Complicated Meconium Ileus Research Articles

Diagnosis and Management of Simple and Complicated Meconium Ileus in Cystic Fibrosis, a Systematic Review.

The early management of neonates with meconium ileus (MI) and cystic fibrosis (CF) is highly variable across countries and is not standardized. We conducted a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. The protocol was registered in PROSPERO (CRD42024522838). Studies from three providers of academic search engines were checked for inclusion criteria, using the following search terms: meconium ileus AND cystic fibrosis OR mucoviscidosis. Regarding the patient population studied, the inclusion criteria were defined using our predefined PICOT framework: studies on neonates with simple or complicated meconium which were confirmed to have cystic fibrosis and were conservatively managed or surgically treated. Results: A total of 566 publications from the last 10 years were verified by the authors of this review to find the most recent and relevant data, and only 8 met the inclusion criteria. Prenatally diagnosed meconium pseudocysts, bowel dilation, and ascites on ultrasound are predictors of neonatal surgery and risk factor for negative 12-month clinical outcomes in MI-CF newborns. For simple MI, conservative treatment with hypertonic solutions enemas can be effective in more than 25% of cases. If repeated enemas fail to disimpact the bowels, the Bishop-Koop stoma is a safe option. No comprehensive research has been conducted so far to determine the ideal surgical protocol for complicated MI. We only found three studies that reported the types of stomas performed and another study comparing the outcomes of patients depending on the surgical management; the conclusions are contradictory especially since the number of cases analyzed in each study was small. Between 18% and 38% of patients with complicated MI will require reoperation for various complications and the mortality rate varies between 0% and 8%. Conclusion: This study reveals a lack of strong data to support management decisions, unequivocally shows that the care of infants with MI is not standardized, and suggests a great need for international collaborative studies.

A remarkable case of intrauterine intussusception, ileal atresia, and complicated meconium ileus: A case report

Background: Intrauterine intussusception, although very rare (<2%), is a potential cause of intestinal atresia. It usually manifests as bowel obstruction during the first hours or days of life. Case Presentation: We report a case with fetal ascites prompting early Cesarean section, with subsequent percutaneous abdominal drainage and laparotomy. Intraoperative findings showed meconium peritonitis and type IIIa ileal atresia with intussusception of the distal part, with the necrosed intussusceptum likely causing perforation and ileal atresia; in addition, the distal ileum demonstrated signs of meconium ileus. After ileostomy creation, the postoperative course was uneventful. Cystic fibrosis was excluded via a sweat test. Conclusion: Prompt diagnosis and management of neonatal intestinal obstruction secondary to the unusual combination of fetal ascites, intussusception, complicated meconium ileus, and ileal atresia results in a favorable outcome.

Sutured Versus Sutureless Enterostomies: An Alternative Technique in Critically Sick Neonates.

Background and objectiveIntestinal perforations and necrotizing enterocolitis (NEC) requiring the formation of temporary intestinal stoma are prevalent conditions worldwide. This prospective study aimed to address the following research question: does sutureless enterostomy lead to fewer complications compared to conventional enterostomy in critically sick surgical neonates who need a stoma?MethodsWe conducted a randomized control trial (TCTR20211011004) from October 2020 to October 2021. A total of 120 patients were randomized to the following two study groups: Group A: conventional sutured enterostomy; Group B: sutureless enterostomy, with 60 patients in each group. The operative time, complications, and mortality were compared between the two groups using the chi-squared test.ResultsThe groups were comparable with respect to the mean age at presentation, gestational age, weight, and sex ratio. In Group A, the main operative diagnoses were NEC in 22 patients, pneumoperitoneum in 25, complicated meconium ileus in nine, bowel atresia in three, and midgut volvulus in one; while in Group B, 25 patients had NEC, 20 had pneumoperitoneum, eight had meconium ileus, two had bowel atresia, and five had midgut volvulus. The mean operative time (93.5 ± 28.71 minutes) was significantly longer in the conventional group compared to the sutureless group (52.08 ± 18.53 minutes). Oral feed was started significantly earlier in the sutureless stoma group. Mortality was 43.3% in Group A and 46.6% in Group B. Complication rate was significantly higher in sutured stoma group.ConclusionThe management of critically sick neonates, especially those with NEC and intestinal perforation, still presents a surgical dilemma. Based on our findings, the sutureless enterostomy may reduce operative time and complications in critically sick neonates.

Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study

BackgroundContemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management. MethodsA prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012–September 2014. Live-born infants with bowel-obstruction from inspissated meconium in the terminal ileum and CF were reported. Data are described as median (interquartile range, IQR). Results56 infants were identified. 14/56(25%) had primary laparotomy (13/23 complicated MI, 1/33 simple), the remainder underwent contrast enema. Twelve, (12/33 (36%) with simple MI) achieved decompression. 8/12 (67%) who decompressed had >1 enema vs 3/20 (15%) with simple MI who had laparotomy after enema. The number of enemas per infant (1–4), contrast agents and their concentration, were highly variable. Enterostomy was formed at 24/44(55%) of laparotomies. In infants with simple MI, time to full enteral feeds was 6 (2–10) days in those decompressing with enema vs 15 (9–19) days with laparotomy after enema. Case fatality was 4% (95% CI 0.4–12%). Two infants, both preterm died, both in the second month after birth. ConclusionsInfants with simple MI achieving successful enema decompression were more likely to have had repeat enemas than those who proceeded to laparotomy. Successful non-operative management was associated with a shorter time to full feeds. The early management of infants with MI is highly variable and not standardised across the UK and Ireland.

Bedside laparotomy in newborns -A single institute experience

Background/purpose: Intra hospital transfer of sick newborns is known to cause adverse events with potential morbidity. Interventions at the bedside in a sick neonate can reduce the need for transport and in turn, potential hazards of transfer. Our single institute experience of performing bedside laparotomies in unstable newborns is reported here. Materials and methods: Seven-year data was collected from electronic medical records. This was a retrospective comparative study with level III evidence. Twenty-eight neonates operated at bedside for intraabdominal sepsis due to Necrotising Enterocolitis (NEC), Spontaneous Intestinal Perforation (SIP), complicated meconium ileus and perforation secondary to atresias were included Group A. Group B had 60 neonates operated for similar indications in the conventional operation theatres. Results: The average corrected gestational age at surgery, associated co-morbidities, average volume of blood loss and duration of surgery were compared between the groups. Group A had lower weight at surgery (1098 vs 1872 gs), greater percentage of neonates on inotropic support (78% vs 20%) with requirement of High Frequency Ventilation (HFO) (50% vs none). A quarter of neonates (7 of 28) in Group A had NEC Totalis as against only one case in group B. There was 25% survival in group A and 76.67% in group B. The lower survival in group A can be attributed to lower weight at surgery, higher inotrope requirement and need for unconventional modes of ventilation. Conclusion: Bedside laparotomy is a feasible option in unstable neonates deemed unsuitable for transport.

Management of Meconium Ileus: 5 years' experience at Dhaka Shishu (Children) Hospital

Background : Meconium ileus (MI) is one of the common cause of intestinal obstruction in neonate. It can be present with various complications (about 50%) like volvulus, atresia, and gangrene of the gut, perforation and meconium cyst.
 Objective : This study aimed to compare various surgical procedures used in the treatment of meconium ileus and to assess their efficacy regarding survival and complications in our center.
 Methods : This retrospective study was done to all cases of meconium ileus admitted in the Department of neonatal surgery of Dhaka Shishu (Children) Hospital during the past 5 years (2011 to2016). The medical records of all patients with meconium ileus were studied. The surgical procedures were Mikulicz procedure, Bishop-Koop procedure done in case of complicated cases and Mikulicz procedure, Bishop-Koop and T tube ileostomy done in uncomplicated cases. Outcomes were compared between complicated and uncomplicated group and between the surgical procedures.
 Results : Total patients were 224. Among them 8 were excluded due to incomplete data. The mean age was 3.23 days. Twenty four neonates were preterm. Fifty-two percent were uncomplicatedMI and 48.15% were complicated. Among 112 uncomplicated cases, Mikulicz procedure done in 33 cases, Bishop-Koop procedure done in 37 cases and rest were treated by T tube ileostomy procedures. Among 104 cases of complicated meconium ileus, Mikulicz procedure done in 75 cases and 42 cases were treated by Bishop-Koop stoma. Predominant complications in Mikulicz procedures were high output fistula (67.6%), sepsis (34.84%) and skin excoriation (58.33%), while in Bishop-Koop procedure were sepsis (59.25%), anastomotic leak and reoperation (25.75%). In T tube ileostomy, complications were intra-peritoneal leak with reoperation and sepsis (9.52%). Twenty five (11.6%) stoma prolapse were found in Mikulicz ileostomy. The overall mortality of meconium ileus was 36.6%, in simple MI 23 out of 112 and in complicated MI 56 out of 104. This difference was significant.
 Conclusion : Considering the study result we concluded that complications occur more frequently in Mikulicz procedure and it is significantly associated with mortality, Bishop- Koop ileostomy can be considered but anastomotic leak is still an important complication of this procedure. In uncomplicated cases T tube ileostomy found as the best option.
 Northern International Medical College Journal Vol.10(1) Jul 2018: 326-329

