Congenital Malformation Of Gastrointestinal Tract Research Articles

Major Congenital Malformations of Gastrointestinal Tract Among Neonates Presenting to Tertiary Care

Background: Major congenital malformations of gastrointestinal tract (CMGIT), are usually manifested in early neonatal period by signs of intestinal obstruction and may be life threatening. A broad spectrum of Gastrointestinal Tract (GIT) anomalies can be seen including both upper and lower GIT. As compared to other congenital anomalies, GIT anomalies are not well studied and till now very few studies have reported prevalence and pattern of GIT malformations. Objective: To determine different type of common gastrointestinal anomalies among neonates presenting at tertiary care hospital. Methods: This descriptive cross sectional study was done at National Institute of Child Health (NICH) Karachi over period of 6 months, from 1-8-17 to 31-1-18. All neonates presenting to emergency department and got hospitalized with CMGIT were enrolled. Results: There were 35 neonates enrolled with CMGIT. Out of these 16(45.7%) were male and 19(54.3%) were females. Mean age 9.4+3.82 (range 1-20) days. Mean weight was 2.5±0.98 (range 0.5-4.5) kg. Mean duration of symptoms was 5.8+1.72 (range 1-9) days. Among the CMGIT, 12 (34.3%) cases were of Anorectal malformation, persistent Vitelline duct was found in 6 (17.1%), Intestinal atresia in 4(11.4%), Intestinal stenosis in 5(14.3%), Hirshsprung disease in 4(11.4%) and Exomphalos in 4 (11.4%). Conclusion: Anorectal malformations were most common anomaly followed by persistent vitelline duct. Antenatal follow up and anomaly scan of all neonates should be done. Keywords: Gastrointestinal anomaly, Neonates, congenital anomaly.

Prenatal Diagnosis of Prepyloric Diaphragm: A Case Report

The prepyloric diaphragm (A type of pyloric atresia) is a rare type of congenital malformation of gastrointestinal tract of unknown etiology. A thin two layered mucus membrane of about 2 to 4 mm, proximal to pylorus causes the gastric outlet obstruction in neonate. We are reporting a case of prepyloric diaphragm which had been diagnosed at 37 weeks of gestation by Ultrasonography and diagnosis was confirmed after birth during surgery. Prenatal ultrasonography of a 37 weeks pregnant mother revealed polyhydramnios, persistently dilated, a fluid filled blind sac at epigastrium and right hypochondrium with peristaltic wave and to & fro movements of fluid contents. There was no double bubble sign. The baby boy was born by Caesarean Section. After breast feeding, baby had non-bilious vomiting with mild abdominal distension. Postnatal ultrasonography showed dilated fluid filled stomach. Exploratory laparotomy on 14thday of life revealed a prepyloric diaphragm with a central hole, 1 cm proximal to the pylorus. The 2 mm thick diaphragm was excised circumferentially. Postoperative period was uneventful. Milk feeding started at 6th post-operative day and discharged with advice at 7th post-operative day. Prepyloric diaphragm or antral web is to be considered as provisional diagnosis if there is prenatal suspicion of gastric outlet obstruction, polyhydramnios of mother and persistent non-bilious vomiting in neonate; as simple excision of the diaphragm is curative for this unusual abnormality if there is no other associated abnormality.
 Chatt Maa Shi Hosp Med Coll J; Vol.18 (2); July 2019; Page 67-69

Multifaceted behavior of Meckel's diverticulum in children.

Meckel's diverticulum (MD) is one of the most common congenital malformations of gastrointestinal tract in children. However, the nonspecific clinical manifestations of MD often cause a diagnostic as well as therapeutic challenge to pediatric surgeon. This study aimed to review our experience in managing this disease while evaluating the management strategies. We retrospectively analyzed the clinical data of all patients diagnosed with MD admitted to our center between January 2010 and December 2015. Factors documented including demographic criteria, clinical manifestations, preoperative examinations, surgical methods, histopathological characteristics, postoperative complications, and outcomes. The patients included 210 males and 76 females, aged from 1day to 15years. In fifty three patients, the MD was an incidental finding at laparotomy or laparoscopy. The remaining 233 patients were symptomatic and presented with various clinical features. Ninety nine patients presented with episodes of bleeding per rectum or melena. Fifty six patients demonstrated symptoms of diverticulitis or perforated MD. Forty patients were diagnosed as intestinal obstruction, and 35 patients with intussusception requiring surgical reduction. Two cases of Littre hernia and one case of foreign body trapped in MD were also observed in this group. Six patients misdiagnosed as appendicitis at another institution were reoperated in our department. Among the 99 patients with bleeding per rectum, 78 underwent a Tc-99m scan that showed a positive tracer in 55 patients and negative in 23. All patients underwent resection of the diverticulum, except for 2 cases of postponed resection. Histology revealed ectopic gastric mucosa or ectopic pancreatic tissue in 154 patients; significant differences were observed between the symptomatic group and the accidentally found group. One patient died of peritonitis and sepsis postoperatively; one case of anastomotic leak and one case of adhesive intestinal obstruction were reoperated. Meckel's diverticulum has various clinical presentations and it is difficult to make a precise diagnosis preoperatively. It is necessary to maintain a high suspicion of MD in the pediatric age group with symptoms of abdominal pain, gastrointestinal hemorrhage or intestinal obstruction. Heterotopic tissue is the main cause of complicated diverticulum, and it is safe and feasible to remove the incidentally found MD. Laparoscopy should become the first choice of methods in diagnosis and treatment of MD. Treatment study. Level IV.

