a distal segment of spiraled bowel that terminates at the cecum. Owing to this anatomy, patients with type IIIB JIA are at increased risk for short bowel syndrome and intestinal failure. In this report, we described the case of a neonate with a prenatal diagnosis of JIA. At exploration, she was initially found to have a type IIIB atresia. However, instead of terminating at the cecum, the distal spiraled segment was followed by 75 cm of normal small bowel and mesentery. Surgical correction proceeded with minimal resection and primary anastomosis. She recovered well from this procedure, tolerated full enteral nutrition by mouth, and displayed good weight gain at outpatient follow-up. Owing to the unique anatomy of the gastrointestinal tract in this case report, we propose the addition of a new class of JIA, type IIIC, to better reflect its prognostication and surgical management.

Is the Bishop-Koop procedure useful in severe jejunoileal atresia?

PURPOSE: The aim of this study was to report our experience using the Bishop-Koop procedure for the treatment of various surgical problems of jejunoileal atresia including luminal discrepancy, complex meconium peritonitis, type IIIb and type IV atresia which we defined as severe jejunoileal atresia. METHODS: This retrospective study was performed on the patients with severe jejunoileal atresia who underwent Bishop-Koop procedure at a tertiary center in China over a five year of period. The mortality, complication rate, nutrition status and the risk factors for postoperative adverse outcomes were explored. RESULTS: A total of 41 neonates underwent the Bishop-Koop procedure. The median duration of the hospital stay and total parenteral nutrition and the point at which oral feeding was initiated postoperatively were 24 days (95% CI =18.99-29.01), 13 days (95% CI = 9.03-16.97) and 11 days (95% CI = 10.17-11.83) respectively. The mortality rate was 7.32% (3/41). The complication rate was 41.4% (17/41) including anastomotic leak, intestinal obstruction, high output stoma and cholestasis. The weight for age Z-score at stoma closure was restored to normal levels (-0.86, 95% confidence interval (CI) = -1.44, -0.28). The main factor associated with adverse outcomes in severe jejunoileal atresia was premature delivery (odds ratio (OR) = 4.44, 95% CI = 1.06-18.67). CONCLUSIONS: Bishop-Koop procedure appears to be a technically efficient method for severe jejunoileal atresia, although larger studies are needed to compare Bishop-Koop procedure and other operation techniques. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: Level IV.

Neonatal intestinal obstruction associated with situs inversus totalis: two case reports and a review of the literature.

BACKGROUND: The association of neonatal intestinal obstruction with situs inversus totalis is extremely rare with only few cases reported in the literature to date. This association poses dilemmas in management. We present two such cases (of Indian origin), and briefly discuss the pertinent literature and measures to prevent unfavorable outcome. CASE PRESENTATION: Case 1: a 1-month-old preterm (1300 g) male neonate belonging to Hindu (Indian) ethnicity presented with recurrent bile-stained vomiting, non-passage of stools and epigastric fullness. A babygram and upper gastrointestinal contrast studies revealed situs inversus and suggested proximal jejunal obstruction with midgut volvulus. Exploration confirmed situs inversus totalis along with reverse rotation and midgut volvulus. There was a small gangrenous area in the proximal jejunal loop. A Ladd's procedure, resection of the gangrenous jejunal loop, and jejunojejunal anastomosis was performed. Note was made of the unusual appearance of the intestines suggestive either of fibrous or fatty infiltration. Postoperatively, our patient developed septicemia and died. Case 2: a 4-day-old female neonate belonging to Hindu (Indian) ethnicity, small (1320 g) for gestation, presented with history of non-passage of meconium since birth, refusal to accept feeds, and episodes of recurrent bilious vomiting with abdominal distension. A plain radiogram revealed situs inversus and proximal jejunal obstruction. Ultrasonography of her abdomen revealed renal dysplastic changes in both her kidneys. Laparotomy confirmed multiple jejunoileal atresias with situs inversus totalis. Resection anastomoses was performed for multiple atresias. Our patient passed a few pellets of meconium stools postoperatively; feeds were started gradually on the sixth day. Our patient gradually developed oliguria and renal failure, followed by respiratory distress and generalized edema requiring ventilatory support. She died later due to multiorgan failure. CONCLUSIONS: Clinicians should have high index of suspicion for malrotation with midgut volvulus or intestinal atresias in neonates of situs inversus presenting with bilious vomiting. The surgical treatment should follow the same surgical principles. In situs inversus, because of transposition of viscera, midgut volvulus may occur in an anticlockwise direction, hence derotation is performed clockwise. Prognosis was poor in our series because of low birth weight, late presentation, presence of gangrenous locus in the small bowel and development of septicemia in our first case and multiorgan fibrosis/dysplasia in our second case. Early diagnosis and timely referral is paramount for favorable outcome.

