Limb Body Wall Complex

D’Souza, John; Indrajit, IK; Menon, S.

doi:10.1016/s0377-1237(04)80169-2

Cited by 16 publications

(18 citation statements)

References 4 publications

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“…43 LBWC is considered by most authors to be uniformly fatal with a purported incidence of 1 in 4,000, though no source is given for this. 44 The precise pathogenesis of these three entities remains unknown, though frequently debated. Many theories have been proposed but none has been validated.…”

Section: Discussionmentioning

confidence: 99%

“…Abdominoschisis, diaphragmatic defect 39 Agenesis of the anal canal, agenesis of the genitourinary tract, hypoplastic lungs 64 Absent diaphragm, bowel atresia, renal agenesis, anal atresia, no external genitalia 44 Anal atresia, no external genitalia, no urinary bladder, hypoplastic lungs 47 Abbreviations: ASD, atrial septal defect; OEIS, omphalocele, exstrophy, imperforate anus, spina bifida; PDA, patent ductus arteriosus; PFO, patent foramen ovale; SVC, superior vena cava. bladder halves.…”

Section: Anencephaly 32mentioning

confidence: 99%

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Fetal MRI in the Identification of a Fetal Ventral Wall Defect Spectrum

Coleman

Marine

Weida

et al. 2018

AJP Rep

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Objective To ascertain if useful criteria for prenatal diagnosis of fetal ventral body wall defects (VBWDs) exists by reviewing published literature on diagnosis of VBWD as compared with our own diagnostic experience. Study Design A comprehensive literature review of diagnostic criteria of fetal VBWD including pentalogy of Cantrell (POC), omphalocele, exstrophy, imperforate anus, spina bifida (OEIS), cloacal exstrophy, limb–body wall complex (LBWC), and body stalk anomaly was performed followed by a retrospective review of all fetal magnetic resonance imaging (MRI) examinations from our medical center over a 2-year period. Results Classically, OEIS is omphalocele, bladder exstrophy, imperforate anus, and spina bifida. POC is defects of the supraumbilical abdomen, sternum, diaphragm, pericardium, and heart. LBWC is two of the following: exencephaly or enencephaly with facial clefts, thoracoschisis or abdominoschisis, and limb defects. Twenty-four cases of VBWD on MRI over a 24-month period were identified with seven cases involving defects of additional organ systems. Six of these seven cases demonstrated findings from two or more of the traditional diagnoses POC, OEIS, and LBWC making diagnosis and counseling difficult. Conclusion There is a lack of consensus on useful diagnostic criteria within the published literature which is reflected in our own diagnostic experience and poses a challenge for accurate prenatal counseling.

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Section: Discussionmentioning

confidence: 99%

Section: Anencephaly 32mentioning

confidence: 99%

Fetal MRI in the Identification of a Fetal Ventral Wall Defect Spectrum

Coleman

Marine

Weida

et al. 2018

AJP Rep

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“…The incidence of this syndrome varies from 1 in 1,200 to 1 in 15,000 live births [1] . However, this congenital anomaly has no proven relationship with sex or familial predilection [9] . The presence of ABS in a fetus usually causes premature labor and delivery.…”

Section: Discussionmentioning

confidence: 99%

Placento-Cranial Adhesions in Amniotic Band Syndrome and the Role of Surgery in Their Management: An Unusual Case Presentation and Systematic Literature Review

et al. 2015

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Amniotic band syndrome is a group of sporadic congenital anomalies that involve the limbs, craniofacial regions and trunk, ranging from simple digital band constriction to complex craniofacial and central nervous system abnormalities. Placento-cranial adhesions in amniotic band syndrome are extremely rare, and severe conditions are associated with high morbidity and mortality rates. In this study, we pooled placento-cranial adhesion case reports that were published in the medical literature and added an unpublished case from our institution. The purpose of this article was to review and discuss the clinical features and outcomes of placento-cranial adhesions in amniotic band syndrome.

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“…Usually, there is no association with chromosomal anomalies; still placental trisomy 16 or maternal uniparental disomy 16 have been reported [71]. The recurrence rate has been demonstrated to be low, and there is no correlation with parental age or fetal gender [68,72]. The reported prevalence is 0.12 cases per 10,000 births (alive and still births) [73] or even higher 1 in 7500 pregnancies [74].…”

Section: Body Stalk Syndrome or Limb: Body Wall Complexmentioning

confidence: 99%

Fetal Abdominal Wall Defects

Dragusin¹,

Şorop-Florea²,

LaurențiuPătru³

et al. 2018

Congenital Anomalies - From the Embryo to the Neonate

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Abdominal wall defects (AWDs) represent a group of congenital anomalies that can be diagnosed early during pregnancy even at the time of the first trimester assessment, with direct impact on pre-and postnatal fetal prognosis and management decisions. The most frequent anomalies in this group are gastroschisis and omphalocele. The key method available, that allows the detection of any deviation from the physiologic midgut herniation, is the ultrasound (US) assessment. A precise algorithmic scan approach is imposed not only for an accurate detection of any abdominal wall defect, but also for a proper location of the defect and of the spatial relation to the umbilical cord insertion, fundamentally important in differentiating among various malformations. Other structural or chromosomal anomalies should be excluded. Suitable multidisciplinary counseling should be considered. Unfortunately, in utero surgery, in these cases, has not been yet successful. Postnatal early interventions are usually required in specialized pediatric centers.

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Limb Body Wall Complex

Cited by 16 publications

References 4 publications

Fetal MRI in the Identification of a Fetal Ventral Wall Defect Spectrum

Fetal MRI in the Identification of a Fetal Ventral Wall Defect Spectrum

Placento-Cranial Adhesions in Amniotic Band Syndrome and the Role of Surgery in Their Management: An Unusual Case Presentation and Systematic Literature Review

Fetal Abdominal Wall Defects

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