Specific sutures
Sagittal craniosynostosis/scaphocephaly
There are two surgical procedures which are commonly used to treat sagittal synostosis. The matter of which procedure is superior is still heavily debated amongst the surgeons treating this condition, however it is generally agreed upon that the cephalic index should be used to assess the efficacy of the preferred surgical intervention. Firstly, the extended strip craniectomy will be discussed, which is a further developed form of the traditional craniectomy. The traditional procedure involved only the excision of the closed suture, with the intent that the compensatory skull deformations would automatically be corrected by the fast growth of the brain during the first year of life.This did not quite result in a true normalization of the cranial vault, causing the development of numerous modifications. One of those is the extended strip craniectomy. This procedure can be performed by using an endoscope, becoming more popular due to the resultant rapid recovery of the child and reduced need for blood transfusion. The patient is afterwards placed in a custom made molding helmet to correct the resting deformities of the skull. In the other widely used surgical procedure, total cranial vault remodelling, the compensatory deformities are corrected during the operation, with excision of the fused suture. Most of the bones that collectively form the cranial vault – i.e. the frontal, the parietal and the occipital bones – are removed and reshaped to normalize the contours.
Treatment
Attacks are self-limiting, and require analgesia and NSAIDs (such as diclofenac). Colchicine, a drug otherwise mainly used in gout, decreases attack frequency in FMF patients. The exact way in which colchicine suppresses attacks is unclear. While this agent is not without side effects (such as abdominal pain and muscle pains), it may markedly improve quality of life in patients. The dosage is typically 1–2 mg a day. Development of amyloidosis is delayed with colchicine treatment. Interferon is being studied as a therapeutic modality. Some advise discontinuation of colchicine before and during pregnancy, but the data are inconsistent, and others feel it is safe to take colchicine during pregnancy.Approximately 5–10% of FMF cases are resistant to colchicine therapy alone. In these cases, adding anakinra to the daily colchicine regimen has been successful. Canakinumab, an anti-interleukin-1-beta monoclonal antibody, has likewise been shown to be effective in controlling and preventing flare-ups in patients with colchicine-resistant FMF and in two additional autoinflammatory recurrent fever syndromes: mevolonate kinase deficiency (hyper-immunoglobulin D syndrome, or HIDS) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS). Epidemiology
FMF affects groups of people originating from around the Mediterranean Sea (hence its name). It is most prominently present in the Armenians, Sephardic Jews, Ashkenazi Jews, Mizrahi Jews, Cypriots, Kurds, Turks and Levantines. History
A New York City allergist, Sheppard Siegal, first described the attacks of peritonitis in 1945; he termed this "benign paroxysmal peritonitis", as the disease course was essentially benign.