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Spondylocostal dysostosis-anal and genitourinary malformations syndrome

MedGen UID:
341373
Concept ID:
C1849069
Congenital Abnormality; Disease or Syndrome
Synonyms: CASAMASSIMA-MORTON-NANCE SYNDROME; CMN SYNDROME; Spondylocostal dysostosis with anal atresia and urogenital anomalies
SNOMED CT: Spondylocostal dysostosis with anal atresia and genitourinary malformation syndrome (723610009); Casamassima Morton Nance syndrome (723610009)
 
Monarch Initiative: MONDO:0010069
OMIM®: 271520

Definition

Spondylocostal dysostosis-anal and genitourinary malformations syndrome is characterized by the association of spondylocostal dysostosis with anal and genitourinary malformations (anal atresia and agenesis of external and internal genitalia). To date, only four cases have been described in the literature. Autosomal recessive inheritance has been suggested. [from MONDO]

Clinical features

From HPO
Hydronephrosis
MedGen UID:
42531
Concept ID:
C0020295
Disease or Syndrome
Severe distention of the kidney with dilation of the renal pelvis and calices.
Atresia of urethra
MedGen UID:
576882
Concept ID:
C0345345
Congenital Abnormality
Congenital anomaly characterized by closure or failure to develop an opening in the urethra.
Aplasia of the uterus
MedGen UID:
98421
Concept ID:
C0425913
Finding
Aplasia of the uterus.
Aplasia of the vagina
MedGen UID:
330738
Concept ID:
C1841990
Finding
Aplasia of the vagina.
Absent external genitalia
MedGen UID:
338563
Concept ID:
C1848869
Congenital Abnormality
Lack of external genitalia in a male or female individual.
Abdominal distention
MedGen UID:
34
Concept ID:
C0000731
Finding
Distention of the abdomen.
Imperforate anus
MedGen UID:
1997
Concept ID:
C0003466
Congenital Abnormality
Congenital absence of the anus, i.e., the opening at the bottom end of the intestinal tract.
Inguinal hernia
MedGen UID:
6817
Concept ID:
C0019294
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Dolichocephaly
MedGen UID:
65142
Concept ID:
C0221358
Congenital Abnormality
An abnormality of skull shape characterized by a increased anterior-posterior diameter, i.e., an increased antero-posterior dimension of the skull. Cephalic index less than 76%. Alternatively, an apparently increased antero-posterior length of the head compared to width. Often due to premature closure of the sagittal suture.
Hemivertebrae
MedGen UID:
82720
Concept ID:
C0265677
Congenital Abnormality
Absence of one half of the vertebral body.
Supernumerary vertebrae
MedGen UID:
75583
Concept ID:
C0265681
Congenital Abnormality
Rib fusion
MedGen UID:
78570
Concept ID:
C0265695
Congenital Abnormality
Complete or partial merging of adjacent ribs.
Missing ribs
MedGen UID:
98093
Concept ID:
C0426816
Finding
A developmental anomaly with absence of one or more ribs.
Short ribs
MedGen UID:
98094
Concept ID:
C0426817
Finding
Reduced rib length.
Thin ribs
MedGen UID:
98095
Concept ID:
C0426818
Finding
Ribs with a reduced diameter.
Block vertebrae
MedGen UID:
375498
Concept ID:
C1844753
Congenital Abnormality
Congenital synostosis between two or more adjacent vertebrae (partial or complete fusion of adjacent vertabral bodies).
Vertebral fusion
MedGen UID:
480139
Concept ID:
C3278509
Anatomical Abnormality
A developmental defect leading to the union of two adjacent vertebrae.
Pulmonary hypoplasia
MedGen UID:
78574
Concept ID:
C0265783
Congenital Abnormality
A congenital abnormality in which the lung parenchyma is not fully developed. It may be associated with other congenital abnormalities.
Short neck
MedGen UID:
99267
Concept ID:
C0521525
Finding
Diminished length of the neck.
Oligohydramnios
MedGen UID:
86974
Concept ID:
C0079924
Pathologic Function
Diminished amniotic fluid volume in pregnancy.
Single umbilical artery
MedGen UID:
278026
Concept ID:
C1384670
Congenital Abnormality
Single umbilical artery (SUA) is the absence of one of the two umbilical arteries surrounding the fetal bladder and in the fetal umbilical cord.
Hypertelorism
MedGen UID:
9373
Concept ID:
C0020534
Finding
Although hypertelorism means an excessive distance between any paired organs (e.g., the nipples), the use of the word has come to be confined to ocular hypertelorism. Hypertelorism occurs as an isolated feature and is also a feature of many syndromes, e.g., Opitz G syndrome (see 300000), Greig cephalopolysyndactyly (175700), and Noonan syndrome (163950) (summary by Cohen et al., 1995).

Recent clinical studies

Etiology

Li G, Strong A, Wang H, Kim JS, Watson D, Zhao S, Vaccaro C, Hartung E, Hakonarson H, Zhang TJ, Giampietro PF, Wu N
Am J Med Genet A 2022 Dec;188(12):3469-3481. Epub 2022 Sep 26 doi: 10.1002/ajmg.a.62972. PMID: 36161696Free PMC Article

Diagnosis

Stoll C, Alembik Y, Dott B, Roth MP
Genet Couns 2008;19(3):331-9. PMID: 18990989
Bannykh SI, Emery SC, Gerber JK, Jones KL, Benirschke K, Masliah E
Am J Med Genet A 2003 Jul 15;120A(2):241-6. doi: 10.1002/ajmg.a.20192. PMID: 12833407

Prognosis

Nagasawa H, Koyama T, Sasai H, Kohno Y, Yamamoto Y, Kondo M, Sugawara M, Terazawa D, Miura R
Congenit Anom (Kyoto) 2014 Aug;54(3):189-92. doi: 10.1111/cga.12052. PMID: 24666313
Onay OS, Kinik ST, Otgün Y, Arda IS, Varan B
Eur J Pediatr Surg 2008 Aug;18(4):272-4. Epub 2008 Jul 15 doi: 10.1055/s-2008-1038364. PMID: 18629769

Clinical prediction guides

Stoll C, Alembik Y, Dott B, Roth MP
Genet Couns 2008;19(3):331-9. PMID: 18990989

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