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Craniosynostoses (Craniosynostosis)

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Premature closure of one or more CRANIAL SUTURES. It often results in plagiocephaly. Craniosynostoses that involve multiple sutures are sometimes associated with congenital syndromes such as ACROCEPHALOSYNDACTYLIA; and CRANIOFACIAL DYSOSTOSIS.

Also Known As:

Craniosynostosis; Acrocephaly; Oxycephaly; Brachycephaly; Craniostenosis; Craniosynostosis, Lambdoidal; Lambdoid Synostosis; Metopic Synostosis; Plagiocephaly, Craniosynostosis; Plagiocephaly, Synostotic; Sagittal Synostosis; Scaphocephaly; Synostotic Anterior Plagiocephaly; Synostotic Posterior Plagiocephaly; Trigonocephaly; Unilateral Coronal Synostosis; Anterior Plagiocephalies, Synostotic; Anterior Plagiocephaly, Synostotic; Brachycephalies; Coronal Synostoses, Unilateral; Coronal Synostosis, Unilateral; Craniostenoses; Craniosynostoses, Lambdoidal; Craniosynostosis Plagiocephalies; Craniosynostosis Plagiocephaly; Lambdoid Synostoses; Lambdoidal Craniosynostoses; Lambdoidal Craniosynostosis; Metopic Synostoses; Plagiocephalies, Craniosynostosis; Plagiocephalies, Synostotic; Plagiocephalies, Synostotic Anterior; Plagiocephalies, Synostotic Posterior; Plagiocephaly, Synostotic Anterior; Plagiocephaly, Synostotic Posterior; Posterior Plagiocephalies, Synostotic; Posterior Plagiocephaly, Synostotic; Sagittal Synostoses; Scaphocephalies; Synostoses, Lambdoid; Synostoses, Metopic; Synostoses, Sagittal; Synostoses, Unilateral Coronal; Synostosis, Lambdoid; Synostosis, Metopic; Synostosis, Sagittal; Synostosis, Unilateral Coronal; Synostotic Anterior Plagiocephalies; Synostotic Plagiocephalies; Synostotic Plagiocephaly; Synostotic Posterior Plagiocephalies; Trigonocephalies; Unilateral Coronal Synostoses

Networked: 1464 relevant articles (31 outcomes, 135 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Synostosis
2.	Congenital Abnormalities (Deformity)
3.	Hydrocephalus (Hydrocephaly)
4.	Craniofacial Abnormalities
5.	Airway Obstruction (Choking)

Experts

1.	Speltz, Matthew L: 23 articles (10/2015 - 12/2004)
2.	Cunningham, Michael L: 20 articles (07/2015 - 04/2004)
3.	Mooney, Mark P: 20 articles (05/2015 - 01/2002)
4.	Bartlett, Scott P: 19 articles (10/2015 - 06/2002)
5.	Wilkie, Andrew O M: 18 articles (09/2015 - 02/2002)
6.	Kapp-Simon, Kathleen A: 17 articles (07/2015 - 12/2004)
7.	Rogers, Gary F: 15 articles (01/2015 - 09/2002)
8.	Wall, Steven A: 13 articles (09/2015 - 02/2002)
9.	Mathijssen, Irene M J: 13 articles (09/2015 - 06/2006)
10.	Taylor, Jesse A: 12 articles (10/2015 - 04/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Craniosynostoses:

1.	remifentanil (Ultiva)FDA Link 01/01/2002 - "Safety and efficacy of remifentanil infusion in craniosynostosis repair in infants." 06/01/2001 - "Safety and efficacy of remifentanil infusion in craniosynostosis repair in infants." 08/01/2000 - "Safety and efficacy of remifentanil in craniosynostosis repair in children less than 1 year old." 08/01/2005 - "Twenty-two infants with craniosynostosis received a slow bolus of remifentanil followed by continuous infusion. " 08/01/2005 - "The aim of the present study was to compare the efficacy of isoflurane-remifentanil and sevoflurane-remifentanil combinations during neurosurgical correction craniosynostosis. "
2.	CosmeticsIBA 02/01/1990 - "Recognition of the prolapsed gland and its replacement into the lacrimal gland fossa in craniosynostosis syndromes, as well as in cases of poor function congenital ptosis in general, allows the temporal eyelid to approach a more normal position, yielding an improved functional and cosmetic result." 11/01/2007 - "An observational study was undertaken of the clinical outcome, operative data, complications and cosmetic satisfaction of these trigonocephaly patients. " 11/01/2006 - "A number of studies have shown good short-term cosmetic outcomes following surgery for isolated sagittal craniosynostosis. " 03/01/2003 - "This study demonstrates that the majority of cases with nonsyndromic trigonocephaly are sporadic and benign, apart from the associated cosmetic implications. " 11/01/2000 - "The study of a personal series of 2,137 craniosynostoses shows that the functional and the cosmetic results are better after early surgery, and that the operative risks are not higher in infants than in older children."
3.	Tranexamic Acid (AMCA)FDA Link 04/01/2011 - "Efficacy of tranexamic acid in pediatric craniosynostosis surgery: a double-blind, placebo-controlled trial." 09/01/2015 - "Hence, the aim of the present study was to investigate the antifibrinolytic effect of tranexamic acid (TXA) on intraoperative blood loss and RBC transfusion rates during fronto-orbital advancement (FOA) in isolated metopic synostosis. " 09/01/2015 - "The value of tranexamic acid during fronto-orbital advancement in isolated metopic craniosynostosis." 01/01/2015 - "Use of tranexamic acid in craniosynostosis surgery." 04/01/2013 - "Population pharmacokinetics of tranexamic acid in paediatric patients undergoing craniosynostosis surgery."
4.	Erythropoietin FDA Link 04/01/2011 - "In children undergoing surgical correction of craniosynostosis and pretreated with erythropoietin, intraoperative TXA reduces the transfusion requirement." 06/01/2003 - "Erythropoietin therapy and acute preoperative normovolaemic haemodilution in infants undergoing craniosynostosis surgery." 06/01/2002 - "A total of 29 patients (<8 years) undergoing craniosynostosis repair were randomized into two groups: one received preoperative erythropoietin (600 U/kg) weekly for 3 weeks, and the other served as a control. " 06/01/2002 - "This prospective, single-blinded, randomized study was undertaken to examine the safety and efficacy of preoperative single weekly dosing with erythropoietin (epoetin alfa) in reducing the rate of transfusion in infants and small children undergoing craniosynostosis repair. " 06/01/2003 - "A retrospective study was performed to evaluate whether pretreatment with erythropoietin and iron combined with acute preoperative normovolaemic haemodilution (APNH) could decrease homologous blood transfusion in craniosynostosis (CS) surgery. "
5.	SASIBA 01/01/2010 - "Spring-assisted surgery (SAS) has demonstrated promising results for the treatment of sagittal craniosynostosis. " 01/01/2008 - "The first 20 consecutive patients who underwent SAS for isolated sagittal synostosis with complete records, and who were 3 years old at the time of this study, were included. " 01/01/2008 - "Spring-assisted cranioplasty (SAS) has been used for the treatment of selected cases of sagittal synostosis at our unit routinely since 1998. "
6.	Epoetin Alfa (Epogen)FDA Link 06/01/2002 - "This prospective, single-blinded, randomized study was undertaken to examine the safety and efficacy of preoperative single weekly dosing with erythropoietin (epoetin alfa) in reducing the rate of transfusion in infants and small children undergoing craniosynostosis repair. "
7.	Isoflurane FDA LinkGeneric 08/01/2005 - "The aim of the present study was to compare the efficacy of isoflurane-remifentanil and sevoflurane-remifentanil combinations during neurosurgical correction craniosynostosis. " 08/01/2005 - "Sevoflurane-remifentanil vs isoflurane-remifentanil for the surgical correction of craniosynostosis in infants."
8.	sevoflurane (Ultane)FDA LinkGeneric 08/01/2005 - "The aim of the present study was to compare the efficacy of isoflurane-remifentanil and sevoflurane-remifentanil combinations during neurosurgical correction craniosynostosis. " 08/01/2005 - "Sevoflurane-remifentanil vs isoflurane-remifentanil for the surgical correction of craniosynostosis in infants."
9.	Neuraminidase deficiency with beta-galactosidase deficiencyIBA 03/19/1998 - "The apparently diagnostic findings of the 15 patients with this syndrome may be helpful in differentiating between Shprintzen-Goldberg syndrome and other syndromes with craniosynostosis and marfanoid habitus." 01/01/2014 - "Shprintzen-Goldberg syndrome (S-G) is a rare connective tissue disorder characterised by craniosynostosis, craniofacial dysmorphism, skeletal, cardiovascular, neurological, and other abnormalities. " 03/01/2011 - "Shprintzen-Goldberg syndrome (SGS) is characterized by craniosynostosis and marfanoid habitus. " 06/15/2005 - "The Shprintzen-Goldberg syndrome (SGS) is a disorder of unknown cause comprising craniosynostosis, a marfanoid habitus and skeletal, neurological, cardiovascular, and connective-tissue anomalies. " 03/19/1998 - "Shprintzen-Goldberg syndrome is one of a group of disorders characterized by craniosynostosis and marfanoid habitus. "
10.	N- (4'- fluorobutyrophenone)- 4- (4- chlorophenyl)pyridinium (HPP(+))IBA 05/01/2015 - "The authors discuss their case series of 4 HPP patients treated at their institution with ERT who have undergone successful surgical intervention for craniosynostosis. " 05/01/2015 - "Traditionally, HPP has had a poor prognosis, with few children surviving to exhibit the phenotype of clinical craniosynostosis that requires surgical intervention. " 05/01/2015 - "Patients with HPP have defective bone mineralization as well as craniosynostosis that can be seen in the infantile and childhood forms of this disease. " 01/01/2015 - "These results are very promising especially with regard to the skeletal phenotype but it is unclear whether ERT also has beneficial effects for craniosynostosis and in other affected tissues in HPP such as brain and kidney. " 10/01/2014 - "These findings demonstrate that Alpl(-/-) mice exhibit a craniofacial skeletal phenotype similar to that seen in infants with HPP, including true bony craniosynostosis in the context of severely diminished bone mineralization. "

Therapies and Procedures

Distraction Osteogenesis

01/01/2009 - "Recently, distraction osteogenesis (DO) was reported to be effective in treating most craniosynostosis cases. "
01/01/2014 - "Combination of distraction osteogenesis with strip craniectomy for the management of craniosynostosis is an effective treatment modality with promising results."
01/01/2006 - "After only unidirectional expansion without vertical reduction, the shape of the cranium was satisfactorily improved, which might have been an illusion caused by the relative reduction attribut