Management Strategy of Meconium Ileus-Outcome Analysis.

Background:Meconium ileus (MI) is defined as an intestinal obstruction caused by the impaction of inspissated meconium in the terminal ileum. In this study, we have evaluated the nonoperative management of patients of simple MI without fluoroscopic support –an important requisite of the Noblett's criteria. Besides this, surgical management in cases of failed conservative management and complicated MI was also assessed.Materials and Methods:This was a retrospective observational study. Various clinical and radiological parameters were evaluated. Conservative management included the use of water-soluble contrast diatrizoate meglumine and diatrizoate sodium. In case of nonpassage of meconium in 24 h from first intervention, exploratory laparotomy with ileostomy was performed. All complicated MI underwent exploratory laparotomy with creation of stoma as and when needed.Results:The duration of this study was 6½ years. Twenty-five neonates of MI were admitted. Of these, 22 had simple MI and remaining three had complicated MI. Eighteen neonates responded to the conservative management. In four neonates, who did not respond, exploratory laparotomy was performed. All three neonates having complicated MI underwent exploratory laparotomy. One patient expired in follow-up.Conclusion:MI is an important neonatal emergency, which needed immediate attention of a pediatric surgeon. Proper evaluation of the patient, careful application of principals of conservative management, and timely surgical intervention may fetch satisfactory results.

Recent Change in Management Pattern of Meconium Ileus at Dhaka Shishu Hospital

Background : Meconium Ileus (MI) accounting for 9–33% of neonatal intestinal obstruction and one of the common cause of intestinal obstruction in neonate. Near about 50% can be present with various complications. Options for surgical management of MI include resection of dilated ileum with primary anastomosis, Bishop–Koop ileostomy, Santulli procedure or Mikulicz procedure and T tube ileostomy. In our center, before January 2015, mostly Mikulicz and few Bishop- Koop procedures were done for MI. But, the complications were high. From 2015, we started to perform T tube ileostomy and Bishop-Koop procedure in simple MI. In complicated cases Mikulicz and Bishop-Koop procedure were done and the scenario changed. This study was done to compare the outcome of MI surgery before and after January 2015.Methods: This was a retrospective study done in our center during January 2011 to December, 2016 (5 years). All cases were divided into two groups (Group-A: January 2011 to December 2014 and in Group-B: January 2015 to December 2016). Demographic data and surgical outcomes were compared.Results: Total 216 cases were included, in Group-A: 123 patients and others were in Group- B. Simple MI were 49% in Group-A and 56% were in Group-B. Complicated MI presented with small gut volvulus (43.3%) gangrene of loaded ileum (32.2%) perforation with peritonitis (37.8%) intestinal atresia with adhesions (16.3%) and meconium pseudocysts in 12 cases (16%). 11 patients had more than one complication. All the postoperative complications were found significantly high in Group-A except anastomotic leakage. Mortality was also significantly high in Group-A.Conclusion: Morbidity and mortality were significantly reduced with increasing practice of T tube ileostomy and Bishop-Koop procedure in simple and complicated Meconium ileus.Chatt Maa Shi Hosp Med Coll J; Vol.16 (2); July 2017; Page 5-8

Fetal MRI in management of complicated meconium ileus: Prenatal and surgical imaging.

