Unilateral and bilateral cleft lip, alveolar process, hard and soft palate: clinical guidelines

Applies tochildren2026 editionQ35, Q36, Q37

Clinical Guidelines of the Russian Ministry of Health, Pediatrics. 2026 Edition, ICD-10 codes: Q35, Q36, Q37.

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Brief for Practice

WhiteCoat Pro editorial summary of the guideline text, 2026 edition. Each point links to the original section — verify with it before prescribing.

Cleft lip, alveolar process, and/or palate is a congenital maxillofacial malformation that impairs feeding, speech, hearing, and nasal breathing. The guideline is intended for the management of children from birth to 18 years and outlines staged diagnostics, surgical treatment, and long-term multidisciplinary rehabilitation.

Key points

The malformation has a multifactorial origin: genetic predisposition or a de novo mutation combined with adverse environmental factors, including teratogenic drugs, alcohol, drugs, infections, and nutritional deficiencies, play a role. → in text
Due to the clefting of the nasal and oral cavities, feeding is impaired; chewing and occlusion problems may occur later. Speech, hearing, and nasal breathing are also evaluated. → in text
Management should be staged and multidisciplinary: a maxillofacial surgeon, orthodontist, pediatrician, otolaryngologist, geneticist, medical speech-language pathologist, and psychologist participate in diagnostics, treatment, and rehabilitation. → in text
Dispanserization (clinical follow-up) is carried out from birth up to 18 years of age inclusive (C-level recommendation, evidence level 5). → in text
In the first 0–3 months, special palatal plates are used to facilitate feeding, separate the nasal and oral cavities, and correct jaw deformity prior to surgery (C-level recommendation, evidence level 4). → in text
Surgical treatment is planned taking into account the type of cleft, hypoplasia, and deformity of surrounding structures; it is important to follow a staged approach and consider the child's somatic status (C-level recommendation, evidence level 5). → in text
Care is provided in a hospital setting, on an elective basis, following a staged and periodic principle; the specified type of care is high-tech medical care, including coverage under the Mandatory Medical Insurance (OMС) framework. → in text

Diagnostics

Clarify the hereditary history of maxillofacial pathology and potential exposure to teratogens in the 1st trimester; identify chewing, speech, breathing, and hearing impairments, as well as aesthetic defects (C-level recommendation, evidence level 5). → in text
Establish a clinical diagnosis based on visual examination, anthropometric, radiological, and ultrasound methods. → in text
Perform examinations by a pediatrician, maxillofacial surgeon, otolaryngologist, orthodontist, and geneticist: assess somatic status, dental occlusion, ENT organs, concomitant and potential genetic pathology, and determine the treatment plan (C-level recommendation, evidence level 5). → in text
Order laboratory tests according to the geneticist's indications and the preoperative panel for elective intervention prescribed by the pediatrician. → in text
During pregnancy, perform ultrasound screening at 19–21 weeks for the primary detection of maxillofacial malformations and assessment of the anatomical and physiological features of the fetus (C-level recommendation, evidence level 4). → in text
In children, perform pharyngoscopy with a speech-language pathologist's report in case of dysarthria; rhinoscopy with an ENT consultation is advisable starting from 7 years of age. CT of the maxillofacial region is indicated at 6–11 years to assess the defect, position of maxillary fragments, and plan the bone graft size, and at 12–18 years to assess jaw relationships and indications for orthognathic surgery (C-level recommendation, evidence levels 4–5). → in text

Treatment

Pre-surgical orthodontic preparation is performed prior to surgery, and speech therapy correction is initiated before surgery and continued after uranoplasty (C-level recommendation, evidence level 5). → in text
Special feeding devices are used for newborns. Tube feeding for an isolated malformation is not recommended; it is permissible under strict indications—severe concomitant somatic pathology, prematurity, or immaturity. Daily body weight monitoring is required in the first weeks (C-level recommendation, evidence level 5). → in text
Primary cheilorhinoplasty is performed after 3–6 months of age, within the first year of life. In case of concomitant congenital malformations, birth trauma, postnatal encephalopathy, anemia, or malnutrition, the surgery is postponed until remission (C-level recommendation, evidence level 5). → in text
For secondary cleft palate, tension-free uranoplasty is performed at 1–2 years of age. For soft and hard cleft palate, a two-stage approach is used: soft palate repair at 1–2 years, followed by tension-free uranoplasty after 3–4 years (C-level recommendation, evidence level 5). → in text
Alveolar bone grafting is performed after orthodontic preparation at 8–10 years of age (C-level recommendation, evidence level 5). → in text
Velopharyngeal insufficiency is addressed after consultations with a medical speech-language pathologist and an ENT specialist; correction is advisable starting from 6 years of age (C-level recommendation, evidence level 5). → in text
For secretory otitis media, monitoring and treatment by an ENT specialist are performed, with tympanotomy if necessary to preserve hearing function (C-level recommendation, evidence level 5). → in text
Residual deformities are corrected by revisional cheilorhinoplasty, cheilorhinoseptoplasty, and secondary uranoplasty. Full correction of the skin-cartilaginous part of the nose is performed at 14–16 years, correction of nasal pyramid deformity is postponed until 17 years; orthognathic surgeries and dental implantation are performed after 18 years (C-level recommendation, evidence level 5). → in text