Apple-Peel Intestinal Atresia Along with Isolated Jejunal Duplication Cyst in a Newborn - An Extremely Rare Case Report and Brief Review.

Apple-peel type of intestinal atresia and non-communicating jejunal duplication cyst are rare congenital malformations. The coexistence is not reported in English literature. A five-day-old female neonate having intestinal obstruction and was found to have both the anomalies during laparotomy and was successfully managed. Being an extremely uncommon association between two congenital anomalies of gastrointestinal tract and surgical emergencies, it is reported with review of relevant literature.

Early Reoperations after Primary Repair of Jejunoileal Atresia in Newborns.

Aim: To review nine-year experience in managing jejuno-ileal atresia (JIA) by primary resection and anastomosis and identify factors associated with reoperations. Methods: From April 2006 to May 2015, all consecutive neonates who underwent bowel resection and primary anastomosis for JIA were analyzed retrospectively. Patients with temporary enterostomy were excluded. Patient demographics, types of atresia, surgical techniques, need for reoperations, and long-term outcomes were investigated. Results: A total of forty-three neonates were included, in which nineteen (44.2%) of them were preterm and fourteen (32.6%) were of low birth weight. Thirteen patients (30.2%) had jejunal atresia whereas thirty patients (69.8%) had ileal atresia. Volvulus, intussusception and meconium peritonitis were noted in 12, 8, and13 patients, respectively. Eight patients (18.6%) had short bowel syndrome after operation. Ten patients (23.3%) required reoperations from 18 days to 4 months after the initial surgery due to anastomotic stricture (n=1), adhesive intestinal obstruction (n=1), small bowel perforation (n=2) and functional obstruction (n=6). Prematurity and low birth weight were associated with functional obstruction leading to reoperation (p=0.04 and 0.01 respectively). The overall long-term survival was 97.7%. All surviving patients achieved enteral autonomy and catch-up growth at a median follow-up of 4.7 years. Conclusion: Long-term survival of JIA after primary resection and anastomosis are excellent. However, patients have substantial risk of early reoperations to tackle intraabdominal complications.

Modified Gut Anastomotic Technique in Type III and Type IV Jejunoileal Atresias.

BACKGROUND: Type III and IV jejunoileal atresias are associated with loss of significant length of the gut and can lead to short gut syndrome if further resection of proximal dilated gut is done. We modified the anastomotic technique so that proximal dilated segment of the gut is not resected as to prevent short gut syndrome. MATERIAL AND METHODS: Medical Record of patients of Type III and IV jejuno-ileal atresias managed with modified anastomotic technique in our center during 5-years was reviewed. RESULTS: Fifteen patients were managed with our modified technique. There were no anastomotic leak observed and there was 6% mortality seen in our modified technique. CONCLUSION: We found less mortality and morbidity in our technique compared to recommended techniques described in literature.

Small Bowel Congenital Anomalies: a Review and Update.

The small intestine is a complex organ system that is vital to the life of the individual. There are a number of congenital anomalies that occur and present most commonly in infancy; however, some may not present until adulthood. Most congenital anomalies of the small intestine will present with obstructive symptoms while some may present with vomiting, abdominal pain, and/or gastrointestinal bleeding. Various radiologic procedures can aid in the diagnosis of these lesions that vary depending on the particular anomaly. Definitive therapy for these congenial anomalies is surgical, and in some cases, surgery needs to be performed urgently. The overall prognosis of congenital anomalies of the small intestine is very good and has improved with improved medical management and the advent of newer surgical modalities. The congenital anomalies of the small intestine reviewed in this article include malrotation, Meckel's diverticulum, duodenal web, duodenal atresia, jejunoileal atresia, and duplications.