To review fetal MRI cases surgically proven to have meconium ileus (MI) and obstruction, describe the common fetal MRI findings that distinguish cases of complicated MI, and to compare these findings with surgical images and perinatal outcomes. We performed a retrospective review of all fetal MRI examinations and the corresponding medical record from our tertiary care children's hospital over an 18-month period. Postnatal management and outcomes were reviewed for these patients, and those patients with surgical or postmortem diagnosis of complicated MI were included in the study. Our analysis revealed 7 cases. In this cohort, 3 imaging features of the fetal bowel were repeatedly seen: gradient appearance of intraluminal bowel contents, abnormally localized meconium signal, and collapsed appearance of the colon on MRI. Surgical diagnoses confirmed MI. All live-born infants underwent surgical repair. Fetal MRI should be included in the diagnostic algorithm of any pregnancy where fetal bowel obstruction is suspected to better risk stratify patients.

Complicated Meconium Ileus: Case Report with Review of Literature

Complicated Meconium Ileus: Case Report with Review of Literature

Meconium obstruction in absence of cystic fibrosis in low birth weight infants: an emerging challenge from increasing survival

BackgroundMeconium abnormalities are characterized by a wide spectrum of severity, from the meconium plug syndrome to the complicated meconium ileus associated with cystic fibrosis. Meconium Related Ileus in absence of Cystic Fibrosis includes a combination of highly viscid meconium and poor intestinal motility, low grade obstruction, benign systemic and abdominal examination, distended loops without air fluid levels. Associated risk factors are severe prematurity and low birth weight, Caesarean delivery, Maternal MgSO4 therapy, maternal diabetes. In the last 20 yrs a new specific type of these meconium related obstructions has been described in premature neonates with low birth weight. Its incidence has shown to increase while its management continues to be challenging and controversial for the risk of complicated obstruction and perforation.Materials and methodsAmong 55 newborns admitted between 1992-2008 with Meconium Related Ileus as final diagnosis, data about Low Birth Weight infants (LBW < 1500 g) were extracted and compared to those of patients ≥ 1500 g. Hischsprung's Diseases and Cystic Fibrosis were excluded by rectal biopsy and genetic probe before discharge. A softening enema with Gastrografin was the first option whenever overt perforation was not present. Temporary stoma or trans appendiceal bowel irrigation were elected after unsuccessful enema while prompt surgical exploration was performed in perforated cases. NEC was excluded in all operated cases. Data collected were perinatal history and neonatal clinical data, radiological signs, clinical course and complications, management and outcome.Results30 cases with BW ≥ 1500 g had an M/F ratio16/14, Mean B.W. 3052 g, Mean G.A. 37 w Caesarean section rate 40%. There were 10 meconium plug syndrome, 4 small left colon syndromes, and 16 meconium ileus without Cystic Fibrosis. Five cases were born at our institution (inborn) versus 25 referred after a mean of 2, 4 Days (1-7) after birth in another Hospital (outborn). They were managed, after a Gastrografin enema with 90% success rate, by 1 temporary Ileostomy and 2 trans appendiceal irrigation. 25 cases with BW< 1500 g (LBW) had M/F ratio 11/14, Mean B.W. 818 g, Mean G.A. 27 w, Caesarean section rate 70%, assisted ventilation 16/25. There were 8 inborn and 17 outborn. Gastrografin enema was successful in 6 out 8 inborn infants only, all referred within one week from birth. There were 12 perforations mainly among late referred LBW outborn.ConclusionsMeconium Related Ileus without Cystic Fibrosis responds to conservative management and softening enema in most of mature infants. In LBW clinical course is initially benign but as any long standing bowel obstruction management may present particular challenges. Clinical and plain radiographic criteria are reliable for making diagnosis and testing for Cystic Fibrosis may not be indicated. Enema may be resolutive when performed in a proper environment. Perforated cases may be confused with NEC which is excluded by clinical history, no signs of sepsis, lab signs missing, abdominal signs missing, typical radiological signs missing. The higher complication rate is recorded among cases delivered and initially managed in Neonatal Units without co-located Surgical Facilities. Early diagnosis and aggressive medical therapy may lead to higher success rate and help avoiding surgical interventions. Surgical therapy in uncomplicated cases, unresponsive to medical management, should be minimally aggressive.

