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Pansynostosis

MedGen UID:
867450
Concept ID:
C4021827
Disease or Syndrome
Synonym: Sysnostosis of all cranial sutures
 
HPO: HP:0011325

Definition

Craniosynostosis of all calvarial sutures. [from HPO]

Conditions with this feature

Craniosynostosis 4
MedGen UID:
322167
Concept ID:
C1833340
Disease or Syndrome
Craniosynostosis (CRS) is a primary abnormality of skull growth involving premature fusion of the cranial sutures such that the growth velocity of the skull often cannot match that of the developing brain. This produces skull deformity and, in some cases, raises intracranial pressure, which must be treated promptly to avoid permanent neurodevelopmental disability (summary by Fitzpatrick, 2013). Craniosynostosis-4 (CRS4) includes lambdoid, sagittal, metopic, coronal, and multisuture forms. For a discussion of genetic heterogeneity of craniosynostosis, see CRS1 (123100).
Robinow-Sorauf syndrome
MedGen UID:
356703
Concept ID:
C1867146
Disease or Syndrome
The signs and symptoms of Saethre-Chotzen syndrome vary widely, even among affected individuals in the same family. This condition can cause mild changes in the hands and feet, such as partial fusion of the skin between the second and third fingers on each hand and a broad or duplicated first (big) toe. Delayed development and learning difficulties have been reported, although most people with this condition are of normal intelligence. Less common signs and symptoms of Saethre-Chotzen syndrome include short stature, abnormalities of the bones of the spine (the vertebra), hearing loss, and heart defects.\n\nMost people with Saethre-Chotzen syndrome have prematurely fused skull bones along the coronal suture, the growth line that goes over the head from ear to ear. Other parts of the skull may be malformed as well. These changes can result in an abnormally shaped head, a high forehead, a low frontal hairline, droopy eyelids (ptosis), widely spaced eyes, and a broad nasal bridge. One side of the face may appear noticeably different from the other (facial asymmetry). Most people with Saethre-Chotzen syndrome also have small, rounded ears.\n\nSaethre-Chotzen syndrome is a genetic condition characterized by the premature fusion of certain skull bones (craniosynostosis). This early fusion prevents the skull from growing normally and affects the shape of the head and face.\n\nRobinow-Sorauf syndrome is a condition with features similar to those of Saethre-Chotzen syndrome, including craniosynostosis and broad or duplicated great toes. It was once considered a separate disorder, but was found to result from mutations in the same gene and is now thought to be a variant of Saethre-Chotzen syndrome.

Professional guidelines

PubMed

Tubbs RS, Sharma A, Griessenauer C, Loukas M, Shoja MM, Watanabe K, Oakes WJ
Childs Nerv Syst 2013 May;29(5):745-8. Epub 2012 Dec 12 doi: 10.1007/s00381-012-1981-8. PMID: 23233211

Recent clinical studies

Etiology

Wu M, Massenburg BB, Reddy N, Romeo DJ, Ng JJ, Taylor JA, Swanson JW, Bartlett SP, Lang SS
Childs Nerv Syst 2024 Jul;40(7):2125-2134. Epub 2024 Apr 3 doi: 10.1007/s00381-024-06379-8. PMID: 38568218
Pontell ME, Barrero CE, Wagner CS, Salinero LK, Swanson JW, Taylor JA, Bartlett SP
Childs Nerv Syst 2023 Nov;39(11):3041-3049. Epub 2023 Jul 26 doi: 10.1007/s00381-023-06048-2. PMID: 37493719
Kilcoyne S, Potter KR, Gordon Z, Overton S, Brockbank S, Jayamohan J, Magdum S, Smith M, Johnson D, Wall S, Wilkie AOM
J Craniofac Surg 2021 Jan-Feb 01;32(1):134-140. doi: 10.1097/SCS.0000000000007153. PMID: 33074973
Rogers GF, Greene AK, Proctor MR, Mulliken JB, Goobie SM, Stoler JM
Cleft Palate Craniofac J 2015 Nov;52(6):751-7. Epub 2014 Oct 28 doi: 10.1597/14-092. PMID: 25350344
Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJ, Maher CO
Neurosurg Focus 2011 Sep;31(3):E2. doi: 10.3171/2011.6.FOCUS11107. PMID: 21882907

Diagnosis

Pontell ME, Barrero CE, Wagner CS, Salinero LK, Swanson JW, Taylor JA, Bartlett SP
Childs Nerv Syst 2023 Nov;39(11):3041-3049. Epub 2023 Jul 26 doi: 10.1007/s00381-023-06048-2. PMID: 37493719
Kilcoyne S, Potter KR, Gordon Z, Overton S, Brockbank S, Jayamohan J, Magdum S, Smith M, Johnson D, Wall S, Wilkie AOM
J Craniofac Surg 2021 Jan-Feb 01;32(1):134-140. doi: 10.1097/SCS.0000000000007153. PMID: 33074973
Di Rocco F, Rothenbuhler A, Cormier Daire V, Bacchetta J, Adamsbaum C, Baujat G, Rossi M, Lingart A
Neurochirurgie 2019 Nov;65(5):258-263. Epub 2019 Sep 25 doi: 10.1016/j.neuchi.2019.09.008. PMID: 31562881
Di Rocco F, Baujat G, Cormier-Daire V, Rothenbuhler A, Linglart A
Arch Pediatr 2017 May;24(5S2):5S89-5S92. doi: 10.1016/S0929-693X(18)30022-8. PMID: 29405940
Rogers GF, Greene AK, Proctor MR, Mulliken JB, Goobie SM, Stoler JM
Cleft Palate Craniofac J 2015 Nov;52(6):751-7. Epub 2014 Oct 28 doi: 10.1597/14-092. PMID: 25350344

Therapy

Talwar AA, Lazar SV, Reategui AA, Sun AH, Kameni LH, Lopez J, Steinbacher DM
J Craniofac Surg 2023 Sep 1;34(6):1709-1712. Epub 2023 Jun 15 doi: 10.1097/SCS.0000000000009495. PMID: 37316986
Zhang RS, Wes AM, Naran S, Hoppe IC, Sun J, Mazzaferro D, Bartlett SP, Taylor JA
J Craniofac Surg 2018 May;29(3):566-571. doi: 10.1097/SCS.0000000000004230. PMID: 29381615
White N, Marcus R, Dover S, Solanki G, Nishikawa H, Millar C, Carver ED
J Craniofac Surg 2009 Mar;20(2):378-81. doi: 10.1097/SCS.0b013e31819b9429. PMID: 19258906
O'Hara CM, Izadi K, Albright AL, Bradley JP
Pediatr Neurosurg 2006;42(2):100-4. doi: 10.1159/000090463. PMID: 16465079
Sabry MZ, Wornom IL 3rd, Ward JD
Ann Plast Surg 2001 Aug;47(2):119-25; discussion 126. doi: 10.1097/00000637-200108000-00003. PMID: 11506318

Prognosis

Wu M, Massenburg BB, Reddy N, Romeo DJ, Ng JJ, Taylor JA, Swanson JW, Bartlett SP, Lang SS
Childs Nerv Syst 2024 Jul;40(7):2125-2134. Epub 2024 Apr 3 doi: 10.1007/s00381-024-06379-8. PMID: 38568218
Pennacchietti V, Schulz M, Tietze A, Schwarz K, Thomale UW
Childs Nerv Syst 2021 Oct;37(10):3199-3207. Epub 2021 Sep 16 doi: 10.1007/s00381-021-05355-w. PMID: 34529089Free PMC Article
Sullivan JS, Snider AE, Farrington J, Shiflett JM, Weaver KJ, Humphries LS, Hoppe IC
Childs Nerv Syst 2021 Jul;37(7):2313-2318. Epub 2021 May 10 doi: 10.1007/s00381-021-05202-y. PMID: 33970330
Langford RJ, Sgouros S, Natarajan K, Nishikawa H, Dover MS, Hockley AD
Plast Reconstr Surg 2003 Apr 15;111(5):1598-604. doi: 10.1097/01.PRS.0000057972.87632.ec. PMID: 12655203
Sgouros S, Hockley AD, Goldin JH, Wake MJ, Natarajan K
J Neurosurg 1999 Oct;91(4):617-25. doi: 10.3171/jns.1999.91.4.0617. PMID: 10507384

Clinical prediction guides

White N, Marcus R, Dover S, Solanki G, Nishikawa H, Millar C, Carver ED
J Craniofac Surg 2009 Mar;20(2):378-81. doi: 10.1097/SCS.0b013e31819b9429. PMID: 19258906
Sgouros S, Hockley AD, Goldin JH, Wake MJ, Natarajan K
J Neurosurg 1999 Oct;91(4):617-25. doi: 10.3171/jns.1999.91.4.0617. PMID: 10507384

Recent systematic reviews

Talwar AA, Lazar SV, Reategui AA, Sun AH, Kameni LH, Lopez J, Steinbacher DM
J Craniofac Surg 2023 Sep 1;34(6):1709-1712. Epub 2023 Jun 15 doi: 10.1097/SCS.0000000000009495. PMID: 37316986

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