PubMed · 13488230
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J J CHOSEY, F M BEMAN. 1958. Constitutional hepatic dysfunction.. https://doi.org/10.7326/0003-4819-48-1-202
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Although uncommon, aneurysmal subarachnoid haemorrhage (SAH) in pregnancy can be devastating for both mother and baby. It is the leading cause of other indirect maternal death in England and Wales accounting for 60 deaths in the decade 1988-1999. No single obstetric or neurosurgical unit has sufficiently large database or experience in managing this condition in pregnancy. With significant improvements in antenatal care and management of deliveries, non-obstetric causes of maternal death such as aneurysmal subarachnoid haemorrhage are likely to become increasingly significant. The clinical features of aneurysmal subarachnoid haemorrhage closely resemble those of other commoner conditions seen in pregnancy. It is therefore imperative that awareness by obstetricians and other frontline staff is increased so that a high index of suspicion is maintained when pregnant women present with unique headaches. Prompt neurosurgical referral is vital and early involvement of an experienced neuroradiologist essential. It is only when an early diagnosis is made and an aggressive treatment instituted that the bleak case-fatality figure associated with aneurysmal subarachnoid haemorrhage in pregnancy can be improved. This review, by a multidisciplinary and multicenter team, provides a comprehensive update on the epidemiology, aetiology, clinical presentation, diagnosis and the complexities of the multidisciplinary management of this serious and potentially fatal condition when it occurs in pregnancy.
The medical care of patients affected by rare disorders depends heavily on experiences garnered from prior cases, including those patients evaluated by the treating physician and those published in the medical literature. The utility of published cases is wholly dependent upon accurate diagnosis of those patients. In our experience, the rate of misdiagnosis in Proteus syndrome (PS) is high. Diagnostic criteria have been published, but these criteria have not been applied consistently and were published after many case reports appeared in the literature. We reviewed 205 cases of individuals reported to have PS in the literature and three of us independently applied the diagnostic criteria to these case reports. Our initial diagnostic congruence was 97.1% (199/205); the discrepancies in six cases were easily resolved. Only 97 (47.3%) of reported cases met the diagnostic criteria for PS; 80 cases (39%) clearly did not meet the criteria; and although 28 cases (13.7%) had features suggestive of PS, there were insufficient clinical data to make a diagnosis. Reported cases that met the PS criteria had a higher incidence of premature death, and other complications (scoliosis, megaspondyly, central nervous system abnormalities, tumors, otolaryngologic complications, pulmonary cystic malformations, dental and ophthalmogic complications) compared to those in the non-Proteus group. The cases that met the criteria were more often male, which has implications for hypotheses regarding the etiology and pathophysiology of PS. We also studied the attributes that led authors to conclude the reported patients had PS when we concluded they did not. We found that two of the diagnostic criteria (disproportionate overgrowth and connective tissue nevi) were often misinterpreted. In PS, the abnormal growth is asymmetric, distorting, relentless, and occurred at a faster rate compared to the rest of the body. Furthermore, PS was associated with irregular and disorganized bone, including hyperostoses, hyperproliferation of osteoid with variable calcification, calcified connective tissue, and elongation of long bones with abnormal thinning. In contrast, non-Proteus cases displayed overgrowth that was asymmetric but grew at a rate similar to the growth found in unaffected areas of the body. Also, the overgrowth in non-Proteus cases was associated with normal or enlarged bones together with ballooning of the overlying soft tissues. Taken together, these data show that (1) PS diagnostic criteria sort individuals with asymmetric overgrowth into distinct groups; (2) individuals with PS were more likely to have serious complications; (3) PS affects more males than females; and 4) the published diagnostic criteria are useful for clinical care and research. This article contains supplementary material, which may be viewed at the American Journal of Medical Genetics website at http://www.interscience.wiley.com/jpages/0148-7299/suppmat/index.html.
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