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Congenital Exomphalos

Congenital Omphalocele


Presentation

  • This report details the presentation of congenital fistulation of a Meckel's diverticulum to the surface of an exomphalos minor. Links Authors Show Affiliations, Department of Paediatric Surgery, Guy's Hospital, London.[unboundmedicine.com]
  • Specific sections deal with Physiology and Pathophysiology, Clinical Presentation of Disease, Clinical Manifestations and Management (discussing the Mouth and Esophagus, The Stomach and Duodenum, The Intestine, and The Pancreas), Diagnosis of Gastrointestinal[books.google.ro]
  • Fully revised and up-dated, the book retains the overall format of previous editions, presenting the same practical approach to the examination of both fetuses and perinatal deaths.[books.google.ro]
  • On examination, it was observed and diagnosed that the baby presented with extrusion of multiple abdominal organs through a midline breach in the continuity of anterior abdominal wall [Figure 1].[ijsronline.net]
  • On examination, it was observed and diagnosed that the baby presented with extrusion of multiple abdominal organs through a midline breach in the continuity of anterior abdominal wall [Figure 1] .[ijsronline.net]
Weakness
  • Exomphalos is a weakness of the baby’s abdominal wall where the umbilical cord joins it.[dailymail.co.uk]
  • Exomphalos is a weakness of anterior abdominal wall, which leads to protrusion of abdominal content but within a sac contrary to the case we are reporting.[ijsronline.net]
  • A hernia due to an imperfect closure or weakness of the umbilical ring. It appears as a skin-covered protrusion at the umbilicus during crying, coughing, or straining. The hernia generally consists of omentum or small intestine.[icd10data.com]
Gangrene
  • The herniated contents comprised liver and dark gangrenous loop of intestine [Figure 2]. The breach was about 8 cm in length and stretched to a width of about 6 cm.[ijsronline.net]
  • The herniated contents comprised liver and dark gangrenous loop of intestine [Figure 2] . The breach was about 8 cm in length and stretched to a width of about 6 cm.[ijsronline.net]
  • K42.1 Umbilical hernia with gangrene K42.9 Umbilical hernia without obstruction or gangr...[icd10data.com]
Multiple Congenital Anomalies
  • congenital anomalies(MCA)(26 cases).Malformations of the musculo-skeletal system,the urogenital system,the cardiovascular system,and the central nervous system were the most common other congenital anomalies occurring in patients with MCA.[ashg.org]
Sighing
  • […] coverage of heart anomalies, including arrhythmias and early fetal echocardiography The author’s mission continues to be to provide guidance on how to quickly recognize and diagnose congenital fetal anomalies, beginning at the beginning with ultrasound sigh[books.google.com]
Neglect
  • […] awareness and proper diagnosis yearns for more efficient training of health practitioner, proper reporting, and increased availability of diagnostic tools such as advanced ultrasonographic examination and availability of genetic screening in several neglected[ijsronline.net]
  • I am glad I get to spend each Valentine’s Day with you Sometimes we neglect to State how much we adore one another, but Valentine’s Day is a reminder of just how much you really mean to me personally. Happy Valentine’s Day![happyvalentinesdayworldwide.com]

Workup

  • A genetic workup is justified in most cases. Pacilli et al 2 reported associated malformations in up to 50% of infants with GO.[dovepress.com]
Karyotype Abnormal
  • With associated anomalies, mortality reaches 80% and with a karyotypic abnormality or severe cardiac defect the mortality is near 100% 22,50. Click here to take the quiz. References Kirk EP, Wan RM.[iame.com]
Candida
  • Because of its inert texture and its constant exposure to the air, it constitutes a prime environment for bacterial colonization and mainly for the candidas that swarm rapidly to the abdominal cavity through the anchor points of the membrane, the appearance[dralami.edu]

Treatment

  • Babies with birth defects may need surgery or other medical treatments. Today, doctors can diagnose many birth defects in the womb. This enables them to treat or even correct some problems before the baby is born.[patientsville.com]
  • […] before and after radioiodine treatment.[gov.uk]
  • It is indicated for patients after conservative and surgical treatment of the internal organs diseases.[bookinghealth.com]
  • Information about a therapy, service, product or treatment does not in any way endorse or support such therapy, service, product or treatment and is not intended to replace advice from your doctor or other registered health professional.[betterhealth.vic.gov.au]
  • The ultimate judgement regarding a particular clinical procedure or treatment must be made by the clinician in the light of the clinical data presented by the patient and the diagnostic or treatment options available.[uhs.nhs.uk]

Prognosis

  • Very sparse numbers of patients with such defect were associated with good prognosis (8%) [1]. The etiopathogenesis of omphalocele and gastroschisis are debatable and have different theories for origin.[ijsronline.net]
  • Very sparse numbers of patients with such defect were associated with good prognosis (8%) [1] . The etiopathogenesis of omphalocele and gastroschisis are debatable and have different theories for origin.[ijsronline.net]
  • Prognosis Prognosis depends on: A. Classification / size of the omphalocele 2.[herryyudha.blogspot.com]
  • Smaller omphaloceles are thought to carry a worse prognosis due to increased risk of associated abnormalities.[radiopaedia.org]
  • Prognosis At the severe end of the spectrum, patients are at risk of early death due to complications arising from hypoglycemia, prematurity, cardiomyopathy, macroglossia, or tumors. In patients who survive childhood, prognosis is generally good.[orpha.net]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Clustering of cases (number and severity) suggests a complex etiology, such as environmental factors acting upon susceptible hosts.[emedicine.medscape.com]
  • Etiology BWS is caused by various epigenetic and/or genetic alterations that dysregulate imprinted genes on chromosome 11p15.5. Molecular subgroups are associated with different recurrence risks and different clinical findings (e.g. tumor risks).[orpha.net]
  • Etiology Embryology etiology of omphalocele is considered due to failure of fusion of cephalic fold of the abdominal wall, caudal, and lateral to the central defect that resulted in the inhibition of lateral wall and folds occur in the midline omphalocele[herryyudha.blogspot.com]
  • Etiology Some suggest that the formation of an omphalocele may be due to the failure of the medial segments of the two lateral embryonic wall folds to fuse at approximately 3-4 weeks post conception.[radiopaedia.org]

Epidemiology

  • Additionally, epidemiological characteristics of this malformation were described. We observed 516 cases of exomphalos among 3,248,954 live births.[nature.com]
  • An EU review of available evidence from epidemiological studies and case reports concluded there was evidence that carbimazole is associated with an increased risk of congenital malformations, especially when administered in the first trimester of pregnancy[gov.uk]
  • It is not having geographic or racial predilection. [6] In a study conducted in Northern Ireland, the incidence was 1 in 6700. [7] Epidemiologic data complied over last 40 years in United States showed that incidence of omphalocele remained constant and[atmph.org]
  • Unlike exomphalos, there is no covering membrane. [ 2 ] Epidemiology The frequency of abdominal wall defects is complicated by the fact that the pregnancy may be ended by elective termination.[patient.info]
  • Epidemiology, management and outcome of gastroschisis in Sub-Saharan Africa: Results of an international survey. Afr J Paediatr Surg. 2015 Jan-Mar;12(1):1-6. 16. Ford K, Poenaru D, Moulot O, Tavener K, Bradley S, Bankole R, et al.[jpss.eu]
Sex distribution
Age distribution

Pathophysiology

  • Specific sections deal with Physiology and Pathophysiology, Clinical Presentation of Disease, Clinical Manifestations and Management (discussing the Mouth and Esophagus, The Stomach and Duodenum, The Intestine, and The Pancreas), Diagnosis of Gastrointestinal[books.google.ro]
  • It provides essential clinical and pathophysiological information and discusses the pathogenesis of abnormalities as a basis for appropriate methods of investigation.[books.google.ro]

Prevention

  • Centers for Disease Control and Prevention[patientsville.com]
  • Bird TM, Robbins JM, Druschel C, Cleves MA, Yang S, Hobbs CA, & the National Birth Defects Prevention Study. Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study.[cdc.gov]
  • - 0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis[codelay.com]
  • A broad-spectrum antibiotic should be administered to prevent infection.[patient.info]
  • After stabilization of the newborn, the exposed abdominal contents should be covered with a warm sterile moist dressing to prevent temperature and fluid losses.[jmedscindmc.com]

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