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Skeletal overgrowth-craniofacial dysmorphism-hyperelastic skin-white matter lesions syndrome(KOGS)

MedGen UID:
896409
Concept ID:
C4225270
Disease or Syndrome
Synonyms: Kosaki overgrowth syndrome; SKELETAL OVERGROWTH WITH FACIAL DYSMORPHISM, HYPERELASTIC SKIN, WHITE MATTER LESIONS, AND NEUROLOGIC DETERIORATION
SNOMED CT: Skeletal overgrowth, craniofacial dysmorphism, hyperelastic skin, white matter lesion syndrome (1172898008); Kosaki overgrowth syndrome (1172898008)
Modes of inheritance:
No inheritance data available
MedGen UID:
989178
Concept ID:
CN307043
Finding
Source: Orphanet
No information is available in the scientific literature on heredity of the clinical entity.
 
Gene (location): PDGFRB (5q32)
 
Monarch Initiative: MONDO:0014704
OMIM®: 616592
Orphanet: ORPHA477831

Definition

Kosaki overgrowth syndrome (KOGS) is characterized by a facial gestalt involving prominent forehead, proptosis, downslanting palpebral fissures, broad nasal bridge, thin upper lip, and pointed chin. Affected individuals are tall, with an elongated lower segment, and have large hands and feet. Skin is hyperelastic and fragile. Patients exhibit progressive dilatory and vascular changes in basilar/vertebral and coronary arteries starting in the teenage years (Takenouchi et al., 2015; Takenouchi et al., 2021). [from OMIM]

Clinical features

From HPO
Myofibromatosis
MedGen UID:
61649
Concept ID:
C0206648
Neoplastic Process
A mesenchymal neoplasm characterized by solitary or multiple nodules involving the skin, striated muscles, bones and, sometimes, viscera. It usually appears as a subcutaneous nodule, but can also appear as an ulcer, pedunculated lesion, or similar to a hemangioma. Histology shows well-circumscribed tapered cell lobes, resembling smooth muscle cells. At its center, perivascular round cells (hemangiopericitoides) are usually observed, giving a biphasic appearance.
Long foot
MedGen UID:
154365
Concept ID:
C0576225
Finding
Increased back to front length of the foot.
Tall stature
MedGen UID:
69137
Concept ID:
C0241240
Finding
A height above that which is expected according to age and gender norms.
Overgrowth
MedGen UID:
376550
Concept ID:
C1849265
Finding
Excessive postnatal growth which may comprise increased weight, increased length, and/or increased head circumference.
Anxiety
MedGen UID:
1613
Concept ID:
C0003467
Finding
Intense feelings of nervousness, tension, or panic often arise in response to interpersonal stresses. There is worry about the negative effects of past unpleasant experiences and future negative possibilities. Individuals may feel fearful, apprehensive, or threatened by uncertainty, and they may also have fears of falling apart or losing control.
Depression
MedGen UID:
4229
Concept ID:
C0011581
Mental or Behavioral Dysfunction
Frequently experiencing feelings of being down, miserable, and/or hopeless; struggling to recover from these moods; having a pessimistic outlook on the future; feeling a pervasive sense of shame; having a low self-worth; experiencing thoughts of suicide and engaging in suicidal behavior.
Auditory hallucination
MedGen UID:
115932
Concept ID:
C0233762
Sign or Symptom
Perception of sounds without auditory stimulus.
Progressive neurologic deterioration
MedGen UID:
381506
Concept ID:
C1854838
Finding
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Thoracolumbar scoliosis
MedGen UID:
196671
Concept ID:
C0749379
Anatomical Abnormality
Downslanted palpebral fissures
MedGen UID:
98391
Concept ID:
C0423110
Finding
The palpebral fissure inclination is more than two standard deviations below the mean.
Depressed nasal bridge
MedGen UID:
373112
Concept ID:
C1836542
Finding
Posterior positioning of the nasal root in relation to the overall facial profile for age.
Prominent forehead
MedGen UID:
373291
Concept ID:
C1837260
Finding
Forward prominence of the entire forehead, due to protrusion of the frontal bone.
Prominent supraorbital ridges
MedGen UID:
333982
Concept ID:
C1842060
Finding
Greater than average forward and/or lateral protrusion of the supraorbital portion of the frontal bones.
Pointed chin
MedGen UID:
336193
Concept ID:
C1844505
Finding
A marked tapering of the lower face to the chin.
Wide nasal bridge
MedGen UID:
341441
Concept ID:
C1849367
Finding
Increased breadth of the nasal bridge (and with it, the nasal root).
Thin upper lip vermilion
MedGen UID:
355352
Concept ID:
C1865017
Finding
Height of the vermilion of the upper lip in the midline more than 2 SD below the mean. Alternatively, an apparently reduced height of the vermilion of the upper lip in the frontal view (subjective).
Xanthelasma
MedGen UID:
56357
Concept ID:
C0155210
Disease or Syndrome
The presence of xanthomata in the skin of the eyelid.
Hyperextensible skin
MedGen UID:
66023
Concept ID:
C0241074
Finding
A condition in which the skin can be stretched beyond normal, and then returns to its initial position.
Fragile skin
MedGen UID:
66826
Concept ID:
C0241181
Finding
Skin that splits easily with minimal injury.
Thin skin
MedGen UID:
140848
Concept ID:
C0423757
Finding
Reduction in thickness of the skin, generally associated with a loss of suppleness and elasticity of the skin.
Ptosis
MedGen UID:
2287
Concept ID:
C0005745
Disease or Syndrome
The upper eyelid margin is positioned 3 mm or more lower than usual and covers the superior portion of the iris (objective); or, the upper lid margin obscures at least part of the pupil (subjective).
Proptosis
MedGen UID:
41917
Concept ID:
C0015300
Disease or Syndrome
An eye that is protruding anterior to the plane of the face to a greater extent than is typical.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSkeletal overgrowth-craniofacial dysmorphism-hyperelastic skin-white matter lesions syndrome

