Giant Omphalocele Research Articles

Management of narrow stalked giant omphalocele using tissue expansion, staged closure, and amnion preservation technique

Abstract Giant omphaloceles (GO) frequently present challenges to closure that are often influenced by patient factors, including degree of visceroabdominal disproportion, associated comorbidities (including pulmonary hypoplasia), and variation in surgeon preference for both technique and timing of abdominal wall reconstruction. In this case report, we describe our experience with a GO that presented the unique challenge of an uncharacteristically small fascial defect through which all structures (including liver) were herniated. The challenges of the small defect, both in potentially limiting the growth of healthy neoskin and preventing any degree of visceral reduction by compression are highlighted. An innovative reconstructive solution developed in collaboration with plastic surgery is described.

Omphalocele at school age: What do parents report? A call for long-term follow-up of complex omphalocele patients

ObjectiveMany children with omphalocele experience morbidity in early life, which could affect long-term outcomes. We determined parent-reported outcomes in school-aged children treated for minor or giant omphalocele. Study designWe sent paper questionnaires to the parents of all children treated for omphalocele in 2000–2012. Giant omphalocele was defined as defect diameter ≥ 5 cm with liver protruding. Motor function (MABC-2 Checklist) was compared with Dutch reference data; cognition (PedsPCF), health status (PedsQL), quality of life (DUX-25) and behavior (Strengths and Difficulties Questionnaire; SDQ) were compared with those of controls (two per child) matched for age, gender and maternal education level. Possible predictors of cognition and behavior were evaluated using linear regression analyses. ResultsOf 54 eligible participants, 31 (57%) returned the questionnaires. MABC-2 Checklist scores were normal for 21/26 (81%) children. Cognition, health status, quality of life and behavior were similar to scores of matched controls. One quarter (26%) of children with omphalocele scored ≤ − 1 standard deviation on the PedsPCF, compared with 9% of matched controls (p = 0.07). Giant omphalocele and presence of multiple congenital anomalies (MCA) were most prominently associated with lower PedsPCF scores (giant omphalocele: β −22.11 (95% CI: −43.65 to −0.57); MCA -23.58 (−40.02 to −7.13)), although not significantly after correction for multiple testing. ConclusionsParent-reported outcomes of children with omphalocele at school age are reassuring. Children with an isolated, minor omphalocele do not need extensive long-term follow-up of daily functioning. Those with a giant omphalocele or MCA might be at risk for delayed cognitive functioning at school age; we recommend long-term follow-up to offer timely intervention.

Autism spectrum disorder and neurodevelopmental delays in children with giant omphalocele

ObjectiveTo determine the prevalence and identify risk factors of autism spectrum disorders (ASDs) and neurodevelopmental delays in giant omphalocele (GO) survivors. Materials and methodsThe study cohort consists of 47 GO survivors enrolled in our follow-up program between 07/2004 and 12/2015. All patients underwent assessments at 2 years of age or older. Outcomes were assessed by either the Bayley Scales of Infant Development II (prior 2006) or III (after 2006), or the Wechsler Preschool and Primary Scale of Intelligence (children older than 4 years). ASD diagnosis was made based on the Diagnostic and Statistical Manual of Mental Disorders IV (prior to 2014) or 5 criteria. ResultsThe prevalence of ASD in GO children is 16 times higher than the general population (P = 0.0002). ASD patients were more likely to be diagnosed with neurodevelopmental and neurofunctional delays, language disorders, and genetic abnormalities (P < 0.01). While 53.2% of GO children scored within the average range for all developmental domains, 19.1% scored within the mildly delayed and 27.7% in the severe delayed range in at least one domain. Prolonged respiratory support, pulmonary hypertension, gastroesophageal reflux disease, feeding problems, prolonged hospitalization, abnormal BAER hearing screen, presence of delayed motor coordination, and hypotonicity were associated with delayed scores (P < 0.05). ConclusionsThere is a significant rate of ASD in GO survivors. Neurodevelopmental delays, language delays, and genetic abnormalities were strongly associated with ASD. Neurological impairments were present in nearly half of GO children. Surrogate markers of disease severity were associated with below average neurodevelopmental scores.Level of evidenceLevel IV

Operative versus conservative treatment for giant omphalocele: Study of French and Ivorian management

