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Bicoronal synostosis

MedGen UID:
866810
Concept ID:
C4021164
Congenital Abnormality
Synonyms: Bilateral coronal craniosynostosis; Bilateral coronal suture craniosynostosis; Bilateral coronal suture synostosis
 
HPO: HP:0011318

Definition

Synostosis affecting the right and the left coronal suture. [from HPO]

Conditions with this feature

Baller-Gerold syndrome
MedGen UID:
120532
Concept ID:
C0265308
Disease or Syndrome
Baller-Gerold syndrome (BGS) can be suspected at birth in an infant with craniosynostosis and upper limb abnormality. The coronal suture is most commonly affected; the metopic, lambdoid, and sagittal sutures may also be involved alone or in combination. Upper limb abnormality can include a combination of thumb hypo- or aplasia and radial hypo- or aplasia and may be asymmetric. Malformation or absence of carpal or metacarpal bones has also been described. Skin lesions may appear anytime within the first few years after birth, typically beginning with erythema of the face and extremities and evolving into poikiloderma. Slow growth is apparent in infancy with eventual height and length typically at 4 SD below the mean.
Curry-Jones syndrome
MedGen UID:
167083
Concept ID:
C0795915
Disease or Syndrome
Curry-Jones syndrome (CRJS) is a multisystem disorder characterized by patchy skin lesions, polysyndactyly, diverse cerebral malformations, unicoronal craniosynostosis, iris colobomas, microphthalmia, and intestinal malrotation with myofibromas or hamartomas (summary by Twigg et al., 2016).
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).
Craniosynostosis 2
MedGen UID:
346753
Concept ID:
C1858160
Disease or Syndrome
Craniosynostosis 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). For a discussion of genetic heterogeneity of craniosynostosis, see CRS1 (123100).
TCF12-related craniosynostosis
MedGen UID:
811568
Concept ID:
C3715051
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-3 (CRS3) includes coronal, sagittal, and multisuture forms (Sharma et al., 2013). For discussion of genetic heterogeneity of craniosynostosis, see CRS1 (123100).
Craniosynostosis 6
MedGen UID:
904675
Concept ID:
C4225269
Disease or Syndrome
Craniosynostosis 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-6 is a bicoronal form associated with bony defects in the sagittal, metopic, or lambdoid sutures (Twigg et al., 2015). For a discussion of genetic heterogeneity of craniosynostosis, see CRS1 (123100). Structural brain anomalies with impaired intellectual development and craniosynostosis (BAIDCS; 618736) is an allelic disorder.
Structural brain anomalies with impaired intellectual development and craniosynostosis
MedGen UID:
1684861
Concept ID:
C5231485
Disease or Syndrome
Patients with BAIDCS have small head circumference with abnormalities in brain anatomy including variable deficiency of the corpus callosum (including agenesis), abnormal conformation of the ventricles and posterior fossa, hypoplasia of both cerebellar hemispheres, colpocephaly, and partial rhombencephalosynapsis (absence of the cerebellar vermis with fusion of the cerebellar hemispheres). Intellectual development is moderately to severely impaired. Bicoronal synostosis, scoliosis, and tethered cord may be present (Twigg et al., 2015; Vandervore et al., 2018). Craniosynostosis-6 (CRS6; 616602) is an allelic disorder.
Short stature, facial dysmorphism, and skeletal anomalies with or without cardiac anomalies 2
MedGen UID:
1782253
Concept ID:
C5543057
Disease or Syndrome
Short stature, facial dysmorphism, and skeletal anomalies with or without cardiac anomalies-2 (SSFSC2) is characterized by thin and short long bones, distinctive facial dysmorphism, and dental and skeletal abnormalities, in the absence of developmental delay or intellectual disability. Cardiac anomalies have been reported in some patients (Lin et al., 2021). For a discussion of genetic heterogeneity of SSFSC, see SSFSC1 (617877).
Hypogonadotropic hypogonadism 26 with or without anosmia
MedGen UID:
1811919
Concept ID:
C5676903
Disease or Syndrome
HH26 is characterized by micropenis and cryptorchidism at birth in male patients, and absent puberty and anosmia in male or female patients. Some affected individuals also exhibit craniosynostosis (Davis et al., 2020). Congenital idiopathic hypogonadotropic hypogonadism (IHH) is a disorder characterized by absent or incomplete sexual maturation by the age of 18 years, in conjunction with low levels of circulating gonadotropins and testosterone and no other abnormalities of the hypothalamic-pituitary axis. Idiopathic hypogonadotropic hypogonadism can be caused by an isolated defect in gonadotropin-releasing hormone (GNRH; 152760) release, action, or both. Other associated nonreproductive phenotypes, such as anosmia, cleft palate, and sensorineural hearing loss, occur with variable frequency. In the presence of anosmia, idiopathic hypogonadotropic hypogonadism has been called 'Kallmann syndrome (KS),' whereas in the presence of a normal sense of smell, it has been termed 'normosmic idiopathic hypogonadotropic hypogonadism (nIHH)' (summary by Raivio et al., 2007). Because families have been found to segregate both KS and nIHH, the disorder is here referred to as 'hypogonadotropic hypogonadism with or without anosmia (HH).' For a discussion of genetic heterogeneity of hypogonadotropic hypogonadism with or without anosmia as well as a discussion of oligogenicity of this disorder, see 147950.
Tessadori-Van Haaften neurodevelopmental syndrome 4
MedGen UID:
1804234
Concept ID:
C5677016
Disease or Syndrome
Tessadori-Bicknell-van Haaften neurodevelopmental syndrome-4 (TEBIVANED4) is characterized by global developmental delay with poor overall growth, variably impaired intellectual development, learning difficulties, distal skeletal anomalies, and dysmorphic facies. Some patients have visual or hearing deficits. The severity and manifestations of the disorder are highly variable (Tessadori et al., 2022). For a discussion of genetic heterogeneity of TEBIVANED, see TEBIVANED1 (619758).

Professional guidelines

PubMed

Kennedy-Williams P, Care H, Dalton L, Horton J, Kearney A, Rooney N, Hotton M, Pinckston M, Huggons E, Culshaw L, Kilcoyne S, Johnson D, Wilkie AOM, Wall S
J Craniofac Surg 2021 May 1;32(Suppl 3):1263-1268. doi: 10.1097/SCS.0000000000007535. PMID: 33904513
Kim JS, Block LM, Zhu X, Davit AJ
Tech Hand Up Extrem Surg 2020 May 8;25(1):30-34. doi: 10.1097/BTH.0000000000000295. PMID: 32398550

Recent clinical studies

Etiology

Lu X, Forte AJ, Dinis J, Junn A, Alperovich M, Alonso N, Persing JA
Plast Reconstr Surg 2022 May 1;149(5):930e-942e. Epub 2022 Mar 14 doi: 10.1097/PRS.0000000000009051. PMID: 35286288
Lu X, Forte AJ, Junn A, Dinis J, Alperovich M, Alonso N, Persing JA
J Craniomaxillofac Surg 2022 May;50(5):406-418. Epub 2021 Jun 23 doi: 10.1016/j.jcms.2021.06.012. PMID: 35065874
Lu X, Forte AJ, Wilson AT, Park KE, Allam O, Alperovich M, Steinbacher DM, Alonso N, Persing JA
Plast Reconstr Surg 2021 Jul 1;148(1):145-156. doi: 10.1097/PRS.0000000000008101. PMID: 34181610
Vinchon M
Neurochirurgie 2019 Nov;65(5):252-257. Epub 2019 Sep 13 doi: 10.1016/j.neuchi.2019.09.002. PMID: 31525394
Woods RH, Ul-Haq E, Wilkie AOM, Jayamohan J, Richards PG, Johnson D, Lester T, Wall SA
Plast Reconstr Surg 2009 Jun;123(6):1801-1810. doi: 10.1097/PRS.0b013e3181a3f391. PMID: 19483581Free PMC Article

Diagnosis

Lu X, Forte AJ, Wilson AT, Park KE, Allam O, Alperovich M, Steinbacher DM, Alonso N, Persing JA
Plast Reconstr Surg 2021 Jul 1;148(1):145-156. doi: 10.1097/PRS.0000000000008101. PMID: 34181610
Lu X, Forte AJ, Wilson AT, Park KE, Allam O, Mozaffari MA, Alperovich M, Steinbacher DM, Alonso N, Persing JA
Plast Reconstr Surg 2020 Sep;146(3):599-610. doi: 10.1097/PRS.0000000000007068. PMID: 32842109
Vinchon M
Neurochirurgie 2019 Nov;65(5):252-257. Epub 2019 Sep 13 doi: 10.1016/j.neuchi.2019.09.002. PMID: 31525394
Murali CN, McDonald-McGinn DM, Wenger TL, McDougall C, Stroup BM, Sheppard SE, Taylor J, Bartlett SP, Bhoj EJ, Zackai EH, Santani A
Am J Med Genet A 2019 Aug;179(8):1442-1450. Epub 2019 May 20 doi: 10.1002/ajmg.a.61199. PMID: 31111620Free PMC Article
Bastidas N, Mackay DDJ, Taylor JA, Bartlett SP
Plast Reconstr Surg 2012 Oct;130(4):877-883. doi: 10.1097/PRS.0b013e318262f2fd. PMID: 22691840

Therapy

Williams CT, Segar DJ, Naidoo SD, Skolnick GB, Proctor MR, Smyth MD, Patel KB
J Craniofac Surg 2019 Mar/Apr;30(2):453-457. doi: 10.1097/SCS.0000000000005118. PMID: 30640858Free PMC Article
Britto JA, Gwanmesia I, Leshem D
Childs Nerv Syst 2012 Sep;28(9):1375-80. Epub 2012 Aug 8 doi: 10.1007/s00381-012-1806-9. PMID: 22872251
Bastidas N, Mackay DDJ, Taylor JA, Bartlett SP
Plast Reconstr Surg 2012 Oct;130(4):877-883. doi: 10.1097/PRS.0b013e318262f2fd. PMID: 22691840
Seruya M, Oh AK, Boyajian MJ, Posnick JC, Myseros JS, Yaun AL, Keating RF
Plast Reconstr Surg 2011 Jun;127(6):2397-2406. doi: 10.1097/PRS.0b013e318213a178. PMID: 21311385

Prognosis

Vinchon M
Neurochirurgie 2019 Nov;65(5):252-257. Epub 2019 Sep 13 doi: 10.1016/j.neuchi.2019.09.002. PMID: 31525394
Williams CT, Segar DJ, Naidoo SD, Skolnick GB, Proctor MR, Smyth MD, Patel KB
J Craniofac Surg 2019 Mar/Apr;30(2):453-457. doi: 10.1097/SCS.0000000000005118. PMID: 30640858Free PMC Article
Assadsangabi R, Hajmomenian M, Nabavizadeh SA, Schmitt JE, Vossough A
Pediatr Neurol 2018 Feb;79:3-7. Epub 2017 Oct 9 doi: 10.1016/j.pediatrneurol.2017.09.018. PMID: 29290519
Stella I, Vinchon M, Guerreschi P, De Berranger E, Bouacha I
Childs Nerv Syst 2017 Dec;33(12):2181-2186. Epub 2017 Jul 31 doi: 10.1007/s00381-017-3553-4. PMID: 28762040
Bastidas N, Mackay DDJ, Taylor JA, Bartlett SP
Plast Reconstr Surg 2012 Oct;130(4):877-883. doi: 10.1097/PRS.0b013e318262f2fd. PMID: 22691840

Clinical prediction guides

Lu X, Forte AJ, Dinis J, Junn A, Alperovich M, Alonso N, Persing JA
Plast Reconstr Surg 2022 May 1;149(5):930e-942e. Epub 2022 Mar 14 doi: 10.1097/PRS.0000000000009051. PMID: 35286288
Williams CT, Segar DJ, Naidoo SD, Skolnick GB, Proctor MR, Smyth MD, Patel KB
J Craniofac Surg 2019 Mar/Apr;30(2):453-457. doi: 10.1097/SCS.0000000000005118. PMID: 30640858Free PMC Article
Assadsangabi R, Hajmomenian M, Nabavizadeh SA, Schmitt JE, Vossough A
Pediatr Neurol 2018 Feb;79:3-7. Epub 2017 Oct 9 doi: 10.1016/j.pediatrneurol.2017.09.018. PMID: 29290519
Assadsangabi R, Hajmomenian M, Bilaniuk LT, Vossough A
Childs Nerv Syst 2015 May;31(5):735-41. Epub 2015 Feb 17 doi: 10.1007/s00381-015-2639-0. PMID: 25686894
Tovetjärn R, Maltese G, Kölby L, Kreiborg S, Tarnow P
J Craniofac Surg 2012 Jul;23(4):977-81. doi: 10.1097/SCS.0b013e31824e2bec. PMID: 22777440

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