Comparison of outcomes of infants with giant omphaloceles over two decades.

BACKGROUND: The purpose of this study was to compare the outcomes of infants with giant omphalocele (GO) born in two different epochs over two decades at a single institution. Specifically, it examined whether the utilization of selective pulmonary vasodilators and extracorporeal membrane oxygenator (ECMO) in the management of pulmonary hypertension in the second epoch were associated with improved outcomes. METHODS: The medical records of all patients diagnosed with GO at a large children's hospital from January 1, 1996 to December 31, 2016 were reviewed and divided into two epochs. Patients were classified as having an isolated GO or GO with minor or major associated anomalies. GO was defined as a defect more than or equal to 5 cm in size and/or liver in the sac. RESULTS: During the study period, 59 infants with GO were identified. The duration of invasive mechanical ventilation was significantly shorter among the survivors from the second epoch (p = 0.03), with none greater than seven days. There were no significant differences in the outcomes of survival to NICU discharge and length of stay (LOS) between infants in the two epochs. CONCLUSIONS: Infants with GO who required invasive mechanical ventilation for more than seven days did not survive in the second epoch. Survival did not improve with use of selective pulmonary vasodilators and ECMO. This information could be shared with families during prenatal and postnatal counselling to facilitate informed decision making regarding goals of care.

Outcomes of patients with exomphalos and associated congenital heart diseases.

INTRODUCTION: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. METHODS: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990-2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). RESULTS: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M:F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). In the ExoMin group; 9% had major CHD (6/64), M:F 42:22, 18% premature and 10% had low birth-weight. The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation (MV) for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. CONCLUSION: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Combined negative pressure wound therapy with irrigation and dwell time and artificial dermis prevents infection and promotes granulation formation in a ruptured giant omphalocele: a case report.

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. We adopted negative pressure wound therapy with irrigation and dwell time (NPWTi-d) for a case of a ruptured giant omphalocele. Artificial membranes, followed by artificial dermis, were used to promote fibrous capsule formation, and then NPWTi-d was used to promote granulation while controlling infection. However, studies have not been conducted regarding NPWTi-d for ruptured giant omphaloceles; hence, we present our treatment experience with NPWTi-d for a giant omphalocele. CASE PRESENTATION: The patient was a boy born at 38 weeks and 3 days of gestation, weighing 1896 g. He was diagnosed with a ruptured giant omphalocele with a total liver and intestine defect hole of 10 cm × 10 cm. The patient underwent silo placement using an artificial mesh, followed by plicating the artificial mesh at 4 days of age. The herniated viscera were gradually reduced into the abdominal cavity; however, the defect size was still large. Hence, a collagen-based artificial dermis was patched on the defect hole. After creating a fresh and smooth granulated tissue, NPWTi-d was applied at 33 days of age to promote granulation and control infection. We used the 3 M™ V.A.C.® Ulta Therapy Unit with 3 M™ VeraFlo™ therapy. NPWTi-d was stopped at 60 days of age when the granulation tissue was well formed including at the artificial dermis site. The wound was managed with prostandin ointment and appropriate debridement, resulting in complete epithelialization at 5 months of age. CONCLUSIONS: Artificial membranes followed by artificial dermis were used to promote a fibrous capsule and artificial dermis granulation, which protects against organ damage. NPWTi-d achieved better control of infection and promoted wound healing. NPWTi-d combined with artificial dermis can effectively treat ruptured giant omphaloceles.

Can perinatal outcomes of fetal omphalocele be improved at a tertiary center in South China?

OBJECTIVE: To assess the efficacy of positive feedback closed-loop management system (PFCMS) protocol in influencing parents' decision about pregnancy continuation in pregnancies diagnosed with omphalocele. METHODS: This was a retrospective cohort study of patients who were diagnosed with fetal omphalocele prior to 20 weeks' gestation by ultrasound and were referred to Fetal Care Center at a mainland Chinese medical center during an 11-year period. Two management strategies were offered during the two stages of the study period: a single consultant with a routine protocol and a multidisciplinary support team with PFCMS, respectively. We analyzed the two protocols influencing parents' decision about pregnancy continuation. RESULTS: Forty-nine patients diagnosed with fetal omphalocele were included in this study. In Group A including 16 patients with routine protocol during the first stage of the study period, the majority opted for termination, and only five continued the pregnancy. In Group B including 33 patients with PFCMS during the second stage of the study period, less than one third chose TOP, and 23 ended in live births. There was a significantly lower TOP rate in patients treated with the PFCMS protocol. CONCLUSION: The PFCMS protocol may be an efficient approach in managing pregnancies complicated by omphalocele, which may help in preventing unnecessary pregnancy terminations.

