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Aesthetic Plastic Surgery Apr 2022
Review
Topics: Hernia, Umbilical; Humans; Plastic Surgery Procedures; Umbilicus
PubMed: 34331095
DOI: 10.1007/s00266-021-02498-8 -
Pediatric Surgery International Nov 2021The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim...
PURPOSE
The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients.
METHODS
This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register.
RESULTS
The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%.
CONCLUSION
The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high.
TYPE OF STUDY
National register study LEVEL OF EVIDENCE: III.
Topics: Child; Female; Hernia, Umbilical; Humans; Infant, Newborn; Male; Mass Screening; Pregnancy; Prenatal Diagnosis; Prevalence; Sweden
PubMed: 34392395
DOI: 10.1007/s00383-021-04978-z -
The Journal of Maternal-fetal &... Aug 2022An accurate estimated fetal weight (EFW) calculated with traditional formulae in cases of abdominal wall defects (AWDs) can be challenging. As a result of reduced...
BACKGROUND
An accurate estimated fetal weight (EFW) calculated with traditional formulae in cases of abdominal wall defects (AWDs) can be challenging. As a result of reduced abdominal circumference, fetal weight may be underestimated, which could affect prenatal management. Siemer et al. proposed a formula without the use of abdominal circumference, but it is not used in our protocols yet.
OBJECTIVES
Our aim was to evaluate the correlation of EFW and birth weight in fetuses with AWD by using Hadlock 1, Hadlock 2, and Siemer et al.'s formulae. Our secondary goal was to evaluate how often fetuses classified as small for gestational age (SGA) were in fact SGA at birth.
STUDY DESIGN
This was a retrospective cohort study of gestations complicated by gastroschisis and omphalocele at two tertiary-care centers in Brazil and Italy during an 8-year period. Of a total of 114 cases, 85 (44 cases of gastroschisis and 41 cases of omphalocele) met our criteria.
RESULTS
The last prenatal scan was performed 5.2 (±4.1) days before birth. The mean gestational age at birth was 37.2 (±1.8) weeks. Correlation of EFW with birth weight was calculated with the three formulae with and without adjustment for weight gain between scan and birth, with the use of the Spearman coefficient. The correlation between EFW and weight at birth was positive according to all three formulae for the infants with gastroschisis. This finding was not confirmed in the infants with omphalocele. All formulae overestimated the number of SGA cases: although only 17.6% of fetuses were actually SGA at birth, the Hadlock formulae had classified nearly 35% of them as SGA, and Siemer et al.'s formula, 15.3%.
CONCLUSION
All three formulae yielded a good correlation between EFW in the last scan and birth weight in the infants with gastroschisis but not for those with omphalocele. Cases of SGA were overestimated.
Topics: Birth Weight; Female; Fetal Growth Retardation; Fetal Weight; Fetus; Gastroschisis; Gestational Age; Hernia, Umbilical; Humans; Infant, Newborn; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 32814485
DOI: 10.1080/14767058.2020.1808615 -
Andes Pediatrica : Revista Chilena de... Feb 2021Meckel's diverticulum (MD) is the remnant of the vitelline duct (VD) also called omphalomesente ric duct and it is considered the most frequent gastrointestinal...
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.
Topics: Blood Transfusion; Child, Preschool; Diagnosis, Differential; Gastrointestinal Hemorrhage; Hernia, Umbilical; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meckel Diverticulum; Photography
PubMed: 34106190
DOI: 10.32641/andespediatr.v92i1.2470 -
Minerva Obstetrics and Gynecology Apr 2023Omphalocele is associated with many aneuploidies, deletions and congenital anomalies. This study evaluates pregnancies diagnosed with omphalocele and its relevance to...
BACKGROUND
Omphalocele is associated with many aneuploidies, deletions and congenital anomalies. This study evaluates pregnancies diagnosed with omphalocele and its relevance to concomitant genetic disorders.
METHODS
The data of patients with the intrauterine diagnosis of omphalocele who had invasive diagnostic testing performed between January 2017 and January 2020 were evaluated retrospectively. The traditional karyotype analysis was performed to prenatal diagnosis for all fetuses. During the study period, all patients were scanned via ultrasonography by an experienced perinatologist, prenatally.
RESULTS
We evaluated 22 cases of omphalocele whose genetic testing results were available. The mean maternal age was 25 (18-41) years. The median gestational week at diagnosis was 13 (11-22). Invasive genetic testing revealed aneuploidy in 7 patients (31.8%), 2 with trisomy 13 (9.1%), and 5 with trisomy 18 (22.8%). There were 5 fetuses (22.7%) that had extracorporeal liver: 1 had trisomy 18 (20%), 1 had trisomy 13 (20%), and the other 3 fetuses had a normal karyotype (60%). Further, 14 (63.6%) pregnancies were terminated: 4 had trisomy 18 (28.6%), 1 had trisomy 13 (7.1%), and 9 of the terminated pregnancies (64.3%) had additional congenital anomalies. There were 4 infants who died (50%) born from 8 patients who decided to continue with their pregnancy. The omphalocele sac of 1 infant spontaneously regressed in the ensuing weeks of pregnancy who is now 1 year old.
CONCLUSIONS
The chromosomal abnormalities presented in up to 31.8% of cases diagnosed with omphalocele. Moreover, for cases with normal genetic testing results, the propensity for additional structural defects was high and the prognosis remains poor. Counseling parents to consider their option of terminating the pregnancy is appropriate.
Topics: Pregnancy; Female; Humans; Adult; Infant; Pregnancy Outcome; Hernia, Umbilical; Trisomy 18 Syndrome; Retrospective Studies; Trisomy 13 Syndrome; Ultrasonography, Prenatal; Aneuploidy; Karyotype; Fetus
PubMed: 37052892
DOI: 10.23736/S2724-606X.21.04917-4 -
Fetal Diagnosis and Therapy 2023Abdominal wall defects (AWDs) interfere with postnatal respiratory parameters. We aimed to evaluate lung volume (LV) in fetuses with AWD using three-dimensional (3D)... (Observational Study)
Observational Study
INTRODUCTION
Abdominal wall defects (AWDs) interfere with postnatal respiratory parameters. We aimed to evaluate lung volume (LV) in fetuses with AWD using three-dimensional (3D) ultrasound (US) and to correlate AWD with the type (omphalocele and gastroschisis) and size of the defect and neonatal morbidity and mortality.
METHODS
This prospective observational study included 72 pregnant women with fetuses with AWD and a gestational age <25 weeks. The data on abdominal volume, 3D US LV, and herniated volume were acquired every 4 weeks up to 33 weeks. LV was compared with normal reference curves and correlated with abdominal and herniated volumes.
RESULTS
Omphalocele (p < 0.001) and gastroschisis (p < 0.001) fetuses had smaller LV than normal fetuses. LV was positively correlated with abdominal volume (omphalocele, r = 0.86; gastroschisis, r = 0.88), whereas LV was negatively correlated with omphalocele-herniated volume/abdominal volume (p < 0.001, r = -0.51). LV was smaller in omphalocele fetuses that died (p = 0.002), were intubated (p = 0.02), or had secondary closure (p < 0.001). In gastroschisis, a smaller LV was observed in fetuses discharged using oxygen (p = 0.002).
CONCLUSION
Fetuses with AWD had smaller 3D LV than normal fetuses. Fetal abdominal volume was inversely correlated with LV. In omphalocele fetuses, a smaller LV was associated with neonatal mortality and morbidity.
Topics: Infant, Newborn; Pregnancy; Humans; Female; Infant; Gastroschisis; Hernia, Umbilical; Abdominal Wall; Digestive System Abnormalities; Fetus
PubMed: 37379809
DOI: 10.1159/000531594 -
BMC Pediatrics Nov 2023The purpose of this study was to explore echocardiographic parameters of the left ventricle (LV) in relation to the outcomes of omphalocele neonates with pulmonary...
PURPOSE
The purpose of this study was to explore echocardiographic parameters of the left ventricle (LV) in relation to the outcomes of omphalocele neonates with pulmonary hypertension (PH).
METHODS
This retrospective study was conducted among omphalocele patients with PH born from 2019 to 2020. Patients in this study did not have additional severe malformations or chromosomal aberrations. Patients who died under palliative care were excluded. The echocardiographic parameters of LV were obtained within 24 h after birth. Clinical and outcomes data were recorded, echocardiograms evaluated for left ventricular internal dimension in end-diastole (LVIDd), end-diastolic volume (EDV), stroke volume (SV) and cardiac output index (CI), among others.
RESULTS
There were 18 omphalocele newborns with PH, of whom 14 survived and 4 died. Both groups were comparable in the baseline characteristics. Non-survival was associated with a smaller LV [LVIDd (12.2 mm versus15.7 mm, p < 0.05), EDV (3.5 ml versus 6.8 ml, p < 0.05)] and with worse systolic function [SV (2.3 ml versus 4.2 ml, p < 0.05), and CI (1.7 L/min/m versus 2.9 L/min/m, p < 0.01)].
CONCLUSION
In the cohort of omphalocele patients with PH, lower LVIDd, EDV, SV and CI were associated with mortality.
LEVEL OF EVIDENCE
Level III.
Topics: Infant, Newborn; Humans; Heart Ventricles; Hernia, Umbilical; Retrospective Studies; Diastole; Echocardiography; Hypertension, Pulmonary
PubMed: 37990192
DOI: 10.1186/s12887-023-04418-y -
Journal of Pediatric Surgery May 2021Giant omphaloceles can be a challenge for pediatric surgeons and neonatologists worldwide. It is a rare and low-frequency congenital anomaly with no standardized...
INTRODUCTION
Giant omphaloceles can be a challenge for pediatric surgeons and neonatologists worldwide. It is a rare and low-frequency congenital anomaly with no standardized management schemes or treatment protocols. Over the past few decades, we have developed a simple and efficient staged management for giant omphaloceles that allows definitive closure in the neonatal period, the results of which we outline in this report.
MATERIAL AND METHODS
With IRB approval, a retrospective and multicentric cohort study was carried out between 1994 and 2019 with patients with giant omphalocele defined as an abdominal wall defect greater than 5 cm in diameter and/or that contains more than 50% of the liver within the sac. We included all patients managed with the nonsurgical silo technique. Data on demographics, gestational age, associated malformations, amnion reduction and inversion time, anatomic closure, requirement of a mesh, intra- and post-silo complications, mortality and follow-up were collected. The technique consists of the construction of a silo with an adhesive hydrocolloid dressing (Duoderm) to achieve an omphalocele staged-reduction until complete abdominal reintegration of the liver and bowel preservation of the amnion sac. This also enables the simulation of abdominal closure before definitive surgical closure, being managed in the neonatal intensive care unit (NICU).
RESULTS
Forty patients, 21 of whom were female, were managed with this technique. The average weight was 2900 gs (890-3900), and the median gestational age was 38 weeks (28-40). In total, 37.5% of cases had an associated comorbidity. The average silo reduction time was 7.3 days (0-35), the average time of amnion inversion was 5 days (2-9), and the average time to closure was 14.6 days (6-38). Anatomical closure was achieved in 95% of cases. In 4 patients, an absorbable mesh was used to reinforce the anatomical closure, and in 2 patients (5%), a mesh (Dualmesh) was required to achieve an abdominal closure. There was no mortality associated with this nonsurgical silo technique. The average follow-up time was 60 (6 - 288) months.
CONCLUSION
The staged silo management of giant omphalocele in this series is safe and effective and reduces the time to closure and potential morbidity and mortality compared with traditional surgical or medical management.
Topics: Female; Humans; Infant, Newborn; Bandages; Cohort Studies; Hernia, Umbilical; Retrospective Studies; Male
PubMed: 33341259
DOI: 10.1016/j.jpedsurg.2020.12.003 -
Aesthetic Surgery Journal Jun 2021Different methods of performing full abdominoplasty and umbilical hernia (UH) repair simultaneously have been proposed. (Review)
Review
BACKGROUND
Different methods of performing full abdominoplasty and umbilical hernia (UH) repair simultaneously have been proposed.
OBJECTIVES
The aim of this study was to review and compare UH repair outcomes and umbilical stalk survival.
METHODS
A literature research was performed through 28 December 2019. Other hernia repairs and mini-abdominoplasty (without umbilical transposition) were excluded. The primary outcomes analyzed were rates of UH recurrence, mesh infection, and umbilical necrosis.
RESULTS
Six studies were included (5 retrospectives series, 1 case report). Hernia was repaired by an open approach (3 studies, 28 patients) or a laparoscopic approach (3 studies, 67 patients). UH repair consisted of mesh placement in the intraperitoneal or retromuscular/preperitoneal plane, or suture technique in the intraperitoneal plane. No hernia recurrence, mesh infection, or umbilical necrosis was described.
CONCLUSIONS
Both open and laparoscopic approaches to simultaneous abdominoplasty and UH repair seem to be safe based on the rates of umbilical stalk vascularization, hernia recurrence, and mesh infection. However, more well-designed studies are needed to prove this hypothesis.
Topics: Abdominoplasty; Hernia, Umbilical; Herniorrhaphy; Humans; Recurrence; Retrospective Studies; Surgical Mesh
PubMed: 33617639
DOI: 10.1093/asj/sjab094 -
Urology May 2021Structural anomalies of the female reproductive tract, known as Mullerian anomalies, can occur in isolation or in association with anomalies of other organ systems. Due... (Review)
Review
Structural anomalies of the female reproductive tract, known as Mullerian anomalies, can occur in isolation or in association with anomalies of other organ systems. Due to shared embryology, the most common association in up to 40% of patients is with renal, ureteral, and bladder anomalies. Affected girls can have a wide range of genitourinary symptoms with urologists playing an integral role in their diagnosis and treatment. To facilitate the recognition and management of these conditions, we provide a review of Mullerian anomalies including the embryology, classifications, syndromes, evaluation, and treatments with attention to their urologic applicability.
Topics: 46, XX Disorders of Sex Development; Anorectal Malformations; Anus, Imperforate; Congenital Abnormalities; Female; Genitalia, Female; Hernia, Umbilical; Humans; Mullerian Ducts; Scoliosis; Urinary Tract; Urogenital Abnormalities
PubMed: 32387292
DOI: 10.1016/j.urology.2020.04.088