Larsen Syndrome

September 15, 2007
Charles J. Buttaci, DO
Charles J. Buttaci, DO

,
Bruce A. Pomeranz, MD
Bruce A. Pomeranz, MD

An abnormality of both first proximal interphalangeal joints was noted in a 54-year-old woman who had recently undergone an elective total hip arthroplasty to treat post-traumatic osteoarthritis. She had had worsening hip pain following an intertrochanteric fracture and open reduction internal fixation several years earlier.

 

An abnormality of both first proximal interphalangeal joints was noted in a 54-year-old woman who had recently undergone an elective total hip arthroplasty to treat post-traumatic osteoarthritis. She had had worsening hip pain following an intertrochanteric fracture and open reduction internal fixation several years earlier.

The patient had mild cervical kyphosis and numerous healed incisions on the hips, knees, and ankles in addition to the recent incision on the right hip. There was significant loss of active and passive range of motion at the knees and hips. Neurological findings were normal.

The patient had a history of multiple orthopedic interventions that involved her hips and knees. Her family reported that she was born with bilateral hip and tibiofemoral dislocations and club feet, which required multiple staged reconstruction procedures during childhood.

Larsen syndrome was diagnosed when this patient was a young child. This rare congenital abnormality is characterized by multiple joint dislocations, spine anomalies, and unusual facial features. Spinal pathology, including spondylolysis, cervical kyphosis, and hypoplastic vertebrae, may be severe and has led to quadriplegia in a few cases.1,2

Despite the significant abnormality and functional limitation of her thumbs, the patient was employed for nearly 30 years as an office manager and had raised 2 children. Over the past few years, she had limited her ambulation because of progressive hip and low back pain. She also complained of a worsening discrepancy in leg length, possibly because of poor healing of the intertrochanteric fracture.

After the patient completed a typical postoperative hip rehabilitation program, she was able to ambulate with a walker.

References:

REFERENCES:


1.

Luk KD, Yip DK. Congenital anteroposterior spinal dissociation in Larsen's syndrome: report on two operated cases with long-term follow-up.

Spine.

2002;27:e296-e300.

2.

Katz DA, Hall JE, Emans JB. Cervical kyphosis associated with anteroposterior dissociation and quadriparesis in Larsen's syndrome.

J Pediatr Orthop.

2005;25:429-433.