Spinal instability in Larsenʼs syndrome

<p>Larsen syndrome is a rare inherited disorder characterized by congenital dislocation of multiple joints along with other anomalies of heart, face, hands and bones. Larsen syndrome was first described in 1950 by Larsen, Schottstaedt and Bost. In the present report, we describe a 10 year old girl who presented with mid facial hypoplasia with depressed nasal bridge, high arched palate, bilateral talipes equinovarus and high arched feet. On examination, she had short stature (HAZ -3.5 SD) with hyperextension of knee joint, fixed flexion of elbow joint. Awareness of this condition and associated complications may help in management and follow up of these patients.</p><p> </p>

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“…3,8,9 In our case also, kyphosis and mild scoliosis of cervical spine were present. Photo of her jaw showed overcrowded dentition.…”

Section: Article Infosupporting

confidence: 62%

Larsen syndrome

Islam

Haque

Daraz

et al. 2016

Bangabandhu Sheikh Mujib Medical University Journal

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“…The autosomal dominant form has been suggested on the basis of affected individuals in several generations of a family [McFarlane, 1947;Harris and Cullen, 1971;Latta et al, 1971;Bartsocas and Dimitriou, 1972;Sugarmann, 1975;Habermann et al, 1976;Oki et al, 1976;Ventruto et al, 1976;Beluffi and Segre, 1978;Rpnningen and Bjerkreim, 1978;Trigueros et al, 19781. In the absence of affected kin, genetic counseling is not easy since it is difficult to classify the isolated cases [Reiner, 1904;Bacilieri, 1905;McFarland, 1929;Sorrel and Le Grand-Lambling, 1932;Kohler, 1933;Kaijser, 1935;Rotter and Erb, 1949;Larsen et al, 1950;Hass and Hass, 1958;Silverman, 1972;Lee, 1973;Salmon and Lindenbaum, 1973;Azimi et al, 1974;Kozlowski et al, 1974;Szabo and Perjes, 1974;Buffatti and Pannone, 1975;Fait and Janovec, 1975;Robertson et al, 1975;Simpson et al, 1975;Swensson et al, 1975;Faure et al, 1976;Henriksson et al, 1977;Micheli et al, 1976;Nyhan and Sakati, 1976;O'Herlihy and O'Brien, 1977;Haarmeyer, 1978;Engber, 19791. In many "sporadic" cases no mention is made of whether relatives were examined for minor manifestations of the syndrome, and it is possible that the diagnosis has ...…”

Section: Discussionmentioning

confidence: 99%

A lethal, larsen‐like multiple joint dislocation syndrome

et al. 1982

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We describe here two isolated cases of lethal, Larsen-like multiple joint dislocation syndrome. Death occurred shortly after birth with pulmonary insufficiency due to tracheomalacia and/or lung hypoplasia. Both had abnormal dermal collagen bundles. In one case, histochemical studies showed abnormal cartilagenous matrix and striking deficiency in the dense collagen bundles of joint capsules; electronmicroscopic studies of the hyaline cartilage of the trachea documented a relative increase of the small, short fibers with decrease of the matrix collagen fibers. This study confirms that this Larsen-like syndrome is a disorder of connective tissue, possibly involving dysmaturity of the collagen fibers, with a predilection for joint capsules and tracheal cartilage. This lethal condition is characterized by pulmonary insufficiency, tracheomalacia, and collagen fiber dysmaturity.

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“…The similarities in the elbow include dysplasia of the distal end of the humerus, anterior angulation of the capitellum, presence of abnormal ossicles around the elbow joint and anterior dislocation of the humero-ulnar joint. However, the other pathognomic features of Larsen's syndrome, which include joint laxity, multiple joint dislocations [2,3,7,8,9,10,11,12,13], spatulate thumb [2,3,7,8,9,13], cylindrical fingers [2,3,7,8,9,11,12,13], spinal anomalies [2,3,7,8,9,10,12,13], and abnormal ossification centres around the wrist and feet [2,3,7,8,9,12,13,14], were all absent in this child. Thus, although the child had some features resembling Larsen's syndrome, she could not be placed under this umbrella because many of the pathognomic features of this syndrome were absent.…”

Section: Benjamin Joseph Renjit a Varghesementioning

confidence: 99%

Congenital distal humeral dysplasia: a case report

Joseph

Varghese

2003

Pediatric Radiology

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Congenital dysplasia of the humerus is very rare. It is characteristically seen in omodysplasia and has also been reported as one of the associated features of Larsen's syndrome. We report a 4-year-old girl with bilateral humero-ulnar dysplasia, with dislocation of the elbows, facial dysmorphism, ball-and-socket ankles and foot deformities. Although the elbow dysplasia is similar to that seen in Larsen's syndrome, other pathognomic features of Larsen's syndrome were absent. The changes seen in the elbows in this patient are also different from those encountered in omodysplasia. We believe that this condition may be a distinct form of skeletal dysplasia hitherto undescribed.

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Spinal instability in Larsenʼs syndrome

Cited by 47 publications

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Larsen syndrome

Larsen syndrome

A lethal, larsen‐like multiple joint dislocation syndrome

Congenital distal humeral dysplasia: a case report

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