Congenital malformations of gastrointestinal tract in children aged less than 5 years of age: A retrospective and prospective study

Introduction: The spectrum and prevalence of congenital abnormalities of gastrointestinal tract vary in different populations depending upon the environment influences, genetic predisposition and prevalent socioeconomic factors. The aim of this study is to review and analyse the cases registered in the last five years in our centre and study their clinical presentation, spectrum, prevalence and also the histopathological picture. Methods: This cross-sectional descriptive study was taken up in Niloufer Hospital, Hyderabad. This was a 3 year retrospective and 2 year prospective study. All Pediatric surgical specimens and biopsies of children aged less than 5 years received during a period of 5 years were included in the study. Results: During the study period, total 2842 pathological specimens were reviewed, out of which 161 (5.67%) cases were related to GI tract Anomalies. Hirschsprung’s disease was the commonest accounting for about 47.2% of all the anomalies. Together Hirschsprung’s disease, Omphalo mesenteric duct remnants, intestinal atresia’s, duplication cysts and meconium ileus contributed to greater than 75% (¾ ) of the cases. Most of the cases were less than 1 year of age (71%). Male predominance was noted with a male to female ratio of 2.74:1. Conclusion: Congenital and developmental abnormalities of gastro intestinal tract are not very uncommon in the Indian sub continent and these are a cause of significant mortality and morbidity in pediatric age group. This study has highlighted the prevalence and types of congenital anomalies seen in our Geographical area.

Meckel's diverticulum in children: Our 12-year experience.

Background:Meckel's diverticulum (MD) is the one of the most common congenital malformation of gastrointestinal tract and has varied clinical presentations. We are presenting here our 12-year experiences with MD in children at tertiary care hospital in North India. It highlights the fact that isolated gangrene of MD can occur, and it is associated with increased morbidity.Materials and Methods:This retrospective study is conducted by analysing the medical records of the patients who were operated for MD in the last 12 years in paediatric surgery department at our hospital.Results:Sixty-five patients were operated for MD in study period; in this 52 were males and 13 were females with mean age of presentation 3.2 years. The most common presentation was intestinal obstruction seen in 86.1% (56 cases). Intestinal haemorrhage was seen in 4.6% (3 cases) and diverticulitis in 3% (2 cases). Perforation of the gut with peritonitis was present in 6.1% (four cases). Cause of obstruction was intussusception in 21.4% (12 cases), fibrous band connected to umbilicus in 17.8% (10 cases), volvulus in 17.8% (10 cases), kinking in 16.0% (9 cases), knotting in 14.2% (8 cases) and herniation of gut below in 12.5% (7 cases). Isolated gangrene of MD was present in ten cases with intestinal obstruction. The ectopic gastric mucosa was seen in three and pancreatic mucosa in two cases. Mortality and morbidity during the study were one and three cases, respectively.Conclusion:MD may remain clinically silent for lifetime, or it may have life-threatening complications. In our series, intestinal obstruction and not the haemorrhage was the most common presentation. Isolated gangrene of MD with obstruction was present in significant numbers, which we failed to find in literature.

Stages and directions of psychological support to families caring for children with severe congenital malformations

