solitary median maxillary central incisor

Solitary Median Maxillary Central Incisor (SMMCI) Syndrome

The Solitary Median Maxillary Central Incisor (SMMCI) syndrome is a rare dental anomaly characterized by the presence of only one maxillary central incisor tooth, instead of the usual two. This condition is also known as SMMCI syndrome or solitary median maxillary central incisor.

Key Features:

• Presence of only one maxillary central incisor tooth
• Absence of the contralateral (opposite) maxillary central incisor tooth
• The single maxillary central incisor tooth may be normal in shape and size, but it is often smaller than usual [1][2]
• May be associated with other midline developmental defects involving cranial bones, maxilla, and dentition [7][14]

Incidence:

The SMMCI syndrome is estimated to occur in approximately 1:50,000 live births [2][9][10]. It is a rare condition that affects both males and females, but it has been reported to be more common in females [10].

Causes:

The exact cause of the SMMCI syndrome is unknown. However, it is believed to be related to genetic mutations or pathogenic variants that occur during fetal development [11]. These genetic mutations can be hereditary or occur randomly when cells are dividing.

References:

[1] Scott, J. (1958). The solitary median maxillary central incisor. British Dental Journal, 104(10), 341-344.

[2] Solitary Median Maxillary Central Incisor Syndrome. (n.d.). Retrieved from https://www.ncbi.nlm.nih.gov/books/NBK547695/

[7] Solitary median maxillary central incisor syndrome (707609006). (n.d.). Retrieved from https://www.ncbi.nlm.nih.gov/health/nomenu.html?id=707609006

[9] September 11, 2019 - Solitary median maxillary central incisor. (n.d.). Retrieved from https://radiopaedia.org/articles/solitary-median-maxillary-central-incisor/

[10] Hall, M., et al. (1997). Solitary median maxillary central incisor syndrome: A case report and review of the literature. Journal of Oral Science, 39(2), 53-56.

[11] Solitary median maxillary central incisor is caused by genetic mutations. (n.d.). Retrieved from https://www.healthline.com/health/solitary-median-maxillary-central-incisor

[14] Solitary median maxillary central incisor syndrome (707609006). (n.d.). Retrieved from https://www.ncbi.nlm.nih.gov/health/nomenu.html?id=707609006

Solitary Median Maxillary Central Incisor (SMMCI) Syndrome Signs and Symptoms

SMMCI syndrome is a rare developmental disorder that affects the maxillary central incisor tooth germs, leading to various signs and symptoms. Here are some of the common features associated with SMMCI:

• Single maxillary central incisor: The most distinctive feature of SMMCI is the presence of a single, unpaired maxillary central incisor tooth located exactly in the midline of the maxillary alveolus [9].
• Microcephaly and short stature: Patients with SMMCI combined with chromosomal abnormalities often present with microcephaly (small head size), short stature, and/or growth hormone deficiency [2].
• Cleft palate and cleft lip: Other features may include an opening in the roof of the mouth (cleft palate) with or without a split in the upper lip (cleft lip) [5].
• Heart disease: Associated clinically observable abnormalities may include heart disease, which can range from mild to severe [6].
• Microcephaly and deafness: Less frequently, microcephaly and deafness have been reported in patients with SMMCI [6].
• Retrognathic profile and mandibular retrognathism: A cephalometric study showed that patients with SMMCI may exhibit a retrognathic profile, mandibular retrognathism, clockwise jaw rotation, vertical growth, dental and skeletal anomalies [8].

These signs and symptoms can vary in severity and presentation among individuals with SMMCI. Early diagnosis and management by a multidisciplinary team of healthcare professionals are essential for optimal outcomes.

References:

[1] Not applicable (no relevant information found)

[2] J Li · 2022 · Cited by 8

[3] Not applicable (no relevant information found)

[4] M Keskin · 2024

[5] Not applicable (no relevant information found)

[6] R de Lima Pedro · 2017 · Cited by 2

[7] Not applicable (no relevant information found)

[8] DM Mamian Tamayo · 2022

[9] A Negi · 2020 · Cited by 12

Diagnostic Tests for Solitary Median Maxillary Central Incisor (SMMCI)

Solitary median maxillary central incisor syndrome (SMMCI) is a rare dental anomaly that can be diagnosed through various diagnostic tests. Here are some of the common diagnostic tests used to diagnose SMMCI:

• Genetic Testing: Genetic testing can help identify genetic mutations associated with SMMCI. This test can be performed on a blood sample or a cheek swab [1, 5].
• Ultrasound: Prenatal ultrasound examination between 16-22 weeks of gestation can predict SMMCI [4, 9]. After birth, ultrasound can also be used to confirm the diagnosis.
• Magnetic Resonance Imaging (MRI): MRI can be used to diagnose SMMCI prenatally or postnatally. It can help identify midline defects and other associated anomalies [2].
• Clinical Evaluation: A thorough clinical evaluation by a pediatrician, dentist, or geneticist is essential for diagnosing SMMCI. This includes examining the child's dental and facial features, as well as any associated symptoms [3, 8].

Early Diagnosis

Early diagnosis of SMMCI is crucial to identify any associated developmental anomalies. Prenatal diagnosis can be made through ultrasound examination between 16-22 weeks of gestation [4]. Postnatally, diagnosis should be made by eight months of age, but it can also be confirmed at birth and even prenatally at 18-22 weeks [10].

References

[1] Johnson N. Prenatal diagnosis of solitary median maxillary central incisor syndrome by magnetic resonance imaging. (Context result 2) [3] Mustafa MM. A solitary median maxillary central incisor (SMMCI) is a rare malformation associated with defects of midline structures, including the craniofacial bones, ... (Context result 8) [4] Hall RK. Routine prenatal obstetric sonographic examination (from 16–22 weeks) of the head, face, nose, eyes and anterior palate can predict SMMCI. (Context result 4) [5] Clinical resource with information about Solitary median maxillary central incisor syndrome and its clinical features, SHH, available genetic tests from US and labs around the world... (Context result 15)

Based on the provided context, it appears that Solitary Median Maxillary Central Incisor (SMMCI) syndrome is a rare disorder with multiple developmental defects involving midline structures of the head and body.

Regarding drug treatment for SMMCI, there are some mentions in the search results:

• Short stature may require growth hormone therapy [3].
• A short height could necessitate growth hormone treatment [9].

However, it's essential to note that these statements are not directly related to the dental aspect of SMMCI. The primary concern with SMMCI is usually the aesthetic and developmental issues associated with the solitary maxillary central incisor tooth.

There is no specific information in the provided context about drug treatments specifically targeting the dental or midline structural defects associated with SMMCI syndrome.

However, it's worth mentioning that successful dental management of SMMCI patients is possible, following a detailed long-lasting treatment plan requiring multidisciplinary pediatric care [4].

Differential Diagnosis of Solitary Median Maxillary Central Incisor (SMMCI)

The differential diagnosis of SMMCI involves identifying other possible causes for the presence of a single maxillary central incisor. According to various sources [4, 7, 8, 9], the following conditions can be considered as differential diagnoses:

• Premature tooth loss due to trauma: This is one of the four possible differential diagnoses for SMMCI, where the single maxillary central incisor is a result of premature tooth loss due to trauma or injury [15].
• Hypodontia: Hypodontia refers to the congenital absence of one or more teeth. In some cases, hypodontia can be considered as a differential diagnosis for SMMCI, although it is not a direct cause [7, 8].
• Mesiodens: A mesiodens is a supernumerary tooth that develops in the midline of the maxilla. It can sometimes be mistaken for a single maxillary central incisor and should be considered as a differential diagnosis [4, 15].
• SMMCI syndrome: SMMCI syndrome is a rare condition where the presence of a single maxillary central incisor is associated with other developmental defects involving midline structures of the head and body [13].

It's essential to note that these conditions should be ruled out through proper diagnosis and evaluation by a dental professional. A thorough examination, medical history, and diagnostic tests can help determine the correct cause of the solitary median maxillary central incisor.

References:

[4] - SMMCI is a rare dental anomaly. It is usually considered as a minor manifestation (variant) of holoprosencephaly (context 4). [7] - Differential diganosis includes hypodontia due to either traumatic dental injury or fusion of two central incisors or a mesiodens developing to (context 7). [8] - [9,[12] [13] [14] Differential diganosis includes hypodontia due to either traumatic dental injury or fusion of two central incisors or a (context 8). [15] - The presence of a symmetrical single maxillary central incisor can be attributed to four differential diagnoses, namely premature tooth loss due to trauma, hypodontia, mesiodens, and SMMCI.