Submucous Cleft Palate (SMCP)

Overview

Cleft palate is a congenital anomaly that affects the roof of the mouth, occurring when the tissues that form the palate do not fuse properly during fetal development. Cleft palate can occur in isolation or as part of a broader syndrome. Submucous cleft palate (SMCP) is a subtype of cleft palate, characterized by a defect in the musculature of the soft palate, despite an intact mucosal surface.

What is Submucous Cleft Palate?

Submucous cleft palate is a congenital anomaly where there is a defect in the muscles of the soft palate, but the overlying mucosa is intact. This can lead to velopharyngeal insufficiency (VPI), affecting speech and feeding.

Epidemiology

The prevalence of submucous cleft palate is estimated to be around 1 in 1,200 to 1 in 2,000 live births. However, the true prevalence may be higher due to underdiagnosis.

Embryology, Pathology, and Pathophysiology

During embryonic development, the palate forms from the fusion of the medial nasal prominence and the maxillary prominence. In submucous cleft palate, the muscles of the soft palate (tensor veli palatini, levator veli palatini, and musculus uvulae) fail to fuse properly, leading to a deficiency in the velopharyngeal sphincter.

Degrees or Severity and Classification of Submucous Cleft

Submucous cleft palate can be classified based on the severity of the muscular defect and the presence of symptoms. The Calnan criteria are often used to diagnose SMCP, which include:

Clinical Association

Association with Maternal Age at Conception

Advanced maternal age has been associated with an increased risk of cleft palate, including submucous cleft palate.

Association with Down's Syndrome, Pierre Robin's, and Treacher Collins Syndrome

Submucous cleft palate is more common in individuals with certain genetic syndromes, including:

1. Down's syndrome

Individuals with Down's syndrome are at higher risk of having a submucous cleft palate.
2. Pierre Robin sequence

This condition is characterized by micrognathia, glossoptosis, and often, a cleft palate or submucous cleft palate.
3. Treacher Collins syndrome

This genetic disorder affects the development of bones and other tissues of the face, and may include cleft palate or submucous cleft palate.

Symptoms and signs

Symptoms of submucous cleft palate may include:

1. Speech difficulties

Hypernasal speech, nasal emission, or articulation errors.
2. Feeding difficulties

Infants may experience feeding problems, such as nasal regurgitation.
3. Recurrent ear infections

Due to Eustachian tube dysfunction.

Diagnosis

Features at Physical Examination

The physical examination may reveal:

Investigations to Confirm Diagnosis

Diagnosis can be confirmed through:

1. Nasopharyngoscopy

Visualizing the velopharyngeal sphincter during speech.
2. Videofluoroscopy

Assessing velopharyngeal function during speech.
3. Imaging studies

Such as MRI or CT scans, may be used in some cases.

Complications of Submucous Cleft

Untreated submucous cleft palate can lead to:

1. Speech difficulties: Persistent speech problems.
2. Hearing problems: Recurrent ear infections or hearing loss.
3. Feeding difficulties: Persistent feeding problems.

Management of Submucous Cleft

Management typically involves a multidisciplinary team, including:

1. Speech therapy

To address speech difficulties.
2. Surgical intervention

To repair the muscular defect and improve velopharyngeal function.

Age for Surgical Intervention

The timing of surgical intervention varies depending on the severity of symptoms and the child's overall health. Some surgeons recommend repair between 6 months to 2 years of age.

Submucous cleft palate is a congenital anomaly that requires early diagnosis and management to prevent long-term complications. A multidisciplinary team approach is essential for optimal outcomes.

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