Nowadays the medical and social issues associated with hereditary and congenital pathologies, and first of all, congenital malformations and chromosomal diseases are very relevant. For a decade they have been assuming ever greater medical, social and economic importance. Long-term and complicated care of patients with congenital malformations, medical correction of the defects, pedagogical intervention as well as psychological support to social adaptation require a long time and much of finances, material and technical resources. Surgical pathologies in neonates and namely congenital malformations causing most perinatal and infant deaths are in the focus of current neonatology. The prevalence of congenital malformations of gastrointestinal tract and anterior abdominal wall ranges from 13 to 26, 4 cases per 10000 live-born neonates, and diaphragmatocele is registered in 2.5 – 5.0 cases per 10000 of livebirths. These pathologies account for 29.1% of the total congenial malformations ranking the 3 rd position. The most effective and reliable technique to spot congenital malformations of a fetus is ultrasound scanning. The optimal timing for detecting congenital malformations of gastrointestinal tract is 12 – 23 weeks of gestation. In addition to purely medical aspects of the diagnosis of birth defects which may be corrected surgically, there is another, but not less important psychological aspect. There is no doubt about the necessity of psychological support to parents and family members during the prenatal period (from diagnosis to birth), at all stages of surgical correction and postoperative care, regardless of their length. When any congenital complications have been detected the medical and psychological follow-up should be aimed to establish the most accurate diagnosis by experts, to provide detailed informing parents about the diagnosis and all up-to-date methods of treatment of this disease, to afford them ground for consulting with experts experienced in the treatment of certain pathologies. There are several behaviour types demonstrated by parents who become aware of their child’s diagnosis of congenital malformation: compensated families fully accepting a child and its defect (23%); families showing unacceptance of the child with birth defects, family with snail syndrome (26%), dysadvantaged families or families with low or unsatisfactory socio-cultural status (26%). Implementation of a certain type of behaviour by the family starts from the moment of diagnosis. Psychological correction in these families is multiple-aspect, complex and based on a number of principles, compliance with which ensures effective result of the influence: the principles of humane and empathic attitude, the unity of diagnosis and correction, harmonization of family relations, the encouragement of positive thinking towards the child, providing person-centred assistance, creating an inner positive plan for actions, the coordination in the activity of the medical staff, psychological groups and families. Psychological correction is performed in three main vectors (directions): a psychological study of the problem, psychological counselling, and providing psychological and educational support to family members. Prenatal diagnosis of congenital malformations shocks parents, causes psychological discomfort, in some cases mental and emotional stress with elements of emotional unacceptance of the child. The reaction of parents to the news that their child has birth defect is characterized by the staging that includes: phase of shock, phase of reactivity, adaptive phase, the phase of orientation. Since the baby has been born, the major stressful factor is staying in the atmosphere of uncertainty, expectations of surgical treatment and its results. Psychological supporter aimed to develop positive thinking, stipulates the following objectives: to correct unconstructive parental behaviour, to develop parents’ awareness of the problem of their child, to improve family relationship. Next, goals psychological rehabilitation of families where a child is brought up with the defect, the reconstruction of the hierarchy of values in life parents of a sick child, parents exercise personal growth in the process of interaction with your child. Further, the goals of psychological rehabilitation of families caring children with congenital malformation is the reconstruction of parents’ hierarchy of values in life, and the stimulation of personal growth in the process of interaction with the child.

Perforated Meckel’s Diverticulum Lithiasis: An Unusual Cause of Peritonitis

Meckel's diverticulum is the commonest congenital malformation of gastrointestinal tract and represents a persistent remnant of the omphalomesenteric duct. Although it mostly remains silent, it can present as bleeding, perforation, intestinal obstruction, intussusception, and tumours. These complications, especially bleeding, tend to be more common in the paediatric group and intestinal obstruction in adults. Stone formation (lithiasis) in Meckel's diverticulum is rare. We report a case of Meckel's diverticulum lithiasis which presented as an acute abdomen in an otherwise healthy individual. The patient underwent an exploratory laparotomy which revealed a perforated Meckel's diverticulum with lithiasis; a segmental resection with end-to-end anastomosis of small bowel was performed. Patient recovery was delayed due to pneumonia, discharged on day 20 with no further complications at 6 months following surgery.

Meckel's Diverticulum: a Systematic Review

Meckel's diverticulum is the most common congenital malformation of gastrointestinal tract. It can cause complications in the form of ulceration, haemorrhage, intussusception, intestinal obstruction, perforation and, very rarely, vesicodiverticular fistulae and tumours. These complications, especially bleeding, are more common in the paediatric age group than in adults; however it is not uncommon to miss the diagnosis of Meckel's diverticulum in adults. Here, we reviewed the literature regarding the complications of this forgotten clinical entity in adults with potential diagnostic difficulties and management strategies.

Meckel's diverticulum: a systematic review

Meckel's diverticulum is the most common congenital malformation of gastrointestinal tract. It can cause complications in the form of ulceration, haemorrhage, intussusception, intestinal obstruction, perforation and, very rarely, vesicodiverticular fistulae and tumours. These complications, especially bleeding, are more common in the paediatric age group than in adults; however it is not uncommon to miss the diagnosis of Meckel's diverticulum in adults. Here, we reviewed the literature regarding the complications of this forgotten clinical entity in adults with potential diagnostic difficulties and management strategies.