When to refer and how to monitor

For elective surgical treatment, the patient is hospitalized; hospitalization is carried out in stages and periodically in accordance with the treatment algorithm. → in text
From birth, the child should be included in clinical follow-up (dispanserization) up to 18 years of age inclusive (C-level recommendation, evidence level 5). → in text
Referral to a maxillofacial surgeon is required to establish a diagnosis and plan treatment, to an orthodontist to assess occlusion and provide pre- and post-surgical preparation, and to an ENT specialist to assess ENT organs. → in text
In case of speech impairment, the child is referred for pharyngoscopy and subsequent consultation with a medical speech-language pathologist to develop an individualized speech rehabilitation program. → in text

Frequently asked questions

How is the diagnosis of cleft lip and palate confirmed?

The diagnosis is clinical: it is established based on visual examination, anthropometric, radiological, and ultrasound methods. → in text

When should primary cheilorhinoplasty be performed?

Typically, the surgery is performed after 3–6 months of age, within the first year of life. In case of congenital malformations, birth trauma, postnatal encephalopathy, anemia, or malnutrition, the intervention is postponed until remission. → in text

What is the timeline for palate repair?

For secondary cleft palate, tension-free uranoplasty is performed at 1–2 years of age. For soft and hard cleft palate, soft palate repair is performed first at 1–2 years, followed by tension-free uranoplasty after 3–4 years. → in text

When is alveolar bone grafting needed?

It is performed after orthodontic preparation at the age of 8–10 years. → in text

How to feed a newborn with a cleft?

Special feeding devices are used, and parents are trained in feeding expressed breast milk or formula while preventing aspiration. A feeding tube is used only under strict indications: severe somatic pathology, prematurity, or immaturity; body weight is monitored daily during the first weeks. → in text

Status: Applied · approved by the Scientific and Practical Council of the Russian Ministry of Health
Database ID: 1050, edition 1 (ID 1050_1)
Publication date: June 17, 2026
Age category: children
ICD-10 codes: Q35 Q36 Q37
Specialties: Dentistry and Maxillofacial Surgery Pediatrics
Developers: Association of Maxillofacial Surgery Specialists LLC
Russian Society of Plastic, Reconstructive and Aesthetic Surgeons All-Russian Public Organization
Official text: Ministry of Health of Russia Rubricator — 1050_1

List of Abbreviations

CDBL — congenital bilateral cleft lip

CDBLA — congenital bilateral cleft lip and alveolar process

CDBLAP — congenital bilateral cleft lip, alveolar process, and palate

HTMC — high-tech medical care

CDUL — congenital unilateral cleft lip

CDULA — congenital unilateral cleft lip and alveolar process

CDULAP — congenital unilateral cleft lip, alveolar process, and palate

CDP — congenital cleft palate

WHO — World Health Organization

CT — computed tomography

ENT — otolaryngology

ICD-10 — International Classification of Diseases

MSCT — multislice computed tomography

CMI — compulsory medical insurance

OPTG — orthopantomography

Ultrasound — ultrasound examination

MFR — maxillofacial region

Terms and Definitions

Congenital malformation is a set of deviations from the normal structure of the organism that occur during intrauterine development under the influence of various internal and external factors.

Cleft lip, alveolar process, and palate is a congenital malformation characterized by a defect in the midfacial region: splitting of the lip into two or three fragments, deformation of the cartilaginous part of the nose, abnormal insertion and dysfunction of the orbicularis oris muscle bundles, and a defect of the alveolar process of the maxilla. In cleft palate, there is a communication between the oral and nasal cavities through a tissue defect along the midline of the palate, abnormal positioning and insertion of the soft palate muscles, widening of the middle pharynx, and shortening of the soft palate.

Ouranoplasty is a reconstructive surgery performed for cleft hard palate.

Soft palate repair is a reconstructive surgery performed for cleft soft palate.

Cheilorhinoplasty is a reconstructive surgery performed for cleft lip and nasal cartilage deformity.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Cleft lip and/or palate is a severe congenital malformation of the maxillofacial region, manifesting as a disruption of the continuity of the upper lip, alveolar process, and palate, accompanied by significant functional impairments. The difficulties in restoring impaired vital functions of feeding, respiration, and speech, as well as the anatomical reconstruction of the upper lip, nose, and maxilla in a growing organism, are the cause of long-term disability in children with cleft lip and palate [1-4].

In this facial malformation, morphological features are detected as early as the antenatal period [5], and after birth, the following vital functions are impaired:

1) feeding dysfunction: normal suckling during the neonatal period and infancy is impossible in most cases due to the lack of separation between the nasal and oral cavities, while subsequent chewing impairment is associated with dental arch anomalies and malocclusion;

2) speech: the absence of integrity of the velopharyngeal sphincter impedes the formation of correct speech (speech is slurred, quiet, with a nasal tone) and may cause delayed speech development;

3) hearing: splitting and improper insertion points of the palatal musculature cause Eustachian tube dysfunction;

4) nasal breathing: deformation of the terminal and osteocartilaginous parts of the nose leads to changes in airflow, disrupts adequate respiration, and contributes to the development of various comorbid ENT pathologies [6-9].

The cosmetic defect, presenting as an abnormal upper lip anatomy and nasal deformity present at birth, elicits a negative reaction from parents and subsequently from people around them. The harmonious development of a child with a cleft is hindered due to this cosmetic defect and functional impairments [10, 11].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

This malformation has a multifactorial origin. Multifactorially inherited clefts of the lip and palate are characterized by features common to all multifactorial diseases. The emergence of such forms requires either a genetic predisposition or the appearance of a new mutation, as well as the exposure to certain adverse environmental factors (Table 1) [12, 13, 15-18].

Table 1. Etiological factors influencing the development of cleft lip and palate.

Factors	Description
1. Endogenous factors	- gene mutations - biological inferiority of germ cells - parental age
2. Exogenous factors	- physical: mechanical impact on the fetus, uterine malformations, amniotic bands, multiple pregnancy, increased radiation - chemical: inadequate nutrition, hypo- or hypervitaminosis, hormonal dysregulation, exposure to teratogenic poisons and medications, alcohol abuse, drug use - biological: infectious diseases (influenza, rubella, herpes, toxoplasmosis), psychological (hyperadrenalinemia)

Factors

Description

1. Endogenous factors

- gene mutations

- biological inferiority of germ cells

- parental age

2. Exogenous factors

- physical: mechanical impact on the fetus, uterine malformations, amniotic bands, multiple pregnancy, increased radiation

- chemical: inadequate nutrition, hypo- or hypervitaminosis, hormonal dysregulation, exposure to teratogenic poisons and medications, alcohol abuse, drug use

- biological: infectious diseases (influenza, rubella, herpes, toxoplasmosis), psychological (hyperadrenalinemia)

1.3 Epidemiology of the Disease or Condition

Clefts of the upper lip, alveolar process, and palate rank 3rd to 5th among all congenital malformations and first (87%) among congenital facial malformations. According to WHO data (1971), they occur in 0.6–1.6 per 1,000 newborns. The prevalence in Russia is close to the global average (1:650–700 newborns). There are variations in the frequency of this pathology across different regions of our country: 0.6–1.1 per 1,000 (Moscow), 0.4–1.6 per 1,000 (Orenburg Oblast), 1.34 per 1,000 (Volgograd Oblast). The highest birth rate of children with cleft lip and palate is observed in Japan (1.7 per 1,000), while the lowest prevalence of cleft lip and palate (0.4 per 1,000) is noted among the Negroid race [19, 20].

1.4 Features of Coding the Disease or Condition

Q35 - Cleft palate

Q35.0 - Bilateral cleft hard palate

Q35.1 - Cleft hard palate

Q35.2 - Bilateral cleft soft palate

