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Medicine and Pharmacy Reports Oct 2020We present a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a four days old female infant who presented with abdominal distension, bilious...
Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age - case report and review of the literature.
We present a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a four days old female infant who presented with abdominal distension, bilious vomiting, massive hematuria and feeding intolerance which was first interpreted as Prune Belly Syndrome (PBS), referred to our department after iatrogenic gastric and colonic perforation. Berdon syndrome or MMIHS is a rare congenital anomaly characterized by a massive enlarged bladder, distended abdomen, microcolon, functional obstruction of the gastrointestinal tract, and malrotation.
PubMed: 33225270
DOI: 10.15386/mpr-1583 -
The Indian Journal of Medical Research Nov 2020
Topics: Humans; Infant, Newborn; Meconium; Peritonitis
PubMed: 35345217
DOI: 10.4103/ijmr.IJMR_2364_19 -
Pediatrics Oct 2020Although infants with meconium ileus usually present with apparent symptoms shortly after birth, the diagnosis of meconium ileus and cystic fibrosis (CF) may be delayed,...
Although infants with meconium ileus usually present with apparent symptoms shortly after birth, the diagnosis of meconium ileus and cystic fibrosis (CF) may be delayed, awaiting newborn screening (NBS) results. We present the case of an 11-day-old term girl with delayed passage of meconium at 48 hours who had 2 subsequent small meconium stools over the following week. There was a normal feeding history and no signs of abdominal distension or distress. She then presented with an acute abdomen, decompensated shock, bowel perforation, and peritonitis, requiring multiple intestinal surgeries. Her NBS for CF was positive, and CF was ultimately confirmed with mutation analysis. Her course was complicated by prolonged parenteral feedings and mechanical ventilation via tracheostomy. The infant was managed with soy oil, medium chain triglycerides, olive oil, fish oil lipids and experienced only transaminitis without cholestasis and no chronic liver sequelae, with subsequent normalization of her transaminases without treatment. Because her only symptom was decreased stool output and NBS results were unavailable, the CF diagnosis was delayed until she presented in extremis. Delayed meconium passage and decreased stool output during the first week of life should lead to suspicion and additional evaluation for CF while awaiting NBS results. Careful monitoring is indicated to prevent serious, life-threatening complications. The use of soy oil, medium chain triglycerides, olive oil, fish oil lipids for infants requiring prolonged parenteral nutrition may also be considered proactively to prevent cholestasis, particularly for high risk groups.
Topics: Cholestasis; Cystic Fibrosis; Delayed Diagnosis; Female; Fish Oils; Humans; Infant, Newborn; Lipids; Meconium Ileus; Olive Oil; Parenteral Nutrition; Soybean Oil; Triglycerides
PubMed: 32978295
DOI: 10.1542/peds.2019-3717 -
Medicine Aug 2020
PubMed: 32872087
DOI: 10.1097/MD.0000000000022020 -
BMC Gastroenterology Jul 2020Studies of microbiota composition of infants with small intestinal ostomy due to various etiologies are limited. Here, we characterized the intestinal microbiota of...
BACKGROUND
Studies of microbiota composition of infants with small intestinal ostomy due to various etiologies are limited. Here, we characterized the intestinal microbiota of neonates with ileostomy resulting from distinct primary diseases.
METHODS
Fifteen patients with necrotizing enterocolitis, eight patients with meconium peritonitis, and seven patients with Hirschsprung's disease were included in the study. The small intestinal microbiota composition in infants with ileostomy caused by these diseases was studied.
RESULTS
Microbial diversity in neonatal ileostomy fluid was generally low, and was dominated by members of the Proteobacteria and Firmicutes phyla. At the genus level, the most abundant were Klebsiella, Escherichia-Shigella, Streptococcus, Clostridium sensu stricto 1, Enterococcus, and Lactobacillus. Streptococcus and Veillonella are related to carbohydrate metabolism and immunity, and breastfeeding can increase the proportion of these potentially beneficial bacteria. The proportion of Bifidobacterium in the breastfeeding group was higher than that in the non-breastfeeding group, and incidence of colitis and sepsis was reduced in the breastfeeding group. The proportion of Bifidobacterium increased and incidence of colitis and sepsis decreased in the breastfeeding group compared with the non- breastfeeding group, but there was no significant difference. The increase in body weight in the breastfeeding group was observed to be higher than in the non-breastfeeding group.
CONCLUSIONS
Excessive Klebsiella and Escherichia-Shigella and low abundance of Streptococcus, Veillonella and Faecalibacterium suggests that the small intestinal microbiota is in an unhealthy state after ileostomy. However, Streptococcus, Faecalibacterium, and Veillonella species were frequently present, suggesting that expansion of these bacteria might assist the development of the immune system after surgery.
