Giant Omphalocele Ruptured: An Operated Case and Review of the Literature

Background: The omphalocele is a congenital defect of closure of the abdominal wall at the level of the umbilical ring with evisceration of the intra-abdominal organs in the form of a bag covered with a membrane. This membrane can rupture and become an extreme emergency that can put the infant’s life at stake. Aim: The purpose of this case report is to present a case of ruptured omphalocele operated successfully and describe the factors of good prognosis. Case Presentation: A female new-born with a giant omphalocele. This omphalocele ruptured during conservative treatment. An emergency surgery was organised and the defect was closed by using a prosthesis. The postoperative course was simple. During the 12 months follow-up, we have seen no complication. Conclusion: The management of ruptured omphaloceles is a surgical and anesthetic challenge. The mortality of this condition can be improved in our context by setting up well-equipped neonatal resuscitation units.

Pentalogy of Cantrell with Total Ectopia Cordis and a Major Omphalocele-A Case Report

Ectopia cordis(EC)is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen.It can be defined as 0.1%of congenital heart diseases,and it could present isolated or could belong to the spectrum of the Pentalogy of Cantrell(PoC),which is a rare congenital disorder first described in 1958 by Cantrell.We are reporting a rare case of total ectopia cordis,associated to a major omphalocele,total agenesis of the sternum,anterior diaphragmatic deficiency,absence of pericardium,and persistence of the Ductus arteriosus,making therefore these features compatible with a full spectrum of the Pentalogy of Cantrell,encouraging us to report this case.

Twin Pregnancy with Omphalocele: Challenges in Detection and Management

Twins are more likely to have congenital anomalies than singletons. Omphalocele is still a life-threatening congenital abnormality that requires adequate antenatal diagnosis and early treatment. A 31-year-old woman presented with labor pains in the eighth pregnancy month. There was a clear, watery discharge from the birth canal 4 hours before admission. She was previously diagnosed with twins, with one cotwin having suffered omphalocele through a 6-month-ultrasound examination. At admission, the condition was diagnosed as monochorionic-diamniotic twins at 32 - 33 weeks in the 2nd stage of labor: the first baby cephalic presentation;second breech presentation with omphalocele. She vaginally gave birth to twin infants, with those having Apgar 5’ of 6/7, respectively. Both infants were admitted to the intensive care unit and under treatment.

Minor Omphalocele,Type IV Ileal Atresia,Congenital Ostomy

Abstract: Omphalocele is often associated with the presence of other congenital anomalies. Isolated minor omphaloceles are known to have a favorable prognosis. Case reports have demonstrated nonclassical associations occurring in minor omphaloceles. Intestinal atresia is seldom described as being associated with minor omphaloceles. Special attention should be paid to intestinal exploration in small omphaloceles to avoid complications. Here we report a new association of a minor omphalocele, type IV ileal atresia and congenital ostomy.

Omphalocele Diagnosis Problem in Rural Environment

Introduction: An omphalocele is a congenital malformation due to a failure of closure of the umbilical ring with exteriorization of the abdominal viscera covered by the translucent and avascular amniotic membrane. We report a case of giant omphalocele unrecognized at birth followed by a review of the literature. Observation: This was a 4-year-old girl admitted to the department for umbilical swelling that had been present since birth. Clinical and paraclinical examinations made it possible to objectivize a post-omphalocele hernia with hepatic content plus ectopic accession of the left ureter, the surgical procedure consisted of reintroducing the liver associated with a cure of the hernia + ureterovesical reimplantation. Conclusion: Omphalocele is a congenital malformation, antenatal diagnosis is possible during morphological ultrasound from the 12th week.

Ex utero intrapartum treatment for giant congenital omphalocele

Background To determine whether ex utero intrapartum treatment (EXIT) is an appropriate approach for managing fetuses antenatally diagnosed with giant congenital omphaloceles. Methods We retrospectively reviewed patients with omphaloceles who underwent either an EXIT procedure or a traditional repair surgery. Basic and clinical parameters including gender, gestational age, birth weight, maternal blood loss, operative times and operative complications were analyzed. During the 6–12-month follow-ups, postoperative complications including bowel obstruction, abdominal infections, postoperative abdominal distension were monitored, and survival rate was analyzed. Results A total of seven patients underwent the EXIT procedure and 11 patients underwent the traditional postnatal surgery. We found no differences in maternal age, gestational age at diagnosis, gestational age at delivery and birth weight between the two groups. In the EXIT group, the average operation time for mother was 68.3 ± 17.5 minutes and the average maternal blood loss was 233.0 ± 57.7 mL. The operation time in the EXIT group (22.0 ± 4.5 minutes) was shorter than that in the traditional group (35 ± 8.7 minutes), but the length of hospital stay in the EXIT group (20.5 ± 3.1 days) was longer than that in the traditional group (15.7 ± 2.5 days,P < 0.05). During the follow-up, one patient in the EXIT group had an intestinal obstruction, one developed abdominal compartment syndrome and one died in the traditional group. Conclusions In our experience, EXIT is a safe and effective procedure for the treatment of giant congenital omphaloceles. However, more experience is needed before this procedure can be widely recommended.

Gastroesophageal reflux and congenital gastrointestinal malformations

Although the outcome of newborns with surgical congenital diseases(e.g.,diaphragmatic hernia;esophageal atresia;omphalocele;gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery,infant survivors often require intensive treatment after birth,have prolonged hospitalizations,and,after discharge,may have longterm sequelae including gastro-intestinal comorbidities,above all,gastroesophageal reflux(GER).This condition involves the involuntary retrograde passage of gastric contents into the esophagus,with or without regurgitation or vomiting.It is a well-recognized condition,typical of infants,with an incidence of 85%,which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus,in the first few months after birth.Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood,it has been hypothesized that common(increased intra-abdominal pressure after closure of the abdominal defect) and/or specific(e.g.,motility disturbance of the upper gastrointestinal tract,damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects.Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases.The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations.

Perioperative nursing of a newborn with Beckwith-Wiedemann syndrome

Objective:To explore the perioperative nursing of a newborn with Beckwith-Wiedemann syndrome.Methods:We observed the blood glucose of the child and prevent infection of omphalocele before transpor tation and before the operation;after the operation,we performed airway nursing,blood glucose monitoring,urination observation,and tumor marker monitoring;finally,at the time of discharge from the hospital,we provided health education.Results:After a series of perioperative nursing measures,the child was discharged smoothly from hospital 34 d after operation.Conclusions:Beckwith-Wiedemann syndrome is a rare congenital malformation.Timely and accurate observation and evaluation of the child during perioperative period and targeted nursing measures can effectively reduce or avoid the occurrence of postoperative complications.