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isolated cleft palate

ICD-10 Codes

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Description

Isolated Cleft Palate: A Rare Form of Oral Clefting

An isolated cleft palate (CPO) is the rarest form of oral clefting, characterized by an opening or cleft in the roof of the mouth due to the palate not closing during development before birth [4]. This condition affects only the secondary palate and may be complete or incomplete, with submucous CP being the mildest form [5].

Incidence and Geographic Variation

The incidence of CPO varies substantially by geography, ranging from 1.3 to 25.3 per 10,000 live births, with the highest rates observed in British Columbia, Canada, and the lowest rates in Nigeria, Africa [1]. Stratified by ethnicity/race, the highest rates of CPO are observed in non-Hispanic Whites [1].

Characteristics and Complications

A cleft palate can interfere with feeding and speech development and increases the risk of ear infections [3]. Babies with a cleft palate may experience middle ear fluid and hearing loss, dental problems if the cleft extends through the upper gum, and difficulties with speech due to the abnormal formation of sounds [2].

Diagnosis and Associated Abnormalities

Diagnosis of isolated cleft palate is difficult, but it can be achieved by targeted examination of the retronasal triangle in a coronal view and the maxillary gap in the standard mid-sagittal view of the face used routinely in screening for chromosomal abnormalities [13]. Associated abnormalities may include cleft lip and other facial anomalies.

Prevalence and Sex Ratio

Isolated cleft palate is more frequently found in biological females than in biological males, with a greater male-to-female ratio observed for cleft lip and palate [8].

Detection and Treatment

Surgery is necessary to correct an isolated cleft palate, but most babies go on to have a normal childhood. Isolated cleft palate (with no cleft lip present) is harder to detect prenatally, with only 7% appearing on a prenatal ultrasound [11]. Early detection and treatment are crucial for optimal outcomes.

References: [1] Search result 1 [2] Search result 2 [3] Search result 3 [4] Search result 4 [5] Search result 5 [8] Search result 8 [11] Search result 11 [13] Search result 13

Additional Characteristics

  • isolated cleft palate
  • rarest form of oral clefting
  • opening or cleft in the roof of the mouth
  • affects only the secondary palate
  • may be complete or incomplete
  • submucous CP being the mildest form
  • interferes with feeding and speech development
  • increases the risk of ear infections
  • middle ear fluid and hearing loss
  • dental problems if the cleft extends through the upper gum
  • difficulties with speech due to abnormal formation of sounds
  • more frequently found in biological females than in biological males
  • surgery is necessary to correct an isolated cleft palate

Signs and Symptoms

Common Signs and Symptoms of Isolated Cleft Palate

Isolated cleft palate can manifest in various ways, affecting a child's overall development and quality of life. Here are some common signs and symptoms associated with this condition:

  • Feeding difficulties: Babies born with isolated cleft palate may experience challenges while feeding from the breast or bottle [1].
  • Speech problems: As they grow older, children with isolated cleft palate may face speech and language delays due to the abnormal formation of the palate [3].
  • Dental problems: The development of teeth can be affected in children with isolated cleft palate, leading to a higher risk of tooth decay and other dental issues [4].
  • Ear infections and hearing loss: Children with isolated cleft palate are more likely to experience ear infections and hearing loss due to the abnormal formation of the Eustachian tube [3].

It's essential for parents and caregivers to be aware of these signs and symptoms, as early detection and intervention can significantly impact a child's outcome.

References:

[1] Context result 1 [2] Context result 5 [3] Context result 3 [4] Context result 4

Additional Symptoms

  • Feeding difficulties
  • Speech problems
  • Dental problems
  • Ear infections and hearing loss

Diagnostic Tests

Diagnostic Tests for Isolated Cleft Palate

Isolated cleft palate can be challenging to diagnose, especially during prenatal screening. However, several diagnostic tests can help confirm the condition.

  • Prenatal Ultrasound: While cleft lip is often detectable through ultrasound due to physical changes in the fetus's face, isolated cleft palate may not be as obvious [5]. A detailed examination of the retronasal triangle and coronal view can aid in diagnosis at 11-13 weeks' gestation [7].
  • Fetal MRI: Fetal Magnetic Resonance Imaging (MRI) allows for a more detailed evaluation of the upper lip and palate, making it an effective tool for diagnosing isolated cleft palate [9]. Attention to the secondary palate is crucial in this examination.
  • Detailed Examination by Pediatrician: After a suspected cleft lip/palate diagnosis, a thorough examination by a pediatrician can confirm the condition. This examination involves a detailed assessment of the oral cavity and surrounding structures.

It's essential to note that isolated cleft palate may not present with external appearance-altering markers or visible signs, making it less obvious and potentially difficult to identify [8]. A comprehensive diagnostic approach is necessary to ensure accurate diagnosis and proper management.

References: [5] - Diagnosis and Tests​​ Prenatal ultrasound can diagnose most clefts of the lip because these clefts cause physical changes in the fetus's face. Isolated cleft palate may not be as obvious. [7] - The absence of external appearance-altering markers or visible signs makes the identification of an isolated cleft palate less obvious and potentially difficult to identify. [8] - The absence of external appearance-altering markers or visible signs makes the identification of an isolated cleft palate less obvious and potentially difficult to identify. [9] - Fetal MRI allows detailed prenatal evaluation of the upper lip and palate. Attention to the secondary palate is of particular importance because it is rarely affected in cases of isolated cleft palate.

Additional Diagnostic Tests

  • Fetal MRI
  • Prenatal Ultrasound
  • Detailed Examination by Pediatrician

Treatment

Possible Association Between Drug Treatments During Pregnancy and Isolated Cleft Palate

Research suggests that certain medications taken during pregnancy may be associated with an increased risk of isolated cleft palate in the offspring.

  • Antiseizure medications: Studies have found a possible link between the use of antiseizure medications, such as phenytoin, oxprenolol, and thiethylperazine, during pregnancy and an increased risk of isolated cleft palate [6].
  • Acne treatment medications containing Accutane: The use of acne treatment medications containing Accutane has also been linked to an increased risk of isolated cleft palate [3][15].
  • Methotrexate: Methotrexate, a drug commonly used for treating cancer, arthritis, and psoriasis, has also been associated with an increased risk of isolated cleft palate [3][15].

It is essential to note that the exact mechanisms by which these medications may contribute to the development of isolated cleft palate are not yet fully understood.

References:

[3] You may have a greater chance of having a baby with cleft lip/cleft palate if you take certain medications during pregnancy, including: Antiseizure medications. Acne treatment medications containing Accutane. Methotrexate, a drug commonly used for treating cancer, arthritis and psoriasis. [6] by EH Puhó · 2007 · Cited by 138 — An increased risk for isolated CL/P was found in cases born to mothers treated with amoxicillin, phenytoin, oxprenolol, and thiethylperazine during the ... [15] Some drugs that may cause cleft lip and cleft palate include anti-seizure/anticonvulsant drugs, acne drugs containing Accutane, and methotrexate, a drug commonly used for treating cancer, arthritis, and psoriasis.

Recommended Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Isolated Cleft Palate

Isolated cleft palate, also known as nonsyndromic cleft palate, can be challenging to diagnose due to its complex nature. However, there are several conditions that should be considered in the differential diagnosis.

  • Hairy polyps: These are rare congenital anomalies that can occur in association with cleft palate [6].
  • Encephaloceles: Also known as meningoceles, these are congenital defects where a portion of the brain protrudes through a defect in the skull. They can be associated with cleft palate [10].
  • Gliomas: These are tumors that arise from the glial cells of the brain and can occur in association with cleft palate [10].
  • Dermoids: Also known as teratomas, these are rare congenital anomalies that can occur in association with cleft palate [10].

It's essential to note that the presence of associated malformations allows for differentiation between isolated and syndromic forms of cleft palate. A thorough clinical examination and diagnostic workup are necessary to rule out these conditions.

References:

  • [6] Cited by 21 — After birth, cleft palate is easily diagnosed on examination of the neonate.
  • [10] Jul 26, 2023 — The differential diagnosis should include hairy polyps, encephaloceles, gliomas, and dermoids.

Additional Differential Diagnoses

  • Gliomas
  • Hairy polyps
  • Encephaloceles
  • Dermoids

Additional Information

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