scholarly journals Congenital Abdominal Wall Defects: A Topic Review and a Case Report of a Lethal Polymalformation Syndrome with a Giant Abdominoschisis

2020 ◽  
Vol 20 (3) ◽  
pp. 133-137
Author(s):  
V. Bartos
Keyword(s):  
Abdominal Wall ◽  
Body Wall ◽  
Wide Spectrum ◽  
Postnatal Life ◽  
Chromosomal Anomalies ◽  
Abdominal Wall Defects ◽  
Genetic Syndromes ◽  
Lumbosacral Region ◽  
Female Fetus ◽  
The Right

AbstractAbdominal wall defects (AWDs) represent a wide spectrum of congenital anomalies which are characterized by a herniation of abdominal organs through a pathologic opening in the abdominal region. The author describes a 29-year-old pregnant woman who was prenatally found to have a living female fetus with a giant omphalocele and a spinal deformation in the lumbosacral region. An evisceration of the abdominal contents into the amnionic cavity was visible. Based on USG findings the limb-body wall complex was suggested. An amniocentesis was performed and a genetic testing identified a chromosome 17q23.1–q23.2 duplication. As the fetal anomaly was considered incompatible with life, a medical termination of the pregnancy was carried out. Postmortem examination of the fetus revealed a huge abdominoschisis with a complete evisceration of the liver, stomach, small and large intestines, spleen, and the right kidney. A severe left-sided scoliosis deformity of the spine with crooked trunk was evident. The gross findings shared the features of omphalocele and limb-body wall complex with no postnatal life expectancy. Most fetal AWDs have poor prognosis. The pathology of these conditions differ greatly and require specific prenatal evaluation and pregnancy management for each entity. Accurate assessment of AWDs will allow the identification of isolated forms with better clinical outcomes compared to associated multiple malformations with chromosomal anomalies or genetic syndromes.

Antenatal diagnosis and perinatal care of anterior abdominal wall defects

1995 ◽  
Vol 7 (3) ◽  
pp. 109-127 ◽  
Author(s):  
Hratch L Karamanoukian ◽  
Stuart J O'Toole ◽  
Philip L Glick
Keyword(s):  
Parenteral Nutrition ◽  
Abdominal Wall ◽  
Antenatal Diagnosis ◽  
Anterior Abdominal Wall ◽  
Wide Spectrum ◽  
Perinatal Care ◽  
Postoperative Management ◽  
Abdominal Wall Defects ◽  
Anaesthetic Care ◽  
Prognostic Implications

Improvements in anaesthetic care and postoperative management over the last two decades have significantly improved survival of neonates with ventral abdominal wall defects, from a dismal 47% in 1971, to 96% for both gastroschisis and isolated omphalocele in two recent series. This increased survival has generally been attributed to result from improvements in the pre and postoperative management of these fragile neonates. Specifically, the routine use of total parenteral nutrition, and staged repairs for cases with severe “viscero-abdominal disproportion” have been implicated in a decreased incidence of sepsis, morbidity and mortality. In addition, an appreciation of the wide spectrum of anomalies uniquely associated with gastroschisis and omphalocele have helped improve survival, as each has unique pathophysiologic features that have prognostic implications for the fetus before, during and after delivery.

Left-Sided Gastroschisis

2006 ◽  
Vol 72 (7) ◽  
pp. 637-640 ◽  
Author(s):  
Kenneth W. Gow ◽  
Amina Bhatia ◽  
Daniel F. Saad ◽  
Mark L. Wulkan ◽  
Kurt F. Heiss
Keyword(s):  
Abdominal Wall ◽  
Clinical Course ◽  
Abdominal Wall Defect ◽  
Abdominal Wall Defects ◽  
Wall Defect ◽  
The Right

Gastroschisis is a periumbilical, abdominal wall defect arising to the right of the umbilicus. We describe the clinical course of a left-sided gastroschisis in a term female born at 39 weeks’ gestation. To our knowledge, there have been only 14 left-sided abdominal wall defects reported in the literature. We discuss our case and review the literature to try to determine if there is any clinic difference between right- versus left-sided lesions to make recommendations as to management.

scholarly journals Limb Body Wall Complex: A Rare Anomaly

2018 ◽  
Vol 16 (2) ◽  
pp. 56-58
Author(s):  
Unan Sultana ◽  
Md Qumrul Ahsan
Keyword(s):  
Early Diagnosis ◽  
Abdominal Wall ◽  
Umbilical Cord ◽  
Early Pregnancy ◽  
Body Wall ◽  
Abdominal Cavity ◽  
Abdominal Wall Defects ◽  
Proper Management ◽  
Vascular Disruption ◽  
Abdominal Organs

Body stalk anomalies are a group of massively disfiguring abdominal wall defects in which the abdominal organs lie outside of the abdominal cavity in a sac of amnioperitoneum with absence of or very small umbilical cord. Various hypotheses proposed to explain the pathogenesis of limb body wall complex include early amnion disruptions, embryonic dysplasia, and vascular disruption in early pregnancy. Body stalk anomaly is an accepted fatal anomaly and, hence, its early diagnosis aids in proper management of the patient. We present a case of LBWC, exhibiting combined cranial, abdominal & limb features.Chatt Maa Shi Hosp Med Coll J; Vol.16 (2); July 2017; Page 56-58

scholarly journals Fetal Autopsy Showing Two Ventral Body Wall Defects: An Unusual Presentation

2020 ◽  
Author(s):  
Anupriya Kaur ◽  
Arun Prasad ◽  
Jessy Jayaraman Pushpaja ◽  
Kanchan Kapoora
Keyword(s):  
Abdominal Wall ◽  
Body Wall ◽  
Abdominal Wall Defect ◽  
Lower Lobe ◽  
The Body ◽  
Unusual Presentation ◽  
Thoracic Wall ◽  
Fetal Autopsy ◽  
Wall Defect ◽  
The Right

Thoracoschisis and gastroschisis are types of ventral body wall defects. Usually thoracoabdominoschisis presents as a continuous defect. Here the authors present the case of autopsy of 18 weeks old fetus with two well-separated ventral body wall defects. Thoracoschisis was a right lateral thoracic wall defect while gastroschisis, a midline infraumblical abdominal wall defect. Through the right lateral thoracic wall parts of two organs appeared to be protruding. On dissection, 8th and 9th ribs with the liver and the lower lobe of the right lung were herniating outside the body. The right lung was haemorrhagic. Various embryological theories on ventral body wall defects have been discussed in view of the present case.

Prenatal ultrasonographic diagnosis of limb body wall complex

1970 ◽  
Vol 2 (2) ◽  
pp. 72-74
Author(s):  
Narayan B Thapa ◽  
Ganesh Dangal ◽  
Aruna Karki
Keyword(s):  
Key Words ◽  
Abdominal Wall ◽  
Umbilical Cord ◽  
Body Wall ◽  
Abdominal Wall Defects ◽  
Amniotic Band ◽  
Wall Defect ◽  
Ultrasonographic Diagnosis ◽  
Amniotic Band Syndrome ◽  
Rare Combination

In this report, a case of limb body wall complex (LBWC) diagnosed by ultrasonography is presented. Limbbody wall complex refers to a rare combination of disruptive and lethal abnormalities which start early in the gestational process. Abnormalities commonly associated with this disorder include cranio-facial abnormalities, scoliosis, ventral body wall defect (thoraco-abdominoschisis), limb deformations, short umbilical cord, and others. Other terms used to describe similar findings include short umbilical cord syndrome, body-stalk anomaly, and amniotic band syndrome. This complex should be distinguished from other body-wall defects including omphalocele and gastroschisis since the prognosis for limb-body wall complex is uniformly poor. The diagnosis of limb-body wall complex can be made by prenatal sonography. Key words: limb body wall complex, abdominal wall defects, amniotic bands, amniotic band syndrome, ultrasonography.     doi:10.3126/njog.v2i2.1460 N. J. Obstet. Gynaecol 2007 Nov-Dec; 2 (2): 72 - 74

Horn-induced traumatic caudoventral abdominal wall hernia management and repair in an adult Highland cow

2020 ◽  
Vol 8 (3) ◽  
pp. e001121
Author(s):  
Caroline Knox Benham ◽  
Vincent Doré ◽  
Pierre-Yves Mulon
Keyword(s):  
Abdominal Wall ◽  
Body Wall ◽  
Abdominal Wall Hernia ◽  
Exploratory Laparotomy ◽  
Abdominal Ultrasound ◽  
History Of ◽  
The Right ◽  
Resection And Anastomosis ◽  
Haemorrhagic Diarrhoea

An adult Highland cow weighing 380 kg presented following a five-day history of inappetence, recumbency and trauma inflicted by herd-mates. Physical exam demonstrated significant swelling of the right ventral abdomen with skin excoriations. Abdominal ultrasound of the bulging area indicated significant oedema within pockets, thinning of the right body wall compared with the left side, as well as accumulation of free abdominal fluid. Severe mastitis was noted in three quarters of its udder. The patient was stabilised with intravenous fluids, electrolytes and a belly band. It developed severe, haemorrhagic diarrhoea over the following days, and an exploratory laparotomy was performed. A complete 50 cm-long abdominal wall laceration in the caudoventral right paralumbar fossa was repaired, and jejunal resection and anastomosis performed. The patient recovered with supportive care. Surgical complications included postoperative pneumonia and development of a surgical site infection. The patient maintains good quality of life.

scholarly journals EMBRYOLOGICAL ABDOMINAL WALL ANOMALIES- GASTROSCHISIS & OMPHALOCELE

2020 ◽  
pp. 1-2
Author(s):  
Neelima. P ◽  
Rama Devi. G ◽  
Radha Ramani. B
Keyword(s):  
Abdominal Wall ◽  
Umbilical Cord ◽  
Gestational Age ◽  
Umbilical Hernia ◽  
Congenital Abnormalities ◽  
The Other ◽  
Genetic Defects ◽  
Abdominal Wall Defects ◽  
Female Fetus ◽  
Crown Rump Length

Gastroschisis and omphalocele are the two forms of abdominal wall defects due to embryological anomalies mostly seen during 5th -10th week of intrauterine life which is the hallmark of physiological umbilical hernia . These rare congenital abnormalities reflect the underlying genetic defects or can be a part of syndromes like Beckwith Weidemann syndrome, pentology of Cantrell or Limb bodywall complex. The present study reports two cases of the abdominal wall defects- gastroschisis & omphalocele. The still born fetuses present in the department of Anatomy were thoroughly examined. Two fetuses with gestational age between 30-32 weeks (measured basing on crown-rump length) were found to present with these anomalies. Both the malformed fetuses were females. One fetus exhibited omphalocele with a complete sac containing the loops of intestine. The umbilical cord has been found to emerge from the center of the sac. The other female fetus had coils of intestine along with the liver exposed. No other external anomalies have been detected. The present study reports two cases of still born fetuses showing gastroschisis and omphalocele.

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