Giant omphaloceles

Congenital abdominal wall abnormalities in infants present an interesting and difficult management problem for surgeons. Congenital malformations of the ventral abdominal wall can be diagnosed by their distinctive anatomical presentations. Among them, omphalocele, gastroschisis and umbilical cord hernias are the most frequently observed clinically detected abdominal wall anomalies. Omphalocele refers to the herniation of abdominal contents through a defect in the abdominal wall at the umbilicus with or without the presence of a sac...

INTRODUCTION: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience...

OBJECTIVE: Giant omphaloceles (GO) have associated pulmonary hypoplasia and respiratory complications. Total lung volumes (TLV) on fetal MRI can prognosticate congenital diaphragmatic hernia outcomes; however, its applicability to GO is unknown. We hypothesize that late gestation TLV and observed-to-expected TLV (O/E TLV) on fetal MRI correlate with postnatal pulmonary morbidity in GO. METHOD: A single-institution retrospective review of GO evaluated between 2012 and 2022 was performed...

INTRODUCTION: We compared strategy outcomes and financial impact over the first two years of life (F2YOL) for patients with giant omphaloceles undergoing early repair (ER) (primary or staged) versus delayed repair (DR). METHODS: A retrospective review of giant omphaloceles (fascial defect > 5 cm/> 50% liver herniation) at a tertiary children's hospital between 1/1/2010 and 12/31/2019 was performed. Survival, length of stay, age at repair, ventilation days (VD), time to full enteral feeds, readmissions during the F2YOL, incidence of major associated anomalies, and total hospitalization charges during the F2YOL were compared...

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BACKGROUND: The repair of large abdominal wall defects that cannot be closed primarily is quite challenging. The component separation technique (CST) is a surgical approach using autologous tissue to close large abdominal wall defects. The CST requires extensive dissection between the abdominal skin and the anterior sheath of the rectus abdominis muscle. Subsequently, incisions are made at both sides of the external oblique aponeurosis, releasing the external oblique muscle from the internal oblique muscle, and then the right and left rectus abdominis muscles are brought together in the midline for defect closure...

BACKGROUND: Higher risk of recurrence has been reported in pediatric inguinal hernia patients with specific comorbidities. The purpose of this systematic review was to investigate which comorbidities predispose to recurrent pediatric inguinal hernias (RPIHs). METHODS: A comprehensive search of six databases was performed, reviewing the literature to date on RPIHs and the co-occurrence of comorbidities. English-language publications were considered for inclusion...

We present a case of a four-year-old male with a history of giant omphalocele who underwent ultrasound-guided Botox injection to bilateral anterior abdominal wall musculature in preparation for definitive repair. Botox administration was successfully combined with preoperative subfascial tissue expanders to achieve definitive midline closure of the anterior abdominal wall defect. Our experience suggests that Botox can be safely used as part of the treatment plan for giant omphalocele repair.

UNLABELLED:  Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformation complex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with the incidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology of this complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosis and appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy is considered...

Congenital malformations are defined as single or multiple defects of the morphogenesis of organs or body parts, identifiable during intrauterine life or at birth. With recent advances in prenatal detection of congenital malformations, many of these disorders can be identified early on a routine fetal ultrasound. The aim of the present systematic review is to systematize the current knowledge about the mode of delivery in pregnancies complicated by fetal anomalies. The databases Medline and Ebsco were searched from 2002 to 2022...

RATIONALE: An omphalocele is an abdominal wall birth defect, and a giant omphalocele (GO) is defined as an omphalocele having a diameter >5 cm or containing a herniated liver. GOs are usually treated in stages and in this case, during the silo reduction, dehiscence occurred at the suture site of the axis ring and skin edge, which was repaired using synthetic absorbable mesh. PATIENT CONCERNS: A girl infant was born at 36 weeks with a GO of 8 cm diameter, and herniated multiple organs such as the small bowel, cecum, appendix, and the entire liver...

