Absence of the spleen(s) in conjoined twins: a diagnostic clue of laterality defects? Radiological study of historical specimens

Oostra, Roelof‐Jan; Keulen, N.M.; Jansen, Tessa; Rijn, Rick R. van

doi:10.1007/s00247-011-2316-8

Cited by 9 publications

(9 citation statements)

References 15 publications

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“…However, some of the shared anomalies could be linked to the twinning mechanism itself, especially when they are closely related to the conjunction area. As we will show, these seemingly incidental anomalies are solely and sequentially the results of neo‐axial orientation and interaction aplasia, two embryological adjustments exclusive to conjoined twins (Oostra, Keulen, Jansen, & van Rijn, 2012 ). Although these two mechanisms are currently discussed only in respect of conjoined twins, it could be hypothesized that they are also present in or influence the ultimate morphology of singletons; multiple causes could potentially generate a heterogeneous outcome.…”

Section: Introductionmentioning

confidence: 82%

“…Two important embryonic adjustments, seen exclusively in conjoined twins, are noteworthy: neo-axial orientation and interaction aplasia (Oostra et al, 2012). Both mechanisms are responsible for adjustments and alterations of external and internal morphology and determine the ultimate phenotype of the conjoined twins.…”

Section: Conjoined Twinning: a Phenotypic Spectrum Dictated By Neo-axial Orientation And Interaction Aplasiamentioning

confidence: 99%

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Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Kim

Schepens‐Franke

Winter³

et al. 2021

Clinical Anatomy

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Shared anomalies, always located close to the area of coalescence and observable in virtually every type of conjoined twinning, are currently seen as separate anomalies caused by mostly unknown and seemingly unrelated pathways rather than being connected to the twinning mechanism itself. Therefore, most (case) reports about conjoined twins are mere descriptions of (external) dysmorphologies lacking reflections on the possible origin of their concomitant anomalies. As we will demonstrate in this article, shared anomalies are influenced, and in some cases solely and sequentially explained, by interaction aplasia and neo-axial orientation; two embryological mechanisms to which each set of conjoined twins is subjected and are responsible for their ultimate phenotypical fate.In this review, we consider how the ventral, lateral and caudal conjunction types and their intermediates determine the phenotypic presentation of the twins, including patterns of shared malformations and anomalies, which in themselves can be indistinguishable from those encountered in singleton cases. Hence, it can be hypothesized that certain anomalies in singletons originate in a fashion similar to that in conjoined twins.

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Section: Introductionmentioning

confidence: 82%

Section: Conjoined Twinning: a Phenotypic Spectrum Dictated By Neo-axial Orientation And Interaction Aplasiamentioning

confidence: 99%

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Kim

Schepens‐Franke

Winter³

et al. 2021

Clinical Anatomy

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“…Yapışık ikizler 200 000 canlı doğumda bir ve 200 monozigot ikizde bir sıklığında nadir görülen bir durumdur [1]. Yapışık ikizlerin patogenetik mekanizması ve monozigot ikizlerin nedeni henüz tam olarak açıklanamamıştır.…”

Section: Discussionunclassified

Parapagus ikizlerin ekokardiyografik incelemesi

Gürsu¹,

Varan²,

Erdoğan³

et al. 2014

Cumhuriyet Med J

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ÖzetParapagus ikizler literatürde son derece nadir olarak bildirilmiştir. Tahmin edilen sıklığı iki yüz bin doğumda birdir. Ülkemizdeki sıklığı ise tam olarak bilinmemektedir. Otuz altı yaşındaki annenin 8'inci gebeliğinden 8'inci ve 9'uncu canlı doğum olarak doğan bebeklerin doğum sonrası solunum sıkıntıları olması nedeni ile hastanemize gönderildiler. Vücutları anterolateral olarak birleşmiş ikizlerin iki başı, 3 eli ve 3 ayağı, bir anal açıklığı ve şüpheli genital yapısı vardı. Soldaki bebeğin kalp tepe atımı 137/dak. ve satürasyonu %85, sağdaki bebeğin sırası ile 135/dak. ve %86 idi. Kraniyal ultrasonografik inceleme ve magnetik rezonans görüntüleme normaldi. Abdominal ultrasonografi ve magnetik rezonans görüntülemede ise bir karaciğer, bir dalak, 3 böbrek ve bir mesane görüldü. Yapılan ekokardiyografik incelemede sağdaki bebeğin iki ventrikül, iki atriyumunun, soldakinin ise tek ventrikül ve tek atriyumunun olduğu, sağdaki bebekte komplet atriyoventriküler septal defekt, pulmoner stenoz ve büyük arterlerin transpozisyonu bulunduğu görüldü. Soldaki bebeğin ikinci ventrikülü görülemedi. Sağdaki bebeğin sol ventrikül arka duvarı ile soldaki bebeğin ventrikül duvarı ortaktı. Atriyum ve ventrikül düzeyinde füzyon olduğu düşünüldü. Nadir görülen bir durum olan ve literatürde daha önce bildirilmemiş, farklı kompleks doğumsal kalp hastalığı saptanan parapagus ikizlerin ekokardiyografik inceleme sonuçlarını paylaşmak istedik. Anahtar sözcükler: Doğumsal kalp hastalığı, ekokardiyografi, kardiyak cerrahi AbstractParapagus twins are reported very rarely in the literature. The incidence is estimated one in 200 000 live birth. The incidence in our country isn't known. Because of the mother who was 36 years old, in the 8. pregnancy, the 8. and 9. live birth babie, who have dispnea after birth, were sent to in our hospital. Twins that their bodies are joined anterolaterally, have two complete heads, 3 hands and 3 feet, one anus and suspected genitelia. Heart beat of left sided twin was 137/minute and the saturation was 85% and right sided twin's was 135/minute and 86%. Cranial ultrasonographic examination and magnetic resonance imaging were normal. Abdominal ultrasonography and magnetic resonance imaging showed one liver, one spleen, 3 kidney and one bladder. On echocardiographic examination, there were 2 ventricles, 2 atriums in right sided twin and one ventricle, one atrium in left sided. Complete atrioventricular septal defect, pulmonary stenosis and transposition of the great arteries were seen in right sided twin. We didn't saw second ventricle in left sided twin. Posterior wall of right baby's left ventricle and left baby's single ventricle's wall were fused. There was fusion on atrial and ventricular levels. We want to share the conclusions of echocardiographic examination of parapagus twins who were rarely seen and had different complex congenital heart disease.

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“…Reused with permission from Spencer R, Clin Anat, 2000, 3, 36-53, John Wiley and Sons. distance and mutual position determines the site and degree of conjunction. Depending on the configuration of the initial "duplications" (opposite or angulated), the neo-axial orientation and/or interaction aplasia occurs (Machin, 1993;Spencer, 2003;Oostra et al, 2012). The premise of initial duplication of certain "axial primordia" is strengthened by many experimental studies.…”

Section: "Crowding" Theory: Induction Of Two Axial Primordiamentioning

confidence: 99%

Two is a Crowd

2019

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In this article, we provide a comprehensive overview of multiple facets in the puzzling genesis of symmetrical conjoined twins. The etiopathogenesis of conjoined twins remains matter for ongoing debate and is currently cited—in virtually every paper on conjoined twins—as partial fission or secondary fusion. Both theories could potentially be extrapolated from embryological adjustments exclusively seen in conjoined twins. Adoption of these, seemingly factual, theoretical proposals has (unconsciously) resulted in crystallized patterns of verbal and graphic representations concerning the enigmatic genesis of conjoined twins. Critical evaluation on their plausibility and solidity remains however largely absent. As it appears, both the fission and fusion theories cannot be applied to the full range of conjunction possibilities and thus remain matter for persistent inconclusiveness. We propose that initial duplication of axially located morphogenetic potent primordia could be the initiating factor in the genesis of ventrally, laterally, and caudally conjoined twins. The mutual position of two primordia results in neo‐axial orientation and/or interaction aplasia. Both these embryological adjustments result in conjunction patterns that may seemingly appear as being caused by fission or fusion. However, as we will substantiate, neither fission nor fusion are the cause of most conjoined twinning types; rather what is interpreted as fission or fusion is actually the result of the twinning process itself. Furthermore, we will discuss the currently held views on the origin of conjoined twins and its commonly assumed etiological correlation with monozygotic twinning. Finally, considerations are presented which indicate that the dorsal conjunction group is etiologically and pathogenetically different from other symmetric conjoined twins. This leads us to propose that dorsally united twins could actually be caused by secondary fusion of two initially separate monozygotic twins. An additional reason for the ongoing etiopathogenetic debate on the genesis of conjoined twins is because different types of conjoined twins are classically placed in one overarching receptacle, which has hindered the quest for answers. Clin. Anat. 32:722–741, 2019. © 2019 Wiley Periodicals, Inc.

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Absence of the spleen(s) in conjoined twins: a diagnostic clue of laterality defects? Radiological study of historical specimens

Cited by 9 publications

References 15 publications

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Characterizing the coalescence area of conjoined twins to elucidate congenital disorders in singletons

Parapagus ikizlerin ekokardiyografik incelemesi

Two is a Crowd

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