Giant pseudomeningocele causing urinary obstruction in a patient with Marfan syndrome

Abstract

<jats:p>Defective collagen biosynthesis in Marfan syndrome predisposes to dural defects such as dural ectasia, meningocele, and pseudomeningocele; thus, an increased index of suspicion for these conditions should be present in the clinical setting of Marfan syndrome. The authors describe a young woman with Marfan syndrome who was being treated with anticoagulants for a prosthetic heart valve and who presented with a spontaneous retroperitoneal hemorrhage requiring surgical evacuation. No CSF leak was encountered at surgery, but she developed progressively more severe positional headaches over the following year. She then experienced the sudden onset of acute urinary obstruction, at which time CT revealed a 17 × 15 × 13–cm presacral pseudomeningocele communicating with the thecal sac through a sacral bone defect. An anterior surgical approach was used for drainage of the pseudomeningocele as well as for primary closure of the dural defect with a bovine pericardial patch and autologous subcutaneous fat graft. After a short period of lumbar subarachnoid drainage of the CSF, the patient was able to resume normal activity without recurrent symptoms. To the authors' knowledge, such a pseudomeningocele in a patient with Marfan syndrome has been reported only twice, and this case features the largest pseudomeningocele to date. They also review the pertinent literature regarding presentation, diagnosis, and management of these lesions.</jats:p>

A Rare Case of a Large Meningocele Posterior Sacrococcygeal

“…Associated symptoms are generally rare and mild [17]. Postural headaches due to changes in intracranial pressure may result either from hydrostatic pressure directly on the cyst resulting from the retrograde transmitted Valsalva maneuver to the intracranial region or from hypotension due to position changes in adults [1], [8], [18]. In the newborn, the median swelling, of variable size, along the rachis is very characteristic; associated anomalies (hydrocephalus, Arnold Chiari malformation, Dandy Walker malformation, toe and / or finger malformation) are almost exclusive to SBAs, and all the more frequent the more severe and lower located [1], [10]- [12].…”

“…The posterior transsacral approach, introduced by Adson in 1938, exposes communication with the meningocele. It consists of aspirating its contents through the pedicle and closing the communication with primary ligature or obliteration with a graft [3], [4], [7], [15], [16]- [18]. This is the approach most used by neurosurgeons and has traditionally been considered low risk and with fewer complications.…”

“…Secondly, the plastic surgery team performed a buttock plasty (Discharge incisions on both sides, allowing tension-free closure, plasty of the levator muscles, then of the buttocks muscles and possible drainage by a blade of Delbet then suture of the different planes). The use of endoscopy [5], [7], [18] has been recommended as an alternative, especially for non-obese patients with a small fistula connection.…”

Urinary Disorders and Marfan Syndrome: A Series of 4 Cases

“…Urinary disorders are not described in Marfan syndrome except in an isolated case of urinary retention on dural ectasia external compression [3] and possibility of increased incontinence in women due to connective tissue abnormalities [4]. …”

Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient

“…Dural ectasia involves the enlargement of the spinal canal, concavity of the posterior vertebral body, reduction of cortical bone thickness of the pedicles and laminae, expansion of the neural foramina, formation of a meningocele, scoliosis, spondylolithesis, or vertebral fractures. [ 1 ] Lumbosacral dural ectasia is commonly associated with connective tissue disorders, such as Marfan syndrome,[ 2 3 4 5 6 7 8 9 ] osteogenesis imperfecta, Loeys-Dietz syndrome,[ 10 11 12 13 14 ] ankylosing spondylitis (AS),[ 15 16 ] neurofibromatosis,[ 17 18 ] fibromuscular dysplasia,[ 19 ] and Larsen syndrome;[ 20 ] however, the pathology has also been linked to vertebral fracture, spine surgery,[ 21 ] trisomy 13,[ 22 ] scoliosis,[ 23 ] and trauma.…”