Topics: Bifidobacterium; Gastrointestinal Microbiome; Humans; Ileostomy; Infant; Infant, Newborn; Microbiota; Prognosis
PubMed: 32660555
DOI: 10.1186/s12876-020-01366-0 -
Cureus Jun 2020A moderately preterm, 2.68 kg, male child was born to para 3 live 3 mother by Cesarean delivery done in view of preterm labor with fetal ascites. The baby had...
A moderately preterm, 2.68 kg, male child was born to para 3 live 3 mother by Cesarean delivery done in view of preterm labor with fetal ascites. The baby had antenatally detected ascites. The baby had distended but soft abdomen. Ultrasound abdomen showed gross ascites. X-ray of the abdomen in supine showed faint lucency in the mid-abdomen region posterior to the bowel gas, which was visualized as free gas along the right half of the abdomen in lateral decubitus position, suggestive of bowel perforation. Laparotomy was done on day three of life, intraoperatively found to have perforated Meckel's diverticulum. Ascites resolved postoperatively. Isolated fetal ascites is a rare condition but has a favorable prognosis.
PubMed: 32642348
DOI: 10.7759/cureus.8433 -
The Journal of Surgical Research Nov 2020There is no clear consensus on the optimal operative management of premature infants with surgical necrotizing enterocolitis (sNEC) or spontaneous intestinal perforation...
BACKGROUND
There is no clear consensus on the optimal operative management of premature infants with surgical necrotizing enterocolitis (sNEC) or spontaneous intestinal perforation (SIP); thus, a protocol was developed to guide surgical decision making regarding initial peritoneal drainage (PD) versus initial laparotomy (LAP). We sought to evaluate outcomes after implementation of the protocol.
METHODS
Pre-post study including multiple urban hospitals. Premature infants with sNEC/SIP were accrued after implementation of surgical protocol-directed care (June 2014-June 2019). Patients with a birth weight of <750 g and less than 2 wk of age without pneumatosis or portal venous gas were treated with PD on perforation. PD patients received subsequent LAP for clinical deterioration or continued meconium/bilious drainage. Postprotocol characteristics and outcomes were compared with institutional historical controls. Significance set at P < 0.05.
RESULTS
Preprotocol and postprotocol cohorts comprise 35 and 73 patients, respectively. There was a statistically significant difference in age at intervention between historical control PD (14 ± 13 d) and postprotocol PD (9 ± 4 d) groups (P = 0.01), PD patient's birth weight (716 ± 212 g versus 610 ± 141 g, P = 0.02) and estimated gestational age of LAP patients (27 ± 1.7 wk versus 31 ± 4 wk, P = 0.002). PD was definitive surgery in 27% (12 of 44) of postprotocol patients compared with 13% (3 of 23) historical controls. A trend in improved survival postprotocol occurred in all PD infants (73% versus 65%), all LAP (75% versus 70%), and for initial PD and subsequent LAP (82% versus 67%).
CONCLUSIONS
Utilization of a surgical protocol in sNEC/SIP is associated with improved success of PD as definitive surgery and improved survival.
Topics: Clinical Protocols; Drainage; Enterocolitis, Necrotizing; Female; Florida; Humans; Infant, Newborn; Infant, Premature; Intestinal Perforation; Laparotomy; Male; Postoperative Complications; Treatment Outcome
PubMed: 32615312
DOI: 10.1016/j.jss.2020.05.079 -
Ultraschall in Der Medizin (Stuttgart,... Apr 2022To describe the postnatal outcome of fetal meconium peritonitis and identify prenatal predictors of neonatal surgery. (Meta-Analysis)
Meta-Analysis
PURPOSE
To describe the postnatal outcome of fetal meconium peritonitis and identify prenatal predictors of neonatal surgery.
METHODS
We retrospectively reviewed all fetuses with ultrasound findings suspicious for meconium peritonitis at a single center over a 10-year period. A systematic review and meta-analysis were then performed pooling our results with previous studies assessing prenatally diagnosed meconium peritonitis and postnatal outcome. Prenatal sonographic findings were analyzed to identify predictors for postnatal surgery.
RESULTS
34 cases suggestive of meconium peritonitis were diagnosed at our center. These were pooled with cases from 14 other studies yielding a total of 244 cases. Postnatal abdominal surgery was required in two thirds of case (66.5 %). The strongest predictor of neonatal surgery was meconium pseudocyst (OR [95 % CI] 6.75 [2.53-18.01]), followed by bowel dilation (OR [95 % CI] 4.17 [1.93-9.05]) and ascites (OR [95 % CI] 2.57 [1.07-5.24]). The most common cause of intestinal perforation and meconium peritonitis, found in 52.2 % of the cases, was small bowel atresia. Cystic fibrosis was diagnosed in 9.8 % of cases. Short-term neonatal outcomes were favorable, with a post-operative mortality rate of 8.1 % and a survival rate of 100 % in neonates not requiring surgery.