Tapering Enteroplasty for Complicated Meconium Ileus

Meconium ileus (MI) presenting with complications such as volvulus, atresia, necrosis, perforation, peritonitis, or giant cystic meconium peritonitis demands operative intervention and often requires a small bowel resection. These patients are at increased risk of short bowel syndrome if a significant portion of bowel must be resected. We report on a 1- day-old boy who was found to have a complicated MI with volvulus causing a long ischemic strip of small bowel. An enteroplasty was successfully employed to maintain bowel continuity after removing a 2 by 50 cm segmental area of ischemic bowel. Keywords: Meconium ileus, volvulus, short bowel syndrome, operative technique, cystic fibrosis, enteroplasty, meconium, upper gastrointestinal, laparotomy

Lymphocytic Leiomyositis and Myenteric Ganglionitis Are Intrinsic Features of Cystic Fibrosis: Studies in Distal Intestinal Obstruction Syndrome and Meconium Ileus

Cystic fibrosis (CF) is a multisystem disorder intrinsically associated with inflammation of mucosal surfaces. Because inflammation can result in enteric neuromuscular dysfunction we hypothesized that terminal ileitis in patients with CF may predispose to distal ileal obstruction syndrome (DIOS). Full-thickness terminal ileal tissues from 6 children with CF and severe DIOS, 6 infants with complicated meconium ileus (MI), and 6 children with non-CF intestinal atresia were studied. Lymphocyte-predominant mucosal and transmural ileal inflammation was present in 6 of 6 patients with DIOS. Lymphocytic ganglionitis was present in 4 of 6 although numbers of myenteric neurons were not decreased (5/5). Myocyte proteins were preserved (6/6). Mild submucosal fibrosis was common in DIOS (5/6) and transformation of submucosal fibroblasts to a myofibroblastic phenotype was noted in 4 of 6. Inflammatory changes were distinct from those described in fibrosing colonopathy. Antroduodenal manometry in an individual who had experienced MI/DIOS was consistent with a neuropathic pseudo-obstructive process. Submucosal or transmural lymphocyte predominant inflammation was also present in 6 of 6 infants with complicated MI, which, when coupled with submucosal myofibroblast proliferation (5/6), appeared highly predictive of CF rather than non-CF atresia. Histological findings at birth were similar, although milder, than those seen in DIOS, suggesting that these changes are a primary abnormality in CF. Submucosal or transmural inflammation of the ileum is common in newborns with CF and MI and older children with DIOS. Severe recurrent DIOS should be investigated with seromuscular and mucosal biopsy of the ileum to seek a transmural ileitis potentially amenable to anti-inflammatory therapies.

Intrauterine midgut volvulus without malrotation: Diagnosis from the ‘coffee bean sign’

Fetal midgut volvulus is quite rare, and most cases are associated with abnormalities of intestinal rotation or fixation. We report a case of midgut volvulus without malrotation, associated with a meconium pellet, during the gestation period. This 2.79 kg, 33-wk infant was born via a spontaneous vaginal delivery caused by preterm labor. Prenatal ultrasound showed dilated bowel loops with the appearance of a 'coffee bean sign'. This patient had an unusual presentation with a distended abdomen showing skin discoloration. An emergency laparotomy revealed a midgut volvulus and a twisted small bowel, caused by complicated meconium ileus. Such nonspecific prenatal radiological signs and a low index of suspicion of a volvulus during gestation might delay appropriate surgical management and result in ischemic necrosis of the bowel. Preterm labor, specific prenatal sonographic findings (for example, the coffee bean sign) and bluish discoloration of the abdominal wall could suggest intrauterine midgut volvulus requiring prompt surgical intervention.

Primary resection and anastomosis for complicated meconium ileus: a safe procedure?

Various options are available for the surgical treatment of meconium ileus (MI). This paper examines the use of resection and primary anastomosis as the favoured option for the treatment of complicated meconium ileus. This was a retrospective study. All patients (13 children) with MI treated with primary resection and anastomosis (RA) in the 10-year period (1996-2005) at St Mary's Hospital in Manchester were identified. The case notes were retrieved. The gestational age, type of surgery, length of bowel resection and complications were recorded. Out of 13 (3 males, 10 females), 7 had severely dilated bowels, 5 had perforation and 1 had volvulus. Mean length of bowel resection was 33.8 cm (range 20-50 cm). Overall survival in this group was 85%. Seven patients (54%) developed complications. Four (31%) had surgical complications: two anastomotic strictures with adhesions, one adhesive intestinal obstruction and one intra-abdominal drain retraction. Primary resection and anastomosis is a safe option in the treatment of complicated meconium ileus. It has the advantage of less hospital stay and avoids a secondary laparotomy for closure of the stoma.

