Anterior Sacral Meningocele as a Pelvic Complication of Marfan Syndrome

Voyvodic, Frank; Scroop, Rebecca; Sanders, R. R.

doi:10.1111/j.1479-828x.1999.tb03390.x

Cited by 25 publications

(12 citation statements)

References 7 publications

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“…65 Anterior sacral meningocele has been described rarely as a complication of Marfan syndrome, and may lead to diagnostic confusion when presenting as a pelvic or abdominal mass. 66 Cerebral haemorrhage and other neurovascular disorders are uncommon in Marfan patients, 67 but intracranial aneurysms may be more common in the Loeys -Dietz syndrome. 13 …”

Section: Central Nervous Systemmentioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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In association withMarfan syndrome: clinical diagnosis and management Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys -Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg b-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear -for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.

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Section: Central Nervous Systemmentioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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“…Therefore patients affected by ASM often have other associated urogenital and rectal abnormalities [5]. It is sometimes associated with syndromes such as Currarino [6] and Marfan [7]. In those patients without any urogenital or rectal abnormalities or symptoms, ASM can be difficult to recognize.…”

Section: Discussionmentioning

confidence: 99%

An elusive pelvic cyst: a case report of an anterior sacral meningocele

Johnston

Chou

et al. 2007

Gynecol Surg

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“…Dural ectasia has been related to chronic lumbosacral pain syndrome, and it may give rise to complications in rare circumstances. For example, spontaneous cerebrospinal fluid leaks can result in intracranial hypotension presenting with postural headache; and giant anterior or lateral meningocele may cause constipation, urinary symptoms including retention or incontinence, or dysmenorrhea 94,95…”

Section: Team Membersmentioning

confidence: 99%

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Kodolitsch¹,

Rybczynski²,

Vogler³

et al. 2016

JMDH

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Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS. First, we show how the initiative and collaboration of patient representatives, scientists, and physicians resulted in the foundation of Marfan centers, initially in the US and later in Germany, and how and why such centers evolved over time. Then, we elucidate the three main structural elements; a team of coordinators, core disciplines, and auxiliary disciplines of health care. Moreover, we explain how a multidisciplinary health care team integrates into many other health care structures of a university medical center, including external quality assurance; quality management system; clinical risk management; center for rare diseases; aorta center; health care teams for pregnancy, for neonates, and for rehabilitation; and in structures for patient centeredness. We provide accounts of medical goals and standards for each core discipline, including pediatricians, pediatric cardiologists, cardiologists, human geneticists, heart surgeons, vascular surgeons, vascular interventionists, orthopedic surgeons, ophthalmologists, and nurses; and of auxiliary disciplines including forensic pathologists, radiologists, rhythmologists, pulmonologists, sleep specialists, orthodontists, dentists, neurologists, obstetric surgeons, psychiatrist/psychologist, and rehabilitation specialists. We conclude that a multidisciplinary health care team is a means to maximize therapeutic success.

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Anterior Sacral Meningocele as a Pelvic Complication of Marfan Syndrome

Cited by 25 publications

References 7 publications

Marfan syndrome: clinical diagnosis and management

Marfan syndrome: clinical diagnosis and management

An elusive pelvic cyst: a case report of an anterior sacral meningocele

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

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