CONCLUSION
Meconium pseudocysts, bowel dilation, and ascites are prenatal predictors of neonatal surgery in cases of meconium peritonitis. Fetuses with these findings should be delivered in centers with pediatric surgery services. Though the prognosis is favorable, cystic fibrosis complicates postnatal outcomes.
Topics: Child; Female; Humans; Infant, Newborn; Intestinal Perforation; Meconium; Peritonitis; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 32575129
DOI: 10.1055/a-1194-4363 -
Fetal magnetic resonance imaging contributes to the diagnosis and treatment of meconium peritonitis.BMC Medical Imaging May 2020Meconium peritonitis (MP) is a rare fetal disease that needs to be urgently identified for surgical intervention. We report a series of 35 patients diagnosed prenatally...
BACKGROUND
Meconium peritonitis (MP) is a rare fetal disease that needs to be urgently identified for surgical intervention. We report a series of 35 patients diagnosed prenatally with MP by magnetic resonance imaging (MRI), illustrate the imaging findings and investigate the predictive value of these findings for postpartum management.
METHOD
A consecutive cohort of patients diagnosed with MP who were born at our institution from 2013 to 2018 was enrolled retrospectively. The prenatal ultrasound and MRI findings were analyzed. Fisher's exact probability test was used to evaluate the predictive value of MRI for surgical intervention between the operative group and the nonoperative group.
RESULTS
Ascites (30/35) and distended bowel loops (27/35) were two of the most common prenatal MP-related findings on fetal MRI. Of the 35 infants, 26 received surgical intervention. All fetuses with MRI scans showing bowel dilatation (14/26, p = 0.048) and micro-colorectum (13/26, p = 0.013) required surgery. There were no significant differences in the number of fetuses with meconium pseudocysts and peritoneal calcifications between the two groups.
CONCLUSION
Fetuses with bowel dilatation and micro-colorectum on MRI may need postpartum surgical intervention. Infants with only a small amount of ascites and slight bowel distention were likely to receive conservative treatment.
Topics: Adult; Cohort Studies; Female; Fetal Diseases; Gestational Age; Humans; Infant, Newborn; Magnetic Resonance Imaging; Maternal Age; Meconium; Peritonitis; Predictive Value of Tests; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Ultrasonography, Prenatal; Young Adult
PubMed: 32448115
DOI: 10.1186/s12880-020-00453-8 -
BMC Pediatrics Apr 2020Hydrops fetalis as well as abdominal compartment syndrome (ACS) are conditions that are associated with high mortality rates. A rare case of immature gastric teratoma...
BACKGROUND
Hydrops fetalis as well as abdominal compartment syndrome (ACS) are conditions that are associated with high mortality rates. A rare case of immature gastric teratoma causing fetal hydrops and subsequent ACS is presented. The related pathophysiologic mechanisms are discussed, and the importance of timely recognition and appropriate interventions are highlighted.
CASE PRESENTATION
The male patient was born preterm, weighing 3.9 kg., by Cesarean section. Prior prenatal ultrasounds were normal, but a scan done just before delivery had findings indicating polyhydramnios, fetal ascites, and meconium peritonitis. Upon delivery, the patient had respiratory distress, anasarca and a massively distended abdomen. Resuscitation measures, including ventilatory support, were instituted. Imaging studies showed ascites as well as a large, complex intra-abdominal lesion with calcifications. In the succeeding hours, anuria persisted, anasarca worsened, the abdomen became more distended, and inotrope requirements increased. The occurrence of ACS, from what was presumed to be a retroperitoneal teratoma, was therefore considered. Laparotomy was done on the 28th hour of life, with en bloc excision of a massive tumor and attached section of the greater curvature of the stomach. Passage of urine occurred intra-operatively, and the patient was soon after weaned off inotropes and ventilator support. The histopathologic result was immature gastric teratoma. No chemotherapy was given, and the patient's serum AFP is at normal levels 15 months following surgery.
CONCLUSION
The presence of a massive intra-abdominal lesion can result in the pathophysiologic continuum of hydrops fetalis and neonatal ACS. The early recognition of such an association can enable appropriate expectant management of similarly affected neonates, including emergent decompression laparotomy.
Topics: Cesarean Section; Female; Humans; Hydrops Fetalis; Infant, Newborn; Intra-Abdominal Hypertension; Male; Polyhydramnios; Pregnancy; Teratoma
PubMed: 32340629
DOI: 10.1186/s12887-020-02090-0