RARE COLORECTAL MALFORMATIONS IN NEWBORNS. LITERATURE REVIEW AND OWN OBSERVATIONS

The article presents literature data andown observations of some rare congenital malformationsof the colorectal area in newborns that require surgicaltreatment. These are defects such as congenital pouch colonand congenital segmental dilatation of the colon. Thereare isolated cases of birth of children with these defectsin European countries and North America. They are morecommon in Asian countries, especially in India. Diagnosisand surgical correction of such congenital malformationscause difficulties associated with insufficient awarenessof pediatric surgeons, neonatologists, pediatricians aboutthis pathology. Data is presented on clinical manifestations,antenatal and postnatal diagnosis and treatment tactics, aswell as the histopathological structure of the affected colonin these defects.The analysis of literature data and the presented clinicalobservations of congenital pouch colon and congenitalsegmental dilatation of the colon in newborns indicate thepossibility of their antenatal and preoperative diagnosis.It is concluded that children with anorectal malformationsand Hirschsprung's disease require a thorough differentialdiagnosis with congenital pouch colon and segmentaldilatation of the colon, as the tactics of their surgicalcorrection significantly differ from the treatment tacticsof common anorectal malformations and Hirschsprung'sdisease surgical management.

Ultrasonic Diagnostic Strategy for the Causes of Coronary Artery Dilatation in Infants

Objective:To explore a differential diagnosis strategy for the causes of coronary artery dilatation (CAD) in infants．Methods: Clinical and echocardiography data for 243 infants with CAD from the Shengjing Hospital of China Medical University were analyzed retrospectively. The patients were divided into congenital and acquired groups according to the CAD causes.Results: The lesion detection rate for CAD in 22,925 infants who underwent echocardiography was 1.06% (243/22,925). The acquired group accounted for 84.77% (206/243) of participants, all of which had Kawasaki disease. The congenital group accounted for 15.23% (37/243) of patients, including coronary artery fistula [12.35% (30/243)], anomalous origin of the coronary artery [2.06% (5/243)], severe pulmonary stenosis [0.41% (1/243)], and moderate aortic stenosis [0.41% (1/243)]. There was no significant difference in the Z-score for CAD between the two groups of children (P>0.05). There were differences in the scope and shape of CAD between the two groups (all P<0. 05). Acquired causes mainly manifested as segmental dilatation, while congenital causes manifested as tubular dilatation. The sensitivity and specificity of segmental dilatation in predicting acquired causes were 97.57% and 100%, respectively, and that of tubular dilatation in predicting congenital causes were 97.30% and 98.06%, respectively. Conclusion: It is particularly important to diagnose the cause of CAD because its treatment depends on its etiology. When an echocardiography examination identifies CAD in infants, comprehensive and systematic analysis can quickly and accurately determine the cause of CAD according to the diagnostic strategy process and evaluation of dilatation and cardiac structure characteristics.

Congenital segmental dilatation of intestine with different morphology: A case report

Background: Congenital segmental dilatation of intestine is a rare entity characterized by a localized dilated segment of the intestine. It manifests as neonatal intestinal obstruction. Occurrence with another surgical anomaly may masquerade it and complicate the treatment course. Case Presentation: A 1-day-old male neonate with anorectal malformation underwent colostomy as first stage of the treatment. The stoma did not function postoperatively. At repeat surgery, congenital segmental dilatation of the ileum, with slightly different morphology, was found. Resection of the lesion and ileostomy were formed. Postoperative course remained uneventful. Conclusion: Congenital segmental dilatation of intestine may be missed at initial surgery. Complete surgical resection is curative.

Congenital Segmental Intestinal Dilatation: A Clinical Dilemma

Congenital segmental dilatation of intestine is a rare clinical entity encountered in neonates with very few case reports from world over. It usually presents with symptoms of partial or total obstruction in neonatal period or infancy. We report a case of a preterm baby with segmental dilatation of intestine which was managed surgically. Diagnosis was confirmed intraoperatively as well as histologically. Consideration of this entity in differential diagnosis of neonatal intestinal obstruction may enable for better understanding and more reporting of this rare condition.

Segmental dilatation of ileum in a child with juvenile idiopathic arthritis, localized myopathy with focal stasis induced immunogenic injury as a possible etiology: a case report

Background Segmental dilatation of the intestine (SDI) though uncommon is a well-known entity and more than 150 cases of SDI are reported in the literature. The presentation and association of SDI are well described; however, the association of SDI with juvenile idiopathic arthritis (JIA) has not been reported earlier. We described a case of SDI with JIA, who presented with malnutrition and chronic abdominal distension. Case presentation A 5-year-old female child was getting treated for JIA and referred to us for evaluation of chronic abdominal distension. On laparotomy, a huge SDI was found approximately 40 cm from the ileocecal junction and resection of the dilated part with approximately 2–3 cm of healthy ileum on each side and anastomosis was performed. The child recovered well and the features of arthritis also resolute 6 weeks later. From histologic analysis, we have suggested role of localized myopathy in development of segmental dilatation. We have further emphasized the link between the SDI with development of arthritis. Conclusion Etiology of SDI is multifactorial with architectural malformation of the smooth muscle due to localized myopathy is the key. Focal stasis in SDI affecting permeability and increased exposure to macromolecules, and antigens may give rise to immune-mediated arthritis. Surgical management can reduce and cure the symptoms of such patients.

Diagnostic dilemma of congenital segmental jejunal pseudo-dilatation associated with immature ganglion cells in the colon: A case report

Background: Neonatal intestinal obstruction is one of the emergency conditions with many etiologies, amongst which congenital segmental dilatation of small intestine is one, which is characterized by three to four folds increase in diameter of the bowel. Immaturity of ganglion cells of large intestine is also one of the congenital intestinal neuronal malformations which may cause neonatal intestinal obstruction. Prompt diagnosis of these conditions is very difficult as clinical and radiological features are non-specific to them. Case Presentation: A 3-day-old male baby presented with failure to pass meconium, abdominal distension, and bilious vomiting. At surgery, the baby had congenital segmental pseudo-dilatation of jejunum with immature ganglion cells in colon. Since it was not typical of a congenital segmental dilatation, it was left as such. The baby needed another laparotomy for resection of segmental pseudo-dilatation along with ileostomy. Conclusion: Because of rarity and diagnostic dilemma, re-laparotomy with ileostomy, which could have been avoided, were performed in the baby. Lack of typical features of congenital segmental dilatation along with presence of immature ganglion cells in the colon were unusual findings in this case