Symptomatic Meckel's Diverticulum in pediatrics. / Divertículo de Meckel sintomático en pediatría.

INTRODUCTION: Meckel's diverticulum (MD) is the remnant of the vitelline duct (VD) also called omphalomesente ric duct and it is considered the most frequent gastrointestinal malformation. Most of the cases are asymptomatic and the diagnosis of this type is always a challenge. OBJECTIVE: To describe 3 sympto matic presentations of MD and to discuss its symptoms, signs, and possible diagnostic-therapeutic tools. CLINICAL CASES: Case 1: A six-month-old patient with obstructive bowel syndrome. In explo ratory laparotomy, an MD was identified with a mesodiverticular band causing an internal hernia. Case 2: A three-year-old patient presenting with digestive hemorrhage and severe anemia requiring blood transfusion. Upper gastrointestinal endoscopy did not show bleeding origin. Due to persis tent melena, the patient required a new blood transfusion. An Abdomen/pelvis tomography scan was performed, showing a suspicious image of MD which was confirmed by laparotomy. Case 3: A newborn with prenatal anencephaly and omphalocele diagnosis. In immediate care of the newborn, meconium evacuation from the umbilical defect was noticed. It was managed as ruptured omphalo cele, installing a bowel silo bag. In primary closure, the permeability of the omphalomesenteric duct was confirmed. An intestinal en bloc resection and anastomosis were performed in all 3 cases. The last one developed an anastomosis leakage resulting in a terminal ileostomy. CONCLUSION: MD, frequently asymptomatic, is often overlooked as a differential diagnosis of abdominal emergencies in children. When suspecting DM with gastric ectopic mucosa, Tc-99m pertechnetate scintigraphy should be performed as a diagnostic procedure of choice, according to each case.

Major abdominal wall defects in the low- and middle-income setting: current status and priorities.

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30-100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.

Contemporary practice and perceptions surrounding the management of asymptomatic umbilical hernias in children: A survey of the American Pediatric Surgical Association.

PURPOSE: To explore variation in perceptions regarding the natural history of asymptomatic umbilical hernias, and to characterize the influence of clinical and nonclinical factors on decision-making surrounding timing of repair. METHODS: This was a survey of the American Pediatric Surgical Association. Branching logic and Likert scale questions were used to explore perceptions surrounding natural history (risk of complications and likelihood of spontaneous closure), preferred age for repair, and influence of anatomic, caregiver, sociodemographic, and biological factors on operative timing. RESULTS: 44% of members completed the survey (371/846). The most common age respondents would consider elective repair was 3 years (37%), although the majority preferred to wait until 4 or 5 years (54%). Most respondents estimated a <1% risk of complications for unrepaired defects, and much greater variability was found in the perceived likelihood of spontaneous closure over time. Decision-making surrounding operative timing was most influenced by anatomic factors (larger defects, proboscoid changes, and interval growth) and parental anxiety surrounding need for emergency surgery, cosmesis, and stigma of parental neglect. CONCLUSION: Practice and perceptions surrounding management of asymptomatic umbilical hernias vary widely. More robust epidemiological data are needed to define the likelihood of spontaneous closure in the context of age and physical exam findings. Collaborative efforts between surgeons and referring providers are also needed to optimize management of caregiver anxiety and expectations surrounding need for surgical referral and repair. LEVEL OF EVIDENCE: Level V (expert opinion).

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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Impact of Breast Milk, Respiratory Insufficiency, and Gastroesophageal Reflux Disease on Enteral Feeding in Infants With Omphalocele.

OBJECTIVES: The aim of this study was to document the process of achieving full enteral feeding in infants with omphalocele and to identify factors that affect feeding success. METHODS: After institutional review board approval (study no. 5100169), 123 infants with omphalocele, born between 1993 and 2011 were reviewed. Mortalities were excluded. All survivors had complete follow-up. Variables suspected to impact enteral feeding in infants with non-giant versus giant omphalocele were compared. Independent t test, Mann-Whitney, and χ test were used. Regression evaluated for variable independence. RESULTS: Of 123 infants with omphalocele, 97 (79%) survived, 62/97 (64%) had non-giant, and 35/97 (36%) giant omphalocele. For survivors, the mean gestational age was 37 ±â€Š4 weeks with median follow-up of 4.4 years (range: 1.4-7.4 years). The median time to full feeds was 4 days (range: 0-85 days) for non-giant versus 8 days (range: 1-96 days) for giant, a significant difference (P < 0.01). Breast milk significantly decreased time to full feeds independent of omphalocele size. Giant omphalocele infants had a significantly higher incidence of respiratory insufficiency at birth (P < 0.01) and sac rupture (P = 0.02), but fewer chromosomal anomalies (P = 0.04). Respiratory insufficiency at birth (P < 0.01) and gastroesophageal reflux disease (P < 0.01) independently delayed feeding in omphalocele infants. CONCLUSIONS: Infants with non-giant omphalocele can achieve full enteral feeds within the first week of life, but giant omphalocele infants require significantly more time. Breast milk independently promotes feeding success whereas gastroesophageal reflux disease and respiratory insufficiency at birth independently delay feeding in infants with omphalocele.

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.

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.

Prenatal diagnosis and management of omphalocele.

Omphalocele (exomphalos) is one of the most common abdominal wall defects. The size of the defect and the severity of the associated anomalies determine the overall morbidity and mortality. Routine prenatal screening and diagnosis of the abdominal wall defect and concurrent anomalies is important as it allows for effective prenatal counseling and optimal perinatal management. The purpose of this article is to discuss the approach to prenatal diagnosis and management of omphalocele.

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.

Nonoperative management of giant omphalocele leading to early fascial closure.

PURPOSE: We describe our series of giant omphalocele patients treated with a serial taping method for gradual reduction of the abdominal contents and early fascial closure. METHODS: Between 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. RESULTS: Mean 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). CONCLUSIONS: Serial 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 STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.

Omphaloceles in combination with a self-made hemispherical cushion: a report of 12 cases.

The aim of this study was to discuss the curative effect of applying "capsule-reserved normal saline bag and self-made hemi-spherical cushion oppression" for treating giant omphaloceles. Twelve patients with giant omphaloceles who were admitted to our hospital between January 2008 and June 2016 were selected for treatment as follows: a capsule-reserved normal saline bag was used to promote the gradual return of the abdominal contents into the abdominal cavity in phase I, and a self-made hemi-spherical cushion was used for compression combined with a local dressing change in phase II to treat the giant omphaloceles without surgical treatment. All 12 patients in this group were cured, and after follow-up visits for >10 months, they had no abdominal infections, wound disruption, intestinal obstruction, or other complications, and their growth was normal. Two patients had abdominal hernias, and they recovered after herniorrhaphies. Giant omphaloceles in newborns were treated in stages, and in phase II, non-surgical treatment was applied, which was easily performed with a smaller wound, low cost, an obvious curative effect, and higher safety and effectiveness.

Formal saline versus honey as escharotic in the conservative management of major omphaloceles.

BACKGROUND: The use of honey as an escharotic agent in the conservative management of omphalocele major has not been widely explored in spite of its proven benefits in chronic wound management. We explored the use of local honey as an escharotic agent by comparing its use with 2.5% formal saline in the conservative management of major omphaloceles at the Lagos University Teaching Hospital, Lagos, Nigeria. METHODS: From January 2006 to December 2009, 43 consecutive newborns with intact omphalocele major were alternately assigned into either Honey (H) or formal saline (FS) group. The membrane cover of each omphalocele was painted with the allotted group agent once every 48 h. The occurrence of faecal fistulas, rupture of eschar, intestinal obstruction as well as the mean duration of full wound healing, infection rates and overall mortality rates were compiled for the two groups. RESULTS: Eighteen newborns were assigned to the FS group while 25 others were prospectively enrolled into the H group. The age, sex and weight of newborns in both groups at presentation were comparable. Three omphaloceles (16.7%) ruptured and eviscerated among the FS group during the study while 1 (4%) of these occurred in the H group. Four (22.2%) cases of faecal fistula occurred in the FS group while none was recorded in the H group. One (5.6%) patient in the FS group developed small bowel stricture. This was not recorded in the honey group. Overall, there were 8 (44.4%) complications in the FS group and 1 (4%) in the H group. There was no statistical difference between the two groups concerning the occurrence of fistulae, sac rupture or bowel stricture. However, overall number of complications was statistically more in the FS group when compared to the H group (P < 0.05). Wounds in the H group healed within a mean period of 34.4 ± 4.9 days while those in the FS group healed within a mean period of 45.7 ± 6.8 days P < 0.01). CONCLUSION: Honey is a good escharotics agent in the conservative management of major omphaloceles. Honey promotes faster healing and unlike 2.5% formal saline, is not significantly associated with faecal fistulas, rupture or bowel stricture.

Omphaloceles in combination with a self-made hemispherical cushion: a report of 12 cases

The aim of this study was to discuss the curative effect of applying "capsule-reserved normal saline bag and self-made hemi-spherical cushion oppression" for treating giant omphaloceles. Twelve patients with giant omphaloceles who were admitted to our hospital between January 2008 and June 2016 were selected for treatment as follows: a capsule-reserved normal saline bag was used to promote the gradual return of the abdominal contents into the abdominal cavity in phase I, and a self-made hemi-spherical cushion was used for compression combined with a local dressing change in phase II to treat the giant omphaloceles without surgical treatment. All 12 patients in this group were cured, and after follow-up visits for >10 months, they had no abdominal infections, wound disruption, intestinal obstruction, or other complications, and their growth was normal. Two patients had abdominal hernias, and they recovered after herniorrhaphies. Giant omphaloceles in newborns were treated in stages, and in phase II, non-surgical treatment was applied, which was easily performed with a smaller wound, low cost, an obvious curative effect, and higher safety and effectiveness.

Hernia umbilical en pacientes cirróticos con ascitis: ¿tratamiento conservador o quirúrgico? / Umbilical hernia in liver cirrhosis with ascites: conservative or surgical treatment?

Resumen La ascitis es la complicación más común en los pacientes con cirrosis hepática. Las hernias umbilicales (HU) ocurren en 20% de estos pacientes y 40% en aquellos con ascitis severa. La HU ocurre debido al aumento de presión intraabdominal, al debilitamiento de la fascia abdominal y la pérdida de masa muscular. Además, tienen tendencia a aumentar rápidamente y presentar alto riesgo de complicaciones que amenazan la vida del paciente. El tratamiento de la HU no complicada es controversial, tanto el manejo quirúrgico (herniorrafia) como el manejo conservador (control de ascitis) presentan alta tasa de complicaciones, en consecuencia incrementa la morbimortalidad. Actualmente, se recomienda herniorrafia umbilical con previo control de la ascitis en el manejo de HU no complicada, reduce el riesgo de infección de herida operatoria, evisceración, drenaje de ascitis, peritonitis y reduce hasta 41% la recidiva de HU. El éxito de este enfoque también depende del grado de disfunción hepática. El tratamiento de la HU complicada es quirúrgico (herniorrafia sin malla), con menor tasa de mortalidad respecto al manejo conservador. Estudios revelan ventajas de la herniorrafia umbilical laparoscópica (mínimamente invasiva y sin tensión) en comparación a la cirugía abierta, sin embargo, aún no hay evidencia al respecto.

Ascites is the most common complication in patients with liver cirrhosis. Umbilical hernias (HU) occur in 20% of these patients and 40% in those with severe ascites. HU occurs due to increased intra-abdominal pressure, weakening of the abdominal fascia and loss of muscle mass. In addition, they have a tendency to enlarge rapidly and present high risk of complications that threaten the patient's life. The treatment of the uncomplicated HU is controversial, both the surgical management (herniorrhaphy) and the conservative management (control of ascites) present high rate of complications, consequently high morbidity and mortality. Currently, umbilical herniorrhaphy is recommended with prior control of ascites in uncomplicated HU management, it reduces the risk of surgical wound infection, evisceration, ascites drainage, peritonitis, and it reduces up to 41% of HU recurrence. The success of this approach also depends on the degree of liver dysfunction. The treatment of complicated HU is surgical (herniorrhaphy without mesh), with lower mortality rate compared to conservative management. Studies reveal advantages of umbilical herniorrhaphy laparoscopy (minimally invasive and stress-free) compared to open surgery; however there is still no evidence about it.

Umbilicus and its extensive clinical repertoire.

The umbilicus is involved in a wide range of abnormalities in infants and children. The most severe are evident at birth and include exomphalos (omphalocele) and gastroschisis, both of which can be life-threatening but are easy to diagnose. Exomphalos is often associated with other congenital abnormalities, whereas the associated problems in gastroschisis are largely confined to the gut. Infection of the umbilicus in the neonate presents as omphalitis. The causes of a moist umbilicus following separation of the umbilical stump are multiple, from the relatively minor umbilical granuloma or ectopic bowel mucosa to the more significant patent urachus that leaks urine. Patency of the entire vitello-intestinal (omphalomesenteric) tract allows air and faecal fluid to drain through the umbilicus. The clinical manifestations of persistence of the vitello-intestinal tract vary markedly according to which part remains: clinical presentations include melaena and anaemia, closed-loop bowel obstruction and Meckel diverticulitis. An umbilical hernia occurs when the umbilical cicatrix fails to close. On the other hand, the umbilicus has its uses, which range from being a route for intravenous access in the neonate to being a convenient point of access in laparoscopic surgery.