Symptoma

Single Central Maxillary Incisor

SMMCI

Four cases are presented as neonatal nasal obstruction and also have single central maxillary incisor (SCMI). Computed tomography examination indicates three of the SCMI are unerupted and one is erupted. [ncbi.nlm.nih.gov]

Her originality is to combine approaches both in mice and humans presenting these palate and teeth developmental defects as clinical phenotypical manifestations of rare diseases. [books.google.com]

To accurately describe the characteristic tooth present in this syndrome, it is necessary to specify: • Solitary: the tooth present exists as the only central incisor tooth in... [causes.com]

To accurately describe the characteristic tooth present, it is necessary to specify: Solitary: The tooth present exists as the only central incisor tooth in the maxilla Median: The tooth is present precisely in the midline of the maxillary alveolus (a [jpbsonline.org]

  • Short Stature

    The fifth case has an erupted SCMI and short stature. The associated SCMI in CNPAS is believed to be more than an isolated anomaly. [ncbi.nlm.nih.gov]

    When the association with short stature was recognised in 1976, the name Monosuperoincisivodontic dwarfism was given by Rappaport et al. [9], but it was soon recognised that short stature was not always present in the disorder [10]. [causes.com]

    stature, choanal atresia/midnasal stenosis (SMMCI) syndrome. ( 9431535 ) Hall R.K....Perks A.G. 1997 [malacards.org]

    Choanal stenosis requires emergency surgical treatment and short stature may require growth hormone therapy. [orpha.net]

    She also suffers from short stature which we were told is due to a partial growth hormone deficiency due to an abnormality with her piturity gland. [inspire.com]

  • Crying

    […] incisor in association with hemifacial microsomia: A rare case report and review of literature. ( 22346174 ) Utreja A....Gupta R. 2011 19 Solitary median maxillary central incisor and congenital nasal pyriform aperture stenosis combined with asymmetric crying [malacards.org]

    Baby had difficulty in breast-feeding and developed cyclical cyanosis, which was relieved by crying. [jcnonweb.com]

    P.s - think the facebook things a good idea x Think the facebook things a good idea I must say i felt like crying when i found this page! My daughter is now 10 and has SMMCI. This was not diagnosed till she was 4. [inspire.com]

    The clinical presentation can be variable, with respiratory distress at birth, cyclical cyanosis relieved by crying, or difficulty in breathing during feeding. [5] Anatomically, the pyriform aperture is bounded laterally by the nasal processes of the [ijri.org]

  • Developmental Delay

    Or developmental delays? You can e-mail me at [email protected] and we can chat via e-mail if you want. I have found two other mothers - one in Australia & one is Sweden. I am in the US. [inspire.com]

  • Anosmia

    The mother had mild hypotelorism but no anosmia, and her brain CT was normal. She was of normal intelligence and stature. Other relatives did not have single central maxillary incisors, hypotelorism, or oral clefts. [ncbi.nlm.nih.gov]

  • Small Head

    Our son except for the one tooth, poor vision and small head- he has a small pituitary gland so since 4 months ago- finally- he was put on growth hormones and other hormones. [inspire.com]

    head circumference in 33%, hypotelorism in 45%, intellectual disability varying in degree in 50% (from slow learning to intellectual retardation), possible association with epilepsy and attention-deficit/hyperactivity disorder, and deviant sella turcica [pocketdentistry.com]

  • Dyslexia

    He may tend towards dyslexia as well, as language is not strong. Am relieved to find more people going through the same stuff. I'll post again once I get some feedback from the specialist to share our findings. [inspire.com]

  • Nystagmus

    I look forward to chatting with you. hi, I am marie and we also have a child with smmci, you can read about us on the facebook group one-tooth-wonder-. our son is 3 yrs old and he also has poor vision, he has nystagmus and "an undiagnosed retinal disease [inspire.com]

They may have neurodevelopmental issues. [11, 12, 13, 14, 15] Workup Prenatal diagnosis options include karyotype analysis, microarray testing, ultrasonography, and magnetic resonance imaging (MRI). [emedicine.medscape.com]

Awareness of the associated features of CNPAS among the radiologist and otolaryngologist may help the diagnosis and treatment of the patient. [ncbi.nlm.nih.gov]

The prosthetic treatment was finished with an all-ceramic crown using a zirconia frame. [jstage.jst.go.jp]

There are two treatment options that exist for replacing missing incisors. [jcrsmed.org]

[…] orthodontic treatment following consultations with an oral surgeon or periodontist and restorative dentist to ensure orthodontic alignment will facilitate the surgical, implant and restorative treatment. 2 Aspects of orthodontic treatment required for [cda-adc.ca]

• Tooth transplantation is considered an advantageous treatment to replace a maxillary incisor. • Orthodontic treatment of adolescents with SMMCI syndrome can be performed successfully. [pocketdentistry.com]

Treatment and prognosis The prognosis is dependent on the type of HPE with almost all alobar and semilobar forms incompatible with extrauterine life. There may be recurrence risk for 6% with non chromosomal sporadic HPE. [radiopaedia.org]

Mighel AS, Johnstone ED, Levene M (2009) Postnatal investigations: management and prognosis for fetuses with CNS anomalies identified in utero excluding neurosurgical problems. Prenat Diagn 29:442–449 CrossRef Google Scholar 9. [link.springer.com]

As the prognosis is good with treatment, early recognition and timely intervention is necessary. [jcnonweb.com]

The Frankfort-mandibular plane angle in orthodontic diagnosis, classification, treatment planning, and prognosis. Am J Orthod Oral Surg 1946;32:175-230. 21. Downs WB. Variations in facial relationships; their significance in treatment and prognosis. [ijdr.in]

The prognosis of holoprosencephaly depends on the severity of the malformation. [emedicine.medscape.com]

The etiology is uncertain. Genetics A missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI 2. [radiopaedia.org]

Etiology The aetiology is uncertain. A missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI. [orpha.net]

Abstract Introduction: The solitary median maxillary central incisor syndrome is a rare disease with a prevalence of 1/50000 infants, predominantly female and of unknown etiology in most cases. [omicsonline.org]

The etiology of both the conditions remains uncertain although various theories have been proposed. [contempclindent.org]

Epidemiology It is estimated to occur in 1:50,000 live births. [radiopaedia.org]

Holoprosencephaly in human embryos: epidemiologic studies of 150 cases. Teratology. 1977 Dec. 16(3):261-72. [Medline]. Orioli IM, Castilla EE. Epidemiology of holoprosencephaly: Prevalence and risk factors. [emedicine.medscape.com]

Summary Epidemiology It is estimated to occur in 1:50,000 live births. [orpha.net]

Pathophysiology A combination of genetic and environmental factors is thought to be responsible for the pathogenesis of holoprosencephaly. [emedicine.medscape.com]

PRF accelerates healing by hastened wound closure due to growth factor release and fibrin bandage. [16] It also acts like an interpositional material preventing migration of undesired cells and accelerates wound healing by forming fibrin bandage and releasing [endodontologyonweb.org]

[…] of an SMMCI tooth is unknown, although Hall et al. [1] have postulated that a critical absence of or reduction in lateral growth from the midline, on or around gestation days 37 or 38, results in premature fusion of the epithelial dental lamina, thus preventing [jpbsonline.org]

Available from: Introduction The main objective of endodontic treatment is the thorough mechanical and chemical cleaning of the entire pulp space, to achieve an appropriate disinfection degree for after filling, with an inert material and a coronal filling, preventing [ejgd.org]

After gathering all clinical records, arriving at a diagnosis, and provision of routine preventive care, restorative treatment planning began. [dentistrytoday.com]

This position may prevent food from accumulating on the cervical "table" of the crown. [slideshare.net]