Congenital intestinal anomalies, neonatal short bowel syndrome, and prenatal/neonatal counseling

Background Short bowel syndrome (SBS) is a severe malabsorption caused by bowel loss. Congenital intestinal anomalies (CIA) detectable by prenatal ultrasound as jejunoileal atresia, meconium peritonitis, complicated meconium ileus, and fetal volvulus can be responsible for SBS. Aims This study aims to investigate either frequency of SBS or the morbidity in CIA population during the first admission. Material and methods Records of CIA treated from 1997 to 2003 were reviewed. The prenatal ultrasound findings were correlated with SBS. Student's t and χ 2 tests were performed to analyze epidemiological data, growth at discharge, sepsis, liver disease, catheter-related complications, motor developmental delay, and hospital stay in CIA with and without SBS. Results Forty-four CIA: SBS developed in 43%, ranging from 83% in volvulus to 0% in complicated meconium ileus. Thirty-six prenatal diagnoses: a strong correlation with SBS was observed in isolated dilated bowel (58%). In SBS neonates, birth weight, gestational age, and growth at discharge were statistically lower, whereas sepsis, motor delay, and hospital stay were statistically higher. Conclusions Many neonates with CIA detectable by prenatal ultrasound develop SBS. Short bowel syndrome presents a significant higher morbidity. The counseling should stress the frequent association between CIA and SBS as well as the significant morbidity in SBS.

Immunoreactive trypsin levels in neonates with meconium ileus

Serum immunoreactive trypsin (IRT) is used as a screening test for cystic fibrosis (CF) in neonates in many countries. Variations in IRT levels are observed in healthy and cystic neonates within the first few weeks of life. Fifteen percentage of CF neonates present with meconium ileus (MI). We hypothesised that there may be differences in serum IRT levels in cystic babies with simple and complicated MI. The aim of this study was to investigate the serum levels of IRT in neonates with CF presenting with MI. IRT levels were sequentially measured in neonates (n = 29) with CF with intestinal obstruction due to simple or complicated MI. These were compared to levels obtained from non-cystic neonates/controls admitted with a variety of other intra-abdominal pathologies (n = 49) IRT levels were significantly higher in the CF-MI group than the non-cystic controls (P < 0.001). There was no statistical difference in IRT levels between the simple or complicated MI groups. In the MI group there was no statistical difference between those who required operation, no difference between the pre- and post-operative IRT levels and no significant relationship between IRT levels and birth weight or gestation. Serum IRT levels are significantly elevated in neonates with CF and MI compared with non-cystic, non-MI neonates. The results of this observational study highlight that a single raised level of IRT in a neonate should prompt the analysis for CF regardless of any underlying surgical pathology.

Meconium ileus: a review 1972-1990.

This article reviews the management and mortality of 53 neonates with meconium ileus and cystic fibrosis treated in one paediatric surgical centre from 1972 to 1990. Forty-four patients (83 per cent) presented with simple meconium ileus and 26 of 40 patients in this group were successfully treated with Gastrografin enemas. Four patients suffered a perforation as a result of the enema (perforation rate 5 per cent). The 1-year survival rate for simple meconium ileus was 81 per cent and for complicated meconium ileus 75 per cent.

Surgical considerations in cystic fibrosis: A 32-year evaluation of outcomes

Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature. A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival. A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax. Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.

Neonataler Aszites: Mekoniumileus mit Perforation bei Mukoviszidose

Many conditions are known associated with neonatal ascites such as cardiac and vascular malformations, as well as malformations of the brain, kidney, lung and bone, chromosomal abnormalities, infections, fetal anemias, tumors, metabolic and maternal conditions. In 30% no reason can be found. Meconium ileus is a causal gastrointestinal abnormality. We report about a preterm infant 35 weeks of gestation with complicated meconium ileus because of mucoviscidosis.