INTRODUCTION: Covered abdominal wall defects (CAWD) can be categorized into giant omphaloceles (GOs), nongiant omphaloceles (NGOs), and umbilical cord hernias (UCHs). We sought to evaluate differences in management and outcomes of the different CAWD, treated at a large tertiary children's hospital, with regards to survival and association with other major congenital anomalies. METHODS: A retrospective review of CAWD patients between January 2010 and January 2021 was conducted...

INTRODUCTION: Omphalocele represents a rare congenital abdominal wall defect. In giant omphalocele, due to the viscero-abdominal disproportion, gradual reintegration of eviscerated organs is often associated with medical challenges. We report our preliminary experience combining staged gravitational reduction with vacuum (VAC) therapy as a novel approach for treatment of giant omphalocele. PATIENTS AND METHODS: Retrospective chart review of six patients (five females) born between September 2018 and May 2022 who underwent staged reduction of giant omphalocele in conjunction with VAC therapy was conducted...

BACKGROUND: Omphalocele is a congenital abdominal wall defect of the umbilical cord insertion site. A giant omphalocele, with a fascial defect > 5 cm in diameter and/or containing > 50% of the liver within the hernia sac, can be challenging for pediatric surgeons. Recently, negative pressure wound therapy has been reported as an effective management for giant omphaloceles; however, it is not recommended for an infected wound with necrotic tissue as it may exacerbate infection...

Aorto-right-atrial fistula is an uncommon condition with an unclear pathogenesis. We present the case of a 3-year-old girl with a giant omphalocele repaired days after birth and incidentally discovered with a celiacomesenteric trunk-to-right atrium fistula. Three-dimensional reconstruction CT unveiled its anatomical pattern, and the fistula was successfully closed using a Amplatzer vascular plug II percutaneously.

Tracheo-innominate artery fistula (TIF) is a rare complication of tracheostomy and refers to the formation of a fistula between the trachea and innominate artery. Because TIF is fatal, prevention rather than treatment is very important. Here we report the cases of two high-risk patients who underwent tracheostomy, and in whose cases attempts were made to lower the risk of TIF. In the first patient who developed a chest deformity with Duchenne muscular dystrophy, a tracheostomy was performed with a high-level (cricothyroid level) approach compared with the standard tracheostomy...

The nutritional requirements of neonates with congenital abdominal wall defects (AWD) remain poorly described. In particular, there is a lack of literature on the calorie, protein and micronutrient needs of those with AWD. Nutritional therapy is a cornerstone of care in patients with burns due to the metabolic consequences of injury to the epithelial layer. Similarly, children with AWD may require specialized nutritional plans to support their growth and wound healing. This case series supports the theory that patients with ruptured omphaloceles may require higher calorie, protein, and micronutrient provisions in comparison to patients with intact omphaloceles, due to increased metabolic demand to support wound healing and skin epithelization...

Our objective is to determine perinatal factors contributing to the development of pulmonary hypertension (PH) in patients with isolated giant omphaloceles (GO). All cases of omphaloceles that underwent prenatal and postnatal care at the University Hospital of Lille between 1996 and 2021 were reviewed. We included all infants with isolated GO, including at least a part of the liver, who were treated by delayed surgical closure. Prenatal and postnatal data were recorded and correlated with postnatal morbidities...

Omphalocele is a congenital abdominal wall defect with a constant incidence in recent decades, sometimes representing a real burden for neonatal intensive care units due to prolonged hospitalization and the evolution to death. In our study, we aimed to detect the main risk factors of an unfavorable evolution in the case of omphalocele. METHODS: Retrospective cohort study of all neonates with omphalocele treated in our tertiary pediatric hospital during the last three decades; from 158 patients, 139 patients were eligible for the study...

Background Congenital anomalies are responsible for approximately 20% of all neonatal deaths worldwide. Improvements in antenatal screening and diagnosis have significantly improved the prenatal detection of birth defects; however, these improvements have not translated into the improved neonatal prognosis of babies born with congenital anomalies. Objectives An attempt has been made to summarise the prenatal interventions, if available, the optimal route, mode and time of delivery and discuss the minimum delivery room preparations that should be made if expecting to deliver a fetus with a congenital anomaly...