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Syndrome of the Month Defects in the Determination of Left-Right Asymmetry

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Syndrome of the Month Defects in the Determination of Left-Right Asymmetry 49849 Med Genet 1996;33:498-503 Syndrome of the month J Med Genet: first published as 10.1136/jmg.33.6.498 on 1 June 1996. Downloaded from Defects in the determination of left-right asymmetry Miranda Penman Splitt, John Burn, Judith Goodship Left and right in the embryo is automatically Laterality defects with normal cilia defined by the formation of the anteroposterior The majority of patients with situs inversus or and dorsoventral axes. In an asymmetrical heterotaxy have no symptoms suggestive of organism, specification of the left-right axis ciliary dyskinesia. An important group of these must incorporate two distinct processes, the patients have complete absence of visceral generation of asymmetry and its orientation or asymmetry with evidence for either bilateral handedness. The orientation of the heart and left or bilateral right sidedness. This condition abdominal organs in vertebrates is non-random is known as isomerism sequence. Other de- and highly conserved both across and within scriptive terms include Ivemark syndrome, as- species.' Reversal of orientation may occur plenia/polysplenia syndrome, and the broader without concomitant defects in the generation term, laterality sequence. of asymmetry. However, failure to generate asymmetry is almost always combined with abnormal orientation. The first overt breaking CLINICAL FEATURES OF ISOMERISM SEQUENCE of symmetry across the midline of the embryo "Isomerism" refers to the paired structures occurs when the primitive heart tube loops to which usually have morphologically distinct the right but subtle asymmetries are evident as right and left forms, but in this condition are early as the primitive streak stage.2 mirror images of each other. Specifically, these A spectrum of malformations, ranging from are the cardiac atrial appendages, bronchi, and complete reversed situs to complete failure to lungs. Thus, in left isomerism sequence the establish asymmetry, is seen in man and it is heart has two long narrow atrial appendages likely that their aetiology and genetic basis and typically both lungs are bilobed with long will turn out to be extremely heterogeneous. hyparterial bronchi. In right isomerism se- Nevertheless, genetic animal models indicate quence both atrial appendages are pyramidal that a spectrum of lateralisation defects can be in shape and the lungs are usually trilobed http://jmg.bmj.com/ produced by a single defective gene. In man with short eparterial bronchi bilaterally. Many most cases of disturbed laterality are sporadic earlier studies do not recognise atrial or bron- but there are several well recognised syndromes chial isomerisms as pathological criteria.89 which occur in families. Nevertheless, Ivemark8 in 1955 realised the The usual orientation of the heart and ab- importance of the absence of left-right asym- dominal organs is known as situs solitus. Situs metry in the pathogenesis of the condition inversus refers to a perfect reversal of situs - and suggested that it be called "asplenia a on October 2, 2021 by guest. Protected copyright. solitus with a left heart loop. Any arrangement teratologic syndrome of visceral symmetry". In other than situs solitus or situs inversus, that recent years it has been shown that isomerism of is, random orientation of different organs, is the atrial appendages is a much more constant known as heterotaxy or situs ambiguus. The feature than the state of the spleen or the estimated prevalence ofcomplete situs inversus presence of heterotaxy.'0l is around 1 in 10 OOO.3 An increased incidence There are no population based studies of of parental consanguinity has been found, sug- isomerism sequence. A population based study gesting that a proportion of cases are autosomal ofasplenia in Canada found the incidence to be recessive.`5 The incidence ofheterotaxy is gen- 1 per 22 000 live births.'2 Although it includes a erally much lower,34 but interestingly was found few cases ofisolated asplenia, it largely excludes to be equal to that of situs interversus in an cases of left isomerism sequence which is likely inbred Bedouin population (1 in 4000).6 to be at least as common. In a retrospective Cardiac anomalies are found in between 3 study of children who had undergone cardiac and 9% of cases of complete situs inversus. catheterisation, the incidence of complex heart Heterotaxy, on the other hand, is almost in- defect with heterotaxy and poly/asplenia was Department of Human variably associated with complex cardio- estimated to be 1/24 000 in a white English Genetics, University of vascular malformation as well as splenic and a population, accounting for about 1 % of con- Newcastle upon Tyne, variety of gastrointestinal anomalies. 