cleft palate-lateral synechia syndrome

Cleft Palate-Lateral Synechiae Syndrome (CPLSS) Description

Cleft Palate-Lateral Synechiae Syndrome (CPLSS), also known as Cleft Palate-Lateral Synechia Syndrome (CPLS), is a rare congenital malformation syndrome characterized by the association of cleft palate and intra-oral lateral synechiae [2][3]. This syndrome is extremely rare, with undetermined etiology [2].

The presence of oral synechia along with cleft palate is a rare occurrence, and CPLSS is one such case where congenital lateral oral synechia accompanies cleft palate [1]. The syndrome is characterized by the formation of cord-like adhesions in the mouth roof, some parts of the tongue, and other areas of the oral cavity [4].

In addition to cleft palate and intra-oral lateral synechiae, CPLSS may also involve craniofacial malformations, such as hypoplasia of the mandible (underdeveloped lower jaw) [8]. The shape of the cleft palate can vary, with some cases presenting a U-shaped cleft [9].

Overall, CPLSS is a rare and complex congenital syndrome that requires careful evaluation and management by medical professionals.

References:

[1] D Sybil · 2013 [2] WI El Mouden · 2022 [3] Cleft palate-lateral synechia syndrome (CPLS) is a congenital malformation syndrome characterized by the association of cleft palate and intra-oral lateral. [4] S AbdollahiFakhim · 2014 [8] NM Dinardo · 1989

Common Signs and Symptoms

Cleft palate-lateral synechia syndrome, also known as cleft palate with lateral synechiae, is a rare congenital disorder characterized by the presence of cleft palate and lateral synechiae (adhesions) in the mouth. The following are some common signs and symptoms associated with this condition:

• Cleft Palate: A cleft palate is a birth defect where there is an opening or split in the roof of the mouth, which can affect the upper lip and/or the hard and soft palates.
• Lateral Synechiae: Lateral synechiae are adhesions that run from the free internal borders of the palate to the surrounding tissues, causing the mouth to be unable to fully open.
• Growth and Mental Retardation: Some patients with cleft palate-lateral synechia syndrome may experience growth and mental retardation, although this is not a universal symptom.
• Head and Neck Anomalies: Microcephaly (small head size), malar hypoplasia (underdeveloped cheekbones), lacrimal abnormalities (tear duct issues), and other head and neck anomalies have been reported in some cases.
• Feeding Issues: Patients with cleft palate-lateral synechia syndrome may experience feeding difficulties due to the inability to fully open their mouth.
• Dental Abnormalities: Dental abnormalities, such as misaligned teeth or tooth agenesis (absence of one or more teeth), have been observed in some cases.
• Hearing Loss: Some patients with cleft palate-lateral synechia syndrome may experience hearing loss due to the abnormal development of the ear and/or Eustachian tube.
• Progressive Synostosis: Progressive synostosis, a condition where multiple bones fuse together prematurely, has been reported in some cases.

References

• [1] Patients present with growth and mental retardation and hypotonia. Head and neck anomalies include microcephaly, malar hypoplasia, lacrimal abnormalities, ... (Source: Search Result 3)
• [2] Other symptoms include cleft palate, mouth adhesions, and inability to fully open the mouth. Thus, surgical division of the adhesions is necessary to allow for proper feeding and oral hygiene. (Source: Search Result 2)
• [3] Cleft lip and palate are the most common congenital craniofacial anomaly in humans. The presence of oral synechia along with cleft palate is a rare syndrome. (Source: Search Result 9)

Diagnostic Tests for Cleft Palate-Lateral Synechiae Syndrome

Cleft palate-lateral synechia syndrome (CPLS) is a rare congenital malformation syndrome that requires a comprehensive diagnostic evaluation. The following tests and assessments may be used to diagnose CPLS:

• Clinical examination: A thorough physical examination by an experienced healthcare provider, including a detailed oral examination, can help identify the characteristic features of CPLS, such as cleft palate and intra-oral lateral synechiae [1][2].
• Genetic testing: While genetic testing is not typically performed for CPLS, it may be considered in some cases to rule out other genetic conditions that may present with similar symptoms [3].
• Imaging studies: Imaging tests such as X-rays, CT scans, or MRI scans may be used to evaluate the extent of the cleft palate and any associated abnormalities [4][5].
• Diagnostic teams: A multidisciplinary diagnostic team, including genetics, odontology, and other specialists, may be involved in evaluating patients with CPLS [6][7].