The giant omphalocele is currently a surgical challenge. The morbidity and mortality associated with its care is non-negligible. Nowadays, different studies have revived the debate between conservative and surgical management for giant omphalocele. The purpose of this study is to compare the conservative and surgical management of the giant omphalocele in terms of morbidity and mortality. Retrospective study including all giant omphaloceles comparing surgical management (French University hospital centers) and tanning (Ivory Coast University hospital center). Epidemiology was studied as well as medical and surgical managements both intra and post operative. One hundred and forty-seven patients included (98 patients in the "tanning" group and 49 in the "surgery" group). Hospital length of stay is significantly shorter in the "tanning" group as they do not spend time in intensive care unit. Morbidity is higher in "surgery" group. The average duration for oral empowerment was acquired at 179 days in the "surgery" group, whereas in the "tanning" group 90% was immediately and exclusively breastfed. No significant differences in terms of epithelialization time. The tanning treatment has its own place in the therapeutic arsenal in the management of the giant omphalocele no matter where it takes place. However, its realization in surgical environments prevents certain complications related to the technique or the pathology.

Immediate versus staged repair of omphaloceles

Omphalocele is an abdominal wall defect involving the umbilical ring which results in visceral herniation of small and large intestine, liver, spleen and sometimes gonads. The covering of the herniated viscera by a fused membrane consisting of peritoneum, Wharton's jelly and amnion projects viscera from mechanical injury and exposure to chemical irritants in amniotic fluid.Omphalocele is usually diagnosed before birth, is variable in size, and is frequently associated with chromosomal and somatic anomalies, syndromes, and variable degrees of pulmonary hypoplasia which can be lethal.In this article we examine surgical closure options for omphaloceles ranging from early primary fascial repair for small omphaloceles to a staged repair, often facilitated by an amnion preserving silo, which may be necessary for giant omphaloceles that cannot be closed primarily. We also review some of the adjuncts to abdominal wall reconstruction including tissue expansion and mesh. Conservative management (paint and wait) of giant omphaloceles is described elsewhere.

Long term complications and outcomes in omphalocele

After a diagnosis of omphalocele during pregnancy, questions regarding long-term prognosis are of primary importance for parents. It is imperative that their questions are answered with substantiated data to promote confident decisions for their children. They frequently express concerns regarding long-term survival, quality of life, need for more operations, feeding issues, motor and cognitive development, cosmesis, and the unique difficulties of giant omphaloceles. The available outcome studies that address these questions are discussed.

Paint and wait management of giant omphaloceles

Management of the very large defect or those in patients with severe comorbidities has evolved to the use of methods that result in escharification and eventual skin coverage over the viscera. This treatment strategy employs principles that were described in the early 20th century. This review will describe the history, principles, methods, and outcomes from the so called ‘paint and wait’ management of omphalocele.

Respiratory disorders in patients with omphalocele

The respiratory difficulties experienced by infants with omphalocele are being appreciated with greater frequency. These problems represent self-limited difficulties related to omphalocele closure or are the result of severe pulmonary disease including pulmonary hypoplasia and pulmonary hypertension. Infants with giant omphalocele represent a unique group that may experience increased respiratory morbidity which may lead to chronic respiratory problems extending into childhood and adolescence. Importantly, respiratory insufficiency at birth is an independent predictor of mortality for patients with omphalocele. In this review, we will provide a summary of the respiratory difficulties experienced by patients with omphalocele as well as insight into management and surveillance.

Ruptured omphalocele: Diagnosis and management

Omphalocele is an abdominal wall defect which can be classified as small, giant, or ruptured. Ruptured omphaloceles require prompt diagnosis and management to prevent associated morbidity and mortality and represent a challenging surgical condition. This review serves to define the etiology, diagnosis, initial resuscitation, and surgical therapy employed in the treatment of ruptured omphalocele. Resuscitation should focus on maintaining hydration and normothermia. Broad spectrum antibiotics should be initiated. Similar to giant omphaloceles, procedural intervention includes primary closure, silo, synthetic and biologic mesh, negative pressure wound therapy, and topical agents. Despite advances in neonatal care, the prognosis remains guarded and mortality is high.

Variants of prenatal ultrasound imaging of omphalocele in the fetus

Purpose - to present characteristic features of prenatal ultrasound imaging in omphalocele among patients of the Department of Fetal Medicine, to describe most common typical variants. Material and methods. A retrospective analysis of 201 ultrasound examinations in 150 patients with omphalocele in the fetus, which were referred to the Department of Fetal Medicine in 2007-2018. Results. The most common typical variants of ultrasound imaging/anatomical variants of omphalocele in the fetus are presented. According to hernia content there were following frequent variants: “intestine” in 48.15 %, "intestine + liver" in 31.11 %, "intestine + liver + stomach" in 15.56 %, "liver" in 5.19 %. A working classification of omphalocele by size is proposed and 4 gradations are distinguished: small, medium, large and giant. Incidence of chromosomal pathology was significantly higher in small-sized omphalocele (44 %) and rate of associated structural pathology was high in large (54.5 %) and giant omphalocele (72.7 %). Conclusion. The study showed significant variability of size and anatomical variants of omphalocele in the fetus. In 44.9 % of cases anatomical variants of severe pathology with large or giant size omphalocele were present. Key words: congenital malformations, abdominal wall defects, variants of omphalocele, chromosomal pathology.

Repair of giant omphalocele by component separation technique

Abstract Giant omphalocele management has always been a challenge because of the large fascial defect and the associated anomalies. We managed successfully a neonate with giant omphalocele and no associated anomalies by delayed repair constituting escharotic therapy initially and later managed the ventral hernia by component separation technique before her preschool years. Her recovery was non-eventful with no recurrence of the hernia on follow up.

Хирургические методы лечения омфалоцеле больших размеров у детей

Хирургические методы лечения омфалоцеле больших размеров у детей

Nonoperative management of giant omphalocele leading to early fascial closure

PurposeWe describe our series of giant omphalocele patients treated with a serial taping method for gradual reduction of the abdominal contents and early fascial closure. MethodsBetween 2010 and 2017 we cared for ten newborns with giant omphaloceles. The average gestational age was 35.5 weeks (range 29–38) and average birthweight was 2.84 kg. Seven infants had other major anomalies, including one with a variant of Pentology of Cantrell. Four had abnormal chromosomes. None had any attempt to primarily close the defect. Omphalocele defects were serially taped at bedside in the NICU with the child awake until the viscera were completely reduced, and the defect could be closed. ResultsMean time to closure was 13.7 days (median 14 days). Six were closed primarily without a patch. The remaining four infants required Gore-Tex patch (covered by skin) which was later removed and fascia closed in three infants (at 70 days, 75 days, and 11 months of age). Total length of stay was a mean 71.8 days (median 71). ConclusionsSerial taping achieves early fascial closure and avoids complications of a staged surgical approach, such as multiple anesthetics, loss of fascial margin integrity, silo dehiscence, and fistula formation. Compression of the viscera is slow enough to avoid abdominal compartment syndrome and the fascia and amnion are left intact leaving the option available to use escharotic agents if required. Type of studyTreatment study. Level of evidenceLevel III.

Early surgical management for giant omphalocele: Results and prognostic factors

ObjectivesGiant omphalocele often represents a major surgical challenge and is reported with high mortality and morbidity rates. The aim of this study was to assess the outcome of neonates with giant omphalocele managed with early operative surgical treatment, and subsequently to identify possible factors that could alter the prognosis. MethodsWe reviewed the medical records of 29 consecutive newborns with prenatally diagnosed giant omphalocele. In these cases one of two procedures had been performed: either staged closure after silo, or immediate closure with a synthetic patch. The cases were separated into 2 groups: Isolated giant omphalocele (IO group) and giant omphalocele associated with malformation (NIO group). ResultsInfants in the IO group had a lower size of the omphalocele (p<0,001), a shorter hospital stay (95 days [45-915] vs. 41.5 days [10-110] p= 0, 02), and a shorter median ventilation length (10 days [1-33] vs. 27, 5 [6-65] p = 0, 05). In the NIO group, 5 cases displayed a significantly more difficult course than the others. They were compared to the remaining cases for prenatal and anatomic features. Four factors associated with greater morbidity were identified:-Associated cardiopathies (septal defects)(100% vs. 15%, p =0,001),-Associated congenital anomalies (100% vs. 10%, p <0.001),-Omphalocele containing both liver and bowel (80 vs. 30%, p = 0, 04),-Mean omphalocele/abdominal circumference (OC/AC) ratio 0, 82 [0, 60-1, 25] vs. 0, 76 [0,51-1, 20], p=0, 05. ConclusionsIsolated omphalocele, even containing the whole liver, has a very good prognosis with early surgical treatment. Without associated anomalies, 95% of giant omphaloceles can be discharged with a median of 41.5 days in hospital. However, associated anomalies (especially cardiopathies) may burden the prognosis and should be both carefully assessed during pregnancy and taken into account in parental information. Type of StudyRetrospective Study Level of EvidenceLevel I.

Giant Omphalocele with skeletal deformities: a case report

Omphalocele is an anterior abdominal wall defect at the base of the umbilical cord, with herniation of the abdominal contents which are covered by a membrane and associated with a very high incidence of structural and chromosomal anomalies including heart defect, vesical, genital or diaphragmatic malformations. A 1000 gm preterm neonate was born vaginally, to a non-consanguineously married primi mother with uneventful pregnancy. On examination baby had a large anterior abdominal wall defect covered by a thin membrane with widely open and bulging anterior fontanellae. Eyes, ears and lips were well developed but eyelids were fused. Liver, spleen and intestine were visible under a thin transparent membrane. Rib cage was formed, and ribs were palpable but lower limbs were directed posteriorly and genitalia was ambiguous. Baby was in gasping condition and died within 30 minutes after birth. Prenatal accurate diagnosis by providing adequate antenatal care and ultrasonogram would permit an opportunity to counsel the family and to prepare for optimal postnatal care.

Accuracy and impact of prenatal diagnosis in infants with omphalocele

BackgroundAssociated anomalies in omphalocele are common, but to which extent these anomalies are diagnosed before or after birth is less well documented.AimTo investigate the different types of associated anomalies, long-term survival and the extent whether these are diagnosed pre- or postnatally in children with a prenatal diagnosis of omphalocele at a single institution.Materials and methodsRetrospective review of all pregnancies with omphalocele managed and/or born at our institution between 2006 and 2016.ResultsA total of 42 cases with prenatally diagnosed omphalocele were identified. Of those 14 (31%) decided to terminate the pregnancy (TOP). Of the remaining 28 that continued, 12 were giant omphaloceles. The overall mortality rate was 18, 25% for giant and 12% for non-giant omphaloceles. 64% had associated anomalies. Only 1/3 of these anomalies is diagnosed prenatally.ConclusionThe rate of associated malformations that are diagnosed postnatally is high, but the majority was malformations with a minor clinical significance or impact on future health. Beckwith–Wiedemann syndrome was present only in cases of non-giant omphalocele in our cohort.

Omphalocele: from diagnosis to growth and development at 2 years of age

ObjectivesTo compare the prenatal frame of reference of omphalocele (ie, survival of fetuses) with that after birth (ie, survival of liveborn neonates), and to assess physical growth and neurodevelopment in...

A new abdominal wall reconstruction strategy for giant omphalocele

Abstract The mortality rate of giant omphalocele has improved; however long-term follow-up has revealed umbilical defects and deformities after primary closure. We herein report the efficacy of a new abdominal wall reconstruction strategy combining a component separation technique with delayed natural and deep umbilicoplasty.

Dressed for success? Silver impregnated nanocrystalline dressing for initial treatment of giant omphalocele.

The purpose of this study was to describe outcomes and resource utilization in patients treated with twice-weekly silver impregnated (SI) nanocrystalline dressings for initial non-operative management of giant omphalocele (GO). A retrospective review of patients with GO treated with SI dressings was undertaken. Clinical parameters, cost, and complications were recorded. Five patients with GO were treated with SI dressings between 2014 and 2016. Clinical characteristic (mean ± SD) included gestational age 36 ± 4 weeks, birth weight 2.6 ± 0.63 kg, GO size 10.2 ± 4.7 cm, ventilator days 7.5 ± 8.7 d, days in NICU 41 ± 20 d, days to full feeds, 30 ± 15 d, and LOS 62 ± 41 d. The average in-hospital cost of SI dressings was $110 CAD/week. This is comparable to daily silver sulfadiazine dressings ($109CAD/week) which were used historically. All patients were discharged with once- or twice-weekly dressing changes. No ruptures occurred. There was one mortality secondary to pulmonary sepsis. For initial non-operative management of GO, twice weekly SI nanocrystalline dressings is safe and effective. Use of SI dressings results in decreased handling of infants, reduced physician and nursing resource utilization, and favourable outcomes. IV (Retrospective Case Series).

Erratum to: Delayed Closure of Giant Omphaloceles in West Africa: Report of Five Cases.

[This corrects the article DOI: 10.1055/s-0037-1599796.].