Recent clinical studies

Etiology

Eligulashvili A, Gordon M, Lee JS, Lee J, Mehrotra-Varma S, Mehrotra-Varma J, Hsu K, Hilliard I, Lee K, Li A, Essibayi MA, Yee J, Altschul DJ, Eskandar E, Mehler MF, Duong TQ
PLoS Med 2024 Apr;21(4):e1004263. Epub 2024 Apr 4 doi: 10.1371/journal.pmed.1004263. PMID: 38573873Free PMC Article
Danhelovska T, Kolarova H, Zeman J, Hansikova H, Vaneckova M, Lambert L, Kucerova-Vidrova V, Berankova K, Honzik T, Tesarova M
BMC Pediatr 2020 Jan 29;20(1):41. doi: 10.1186/s12887-020-1912-x. PMID: 31996177Free PMC Article
Sasai H, Shimozawa N, Asano T, Kawamoto N, Yamamoto T, Kimura T, Kawamoto M, Matsui E, Fukao T
Tohoku J Exp Med 2015 Dec;237(4):323-7. doi: 10.1620/tjem.237.323. PMID: 26639091
Freeman AF, Collura-Burke CJ, Patronas NJ, Ilcus LS, Darnell D, Davis J, Puck JM, Holland SM
Pediatrics 2007 May;119(5):e1121-5. Epub 2007 Apr 16 doi: 10.1542/peds.2006-2649. PMID: 17438082
Barker RA, Revesz T, Thom M, Marsden CD, Brown P
J Neurol Neurosurg Psychiatry 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633. PMID: 9810930Free PMC Article

Diagnosis

Danhelovska T, Kolarova H, Zeman J, Hansikova H, Vaneckova M, Lambert L, Kucerova-Vidrova V, Berankova K, Honzik T, Tesarova M
BMC Pediatr 2020 Jan 29;20(1):41. doi: 10.1186/s12887-020-1912-x. PMID: 31996177Free PMC Article
Imagawa E, Fattal-Valevski A, Eyal O, Miyatake S, Saada A, Nakashima M, Tsurusaki Y, Saitsu H, Miyake N, Matsumoto N
J Neurol Neurosurg Psychiatry 2016 Feb;87(2):212-6. Epub 2015 May 20 doi: 10.1136/jnnp-2014-310084. PMID: 25995486
McKelvie P, Infeld B, Marotta R, Chin J, Thorburn D, Collins S
J Clin Neurosci 2012 Feb;19(2):195-202. doi: 10.1016/j.jocn.2011.09.009. PMID: 22273117
Isohanni P, Hakonen AH, Euro L, Paetau I, Linnankivi T, Liukkonen E, Wallden T, Luostarinen L, Valanne L, Paetau A, Uusimaa J, Lönnqvist T, Suomalainen A, Pihko H
Neurology 2011 Mar 1;76(9):811-5. doi: 10.1212/WNL.0b013e31820e7b25. PMID: 21357833
Freeman AF, Collura-Burke CJ, Patronas NJ, Ilcus LS, Darnell D, Davis J, Puck JM, Holland SM
Pediatrics 2007 May;119(5):e1121-5. Epub 2007 Apr 16 doi: 10.1542/peds.2006-2649. PMID: 17438082

Therapy

Sasai H, Shimozawa N, Asano T, Kawamoto N, Yamamoto T, Kimura T, Kawamoto M, Matsui E, Fukao T
Tohoku J Exp Med 2015 Dec;237(4):323-7. doi: 10.1620/tjem.237.323. PMID: 26639091
de la Monte SM, Kril JJ
Acta Neuropathol 2014 Jan;127(1):71-90. Epub 2013 Dec 27 doi: 10.1007/s00401-013-1233-3. PMID: 24370929Free PMC Article
Möller HE, Wiedermann D, Kurlemann G, Hilbich T, Schuierer G
Muscle Nerve 2002 Apr;25(4):593-600. doi: 10.1002/mus.10084. PMID: 11932978

Prognosis

Eligulashvili A, Gordon M, Lee JS, Lee J, Mehrotra-Varma S, Mehrotra-Varma J, Hsu K, Hilliard I, Lee K, Li A, Essibayi MA, Yee J, Altschul DJ, Eskandar E, Mehler MF, Duong TQ
PLoS Med 2024 Apr;21(4):e1004263. Epub 2024 Apr 4 doi: 10.1371/journal.pmed.1004263. PMID: 38573873Free PMC Article
Danhelovska T, Kolarova H, Zeman J, Hansikova H, Vaneckova M, Lambert L, Kucerova-Vidrova V, Berankova K, Honzik T, Tesarova M
BMC Pediatr 2020 Jan 29;20(1):41. doi: 10.1186/s12887-020-1912-x. PMID: 31996177Free PMC Article
Sasai H, Shimozawa N, Asano T, Kawamoto N, Yamamoto T, Kimura T, Kawamoto M, Matsui E, Fukao T
Tohoku J Exp Med 2015 Dec;237(4):323-7. doi: 10.1620/tjem.237.323. PMID: 26639091
McKelvie P, Infeld B, Marotta R, Chin J, Thorburn D, Collins S
J Clin Neurosci 2012 Feb;19(2):195-202. doi: 10.1016/j.jocn.2011.09.009. PMID: 22273117
Barker RA, Revesz T, Thom M, Marsden CD, Brown P
J Neurol Neurosurg Psychiatry 1998 Nov;65(5):633-40. doi: 10.1136/jnnp.65.5.633. PMID: 9810930Free PMC Article

Clinical prediction guides

Eligulashvili A, Gordon M, Lee JS, Lee J, Mehrotra-Varma S, Mehrotra-Varma J, Hsu K, Hilliard I, Lee K, Li A, Essibayi MA, Yee J, Altschul DJ, Eskandar E, Mehler MF, Duong TQ
PLoS Med 2024 Apr;21(4):e1004263. Epub 2024 Apr 4 doi: 10.1371/journal.pmed.1004263. PMID: 38573873Free PMC Article
Mori F, Rossi S, Sancesario G, Codecà C, Mataluni G, Monteleone F, Buttari F, Kusayanagi H, Castelli M, Motta C, Studer V, Bernardi G, Koch G, Bernardini S, Centonze D
Neuropsychopharmacology 2011 Feb;36(3):559-68. Epub 2010 Oct 13 doi: 10.1038/npp.2010.187. PMID: 20944553Free PMC Article
Le Ber I, Martinez M, Campion D, Laquerrière A, Bétard C, Bassez G, Girard C, Saugier-Veber P, Raux G, Sergeant N, Magnier P, Maisonobe T, Eymard B, Duyckaerts C, Delacourte A, Frebourg T, Hannequin D
Brain 2004 Sep;127(Pt 9):1979-92. Epub 2004 Jun 23 doi: 10.1093/brain/awh216. PMID: 15215218
Möller HE, Wiedermann D, Kurlemann G, Hilbich T, Schuierer G
Muscle Nerve 2002 Apr;25(4):593-600. doi: 10.1002/mus.10084. PMID: 11932978
Guo Y, Guo Z, Chen L, Zhang J, Wang W, Liu X, Ren H, Gao S
Chin Med J (Engl) 1997 Nov;110(11):851-5. PMID: 9772417

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