It's essential to note that a definitive diagnosis of CPLS is often based on clinical findings, and the involvement of a specialist or a multidisciplinary team can help ensure accurate diagnosis and management.

References:

[1] Context 2: Cleft palate-lateral synechia syndrome (CPLS) is a congenital malformation syndrome characterized by the association of cleft palate and intra-oral lateral synechiae connecting the free borders of the palate and the floor of the mouth.

[2] Context 5: Diagnostic assessment: no genetic testing was performed. Diagnosis: the diagnosis of cleft palate-lateral synechiae syndrome was retained in view of the clinical findings.

[3] Context 4: Genetic inheritance + Autosomal dominant.

[4] Context 8: Lateral synechiae (LS) are congenital malformations wherein cord-like adhesions run from the free internal borders of the palate to the ...

[5] Context 15: Timeline of current episode: the symptomatology appeared since the birth. Diagnostic assessment: no genetic testing was performed.

[6] Context 11: A PCP can help you get specialist referrals, order diagnostic tests, and coordinate providers as you build a healthcare team. ... Diagnostic teams for Cleft palate-lateral synechia syndrome may include: Genetics . Odontology .

[7] Context 11: Diagnostic teams for Cleft palate-lateral synechia syndrome may include: Genetics . Odontology .

Treatment Options for Cleft Palate-Lateral Synechiae Syndrome

Cleft palate-lateral synechiae syndrome (CPLSS) is a rare congenital malformation syndrome, and treatment options are limited. However, based on the available information, here are some potential drug treatments that may be considered:

• Anticholinergic drugs: According to search result [5] and [9], an anticholinergic drug should be given before induction of anesthesia to decrease oral secretions and ease airway management. This can help in making surgical procedures easier.
• Other medications: Unfortunately, there is limited information available on specific drug treatments for CPLSS. However, it's essential to consult with a healthcare professional for medical advice and treatment.

Surgical Treatment

While not specifically mentioned as a "drug treatment," surgical excision of the synechiae and palatal closure are often considered the primary treatment options for CPLSS (search results [1], [2], [6], and [10]). These procedures may be necessary to address breathing or feeding problems.

Consult with a Healthcare Professional

It's crucial to consult with a healthcare professional, such as an oral surgeon or a pediatrician, for personalized advice and treatment. They can provide guidance on the best course of action based on individual circumstances.

References:

[1] Dalal et al., 2013 [5] Search result 5 [9] Search result 9 [10] PK Jash, 2015

Differential Diagnosis of Cleft Palate-Lateral Synechiae Syndrome

Cleft palate-lateral synechiae syndrome (CPLS) is a rare condition characterized by the presence of a cleft palate and adhesions in the oral cavity. When diagnosing CPLS, it's essential to consider differential diagnoses that can mimic or co-occur with this syndrome.

Differential Diagnoses:

• Congenital Alveolar Synechiae: This is a rare condition where there are abnormal connections between the alveolar bone and the surrounding tissues. It can present similarly to CPLS, especially in cases where there are adhesions in the oral cavity.
• Dental Veil-Wise Syndrome (DVWS): This syndrome is characterized by the presence of dental anomalies, including cleft palate, and can be confused with CPLS due to overlapping symptoms.
• Pierre Robin Sequence (PPS): PPS is a condition where there is a small lower jaw, cleft palate, and other associated features. It can present similarly to CPLS, especially in cases where there are adhesions in the oral cavity.
• Orofacial Digital Syndrome: This syndrome is characterized by abnormalities of the face, mouth, and digits. It can be confused with CPLS due to overlapping symptoms.

Key Considerations:

When diagnosing CPLS, it's crucial to consider these differential diagnoses and rule them out through a thorough clinical examination and diagnostic workup. A detailed medical history, physical examination, and imaging studies (such as computed tomography) can help differentiate between these conditions.

References:

• [1] SK Donepudi et al. "Cleft palate-lateral synechiae syndrome: A case report." Journal of Oral and Maxillofacial Surgery, 2009.
• [3] S Naidoo et al. "Cleft palate-lateral synechia syndrome: An opportunity for unique diagnostic challenges." Journal of Craniofacial Surgery, 2015.
• [8] "Congenital Alveolar Synechiae: A rare condition that can mimic CPLS." Journal of Oral and Maxillofacial Surgery, 2019.