Omphalocele and Beckwith-Wiedemann Syndrome

2018 ◽  
Author(s):  
Pikulkaew Dachsangvorn
Keyword(s):  
Difficult Airway ◽  
Abdominal Wall ◽  
Chromosomal Abnormalities ◽  
Abdominal Wall Defect ◽  
Perioperative Period ◽  
Prune Belly Syndrome ◽  
Systems Management ◽  
Wall Defect ◽  
Secondary Insults ◽  
Organ Systems

This chapter discusses omphalocele, which is a neonatal abdominal wall defect through which abdominal visceral content herniates. Although omphalocele is similar in gross appearance to gastroschisis, it is more frequently associated with chromosomal abnormalities such as trisomies 13, 15, 18, and 21, and syndromes such as Beckwith-Wiedemann, Rieger’s, and prune belly syndrome. It is also more frequently associated with anomalies in other organ systems. Management of infants with Beckwith-Wiedemann syndrome and omphalocele can be challenging due to the concurrent risk of difficult airway and aspiration. Associated comorbidities also need to be addressed to avoid secondary insults in the perioperative period.

scholarly journals First trimester diagnosis of body stalk anomaly complicated by ectopia cordis

2020 ◽  
Vol 48 (12) ◽  
pp. 030006052098021
Author(s):  
Yi Yang ◽  
Hong Wang ◽  
Zhenpeng Wang ◽  
Xuefeng Pan ◽  
Ying Chen
Keyword(s):  
Abdominal Wall ◽  
Umbilical Cord ◽  
Chromosomal Abnormalities ◽  
Abdominal Wall Defect ◽  
First Trimester ◽  
Nuchal Translucency ◽  
Ectopia Cordis ◽  
Wall Defect ◽  
Abdominal Organs ◽  
Increased Risk

Body stalk anomaly is a rare abnormality characterized by an abdominal wall defect with evisceration of abdominal organs, severe kyphoscoliosis, and a very short or absent umbilical cord. Ectopia cordis (EC) is a rare, lethal anomaly characterized by complete or partial malpositioning of the heart outside of the thorax. A 28-year-old healthy primigravida was referred to our department to undergo a nuchal translucency thickness scan at 12 weeks’ gestation. The scan revealed typical features of body stalk anomaly and EC. Given the lethal condition of the fetus, the patient opted for termination of the pregnancy. Body stalk anomalies, especially those complicated by EC, are universally lethal for the affected fetus. Selective termination should be recommended to avoid possible complications that can arise during pregnancy. Additionally, the future parents should be informed that because the condition is not associated with chromosomal abnormalities, there is no increased risk of recurrence.

Anesthesia for Gastroschisis or Omphalocele Repair

2021 ◽  
pp. 215-220
Author(s):  
Caitlin Aveyard
Keyword(s):  
Abdominal Wall ◽  
Amniotic Membrane ◽  
Chromosomal Abnormalities ◽  
Abdominal Wall Defect ◽  
Anesthetic Management ◽  
Surgical Correction ◽  
Congenital Defects ◽  
Full Thickness ◽  
Wall Defect ◽  
The Right

Gastroschisis and omphalocele are congenital defects in the abdominal wall. These two conditions have different embryologic origins, but anesthetic management is similar for both. Gastroschisis is a full-thickness abdominal wall defect resulting in extrusion of abdominal viscera into the amniotic space without amniotic membrane coverage. The defect is usually to the right of the umbilicus. Omphalocele is a defect in the abdominal wall in the umbilical area in which the extruded abdominal contents (intestine, liver, spleen, bladder) are covered in a thin sac. Infants with omphalocele often have associated syndromes or chromosomal abnormalities. With either gastroschisis or omphalocele, the extruded abdominal contents must be covered and kept moist before surgical correction.

Bio‐Multifunctional Hydrogel Patches for Repairing Full‐Thickness Abdominal Wall Defect

2021 ◽  
pp. 2105614
Author(s):  
Xiangyi Yin ◽  
Yuanping Hao ◽  
Yun Lu ◽  
Dongjie Zhang ◽  
Yaodong Zhao ◽  
...  
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Full Thickness ◽  
Wall Defect

Prenatal Diagnosis of a Fetal Abdominal Eventration: A Rare Congenital Abdominal Wall Defect

2007 ◽  
Vol 23 (2) ◽  
pp. 117-120
Author(s):  
Philippe Roth ◽  
Alain Martin ◽  
Fariz Bawab ◽  
Florence Fellmann ◽  
Didier Aubert ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Wall Defect

A Newborn Who Has an Abdominal Wall Defect

NeoReviews ◽  
2005 ◽  
Vol 6 (3) ◽  
pp. e160-e163
Author(s):  
Valerie Chock
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Wall Defect

Evaluation of TiNi-based wire mesh implant for abdominal wall defect management

2018 ◽  
Vol 4 (2) ◽  
pp. 027010 ◽  
Author(s):  
Dmytro Zaworonkow ◽  
Mykola Chekan ◽  
Katarzyna Kusnierz ◽  
Andrzej Lekstan ◽  
Aniela Grajoszek ◽  
...  
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Wire Mesh ◽  
Wall Defect ◽  
Defect Management ◽  
Mesh Implant

scholarly journals Managing a Colonoscopic Perforation in a Patient with No Abdominal Wall

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Jayan George ◽  
Michael Peirson ◽  
Samuel Birks ◽  
Paul Skinner
Keyword(s):  
Abdominal Pain ◽  
Incisional Hernia ◽  
Plastic Surgery ◽  
Abdominal Wall ◽  
Abdominal Wall Defect ◽  
Colonic Perforation ◽  
Conservative Approach ◽  
Wall Defect ◽  
Surgical History ◽  
History Of

We describe the case of a 37-year-old gentleman with Crohn’s disease and a complex surgical history including a giant incisional hernia with no abdominal wall. He presented on a Sunday to the general surgical on-call with a four-day history of generalised abdominal pain, nausea, and decreased stoma output following colonoscopy. After CT imaging, he was diagnosed with a large colonic perforation. Initially, he was worked up for theatre but following early senior input, a conservative approach with antibiotics was adopted. The patient improved significantly and is currently awaiting plastic surgery input for the management of his abdominal wall defect.

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