19/20 Claremont Place, genital heart disease.'3 Right isomerism with Newcastle upon Tyne In the present state of knowledge, the most asplenia appears to be more common in NE2 4AA, UK logical way to categorise defects in laterality is males,'4 '5 whereas left isomerism/polysplenia M Penman Splitt on the basis of ciliary function. J Bum shows an equal sex ratio. J Goodship (JtMed Genet 1996;33:498-503) Both left and right isomerism sequence are associated with a wide spectrum of severe heart Correspondence to: Key words: asymmetry; laterality defects; isomerism Dr Penman Splitt. sequence; heterotaxy. malformations. '0 '" These include anomalies Defects in the determination of left-right asymmetry 499 The frequency of common heart lesions found in patients with isomerism sequence Left Right isomerism isomerism (/0) (%/) J Med Genet: first published as 10.1136/jmg.33.6.498 on 1 June 1996. Downloaded from Interruption of IVC with azygos 75 7 continuation Total anomalous pulmonary venous 0 35 connection Common atrium 47 67 Single ventricle 30 70 Atrioventricular septal defect 68 93 Double outlet right ventricle 7 47 Pulmonary stenosis/atresia 48 78 The table refers to the mean of the percentages given in ref- erences 10, 16, and 17 for right isomerism and 10, 17, and 18 for left isomerism. of both systemic and pulmonary venous drain- age, which occur as a consequence of atrial isomerism, and malformations which are thought to be secondary to distortion ofcardiac looping, septal defects and conotruncal an- omalies. The are summarised principal defects Figure 1 A high kilovoltage filtered chest radiograph in the table. The high incidence of total an- showing the bilateral, long hyparterial bronchi of left omalous pulmonary venous drainage, single bronchial isomerism. ventricle, and pulmonary outflow tract ob- struction leads to higher morbidity and mor- tality in right isomerism sequence.'9 musculoskeletal defects, most commonly ac- Patients with right isomerism sequence al- cessory digits and scoliosis, 6% facial defects, most always have asplenia. The converse, that and 7% CNS defects. In a review of midline asplenia is usually accompanied by right iso- anomalies in 166 patients with isomerism se- merism, is also true. Anderson et al" found no quence, males accounted for all those cases instances of complete absence of the spleen with imperforate anus and CNS involvement without evidence of isomerism of the atrial and over 80% of those with genitourinary and appendages in a survey of 1042 necropsies. vertebral anomalies29 (see section on X linked Rarely, patients with right isomerism sequence laterality disturbance). have between one and four non-uniform Bronchial isomerism can be shown on a spleens.20 Antibiotic prophylaxis and pneu- high voltage beam chest x ray30 (fig 1). Atrial mococcal vaccination should be standard morphology can be discerned on ultrasound by management in patients with asplenia. Left an experienced cardiologist. Cross sectional http://jmg.bmj.com/ isomerism sequence is usually accompanied views of the abdominal great vessels are said by the presence of multiple (more than six) to be very helpful." MRI may be useful to uniformly sized spleens or spleniculi but obtain complete anatomical information.'2 patients with smaller numbers of non-uniform Abdominal ultrasound should be routine as spleens are also found.20 Unlike asplenia, Howell Jolly bodies cannot always be relied on polysplenia is found just as frequently without as a sign of asplenia.16 isomerism, either with a normal heart or an on October 2, 2021 by guest. Protected copyright. isolated heart lesion.22 The association of poly- splenia with biliary atresia, absent gall bladder, AETIOLOGY AND GENETICS and intestinal malrotation is well recog- Mouse models nised.2324 Martinez-Frias et a122 also found The best evidence that complete situs inversus, positive associations between polysplenia and heterotaxy, and isomerism sequence may have diaphragmatic hernia, cleft lip, hemivertebrae, the same genetic basis comes from two mouse and agenesis of the corpus callosum. models: a spontaneous mutant inversus viscerum Intestinal rotation and fixation abnormalities (iV)33 and an insertional transgenic mutant in- (IRFA) are common: four patients (14%) in a version of embryo turning (inv). series of 28 required emergency surgery for Broadly speaking, situs is random in homo- intestinal obstruction.25 The authors put the zygote iv/iv animals, 50% having situs solitus case for routine GI imaging and elective Ladd and 50% situs inversus. Heterozygotes are en- procedure in cases of isomerism sequence. tirely normal. However, more detailed analysis Hiatus hernia is also a common complication.26 shows that around 30% of adults have ab- Other GI anomalies rarely associated with iso- normal spleens and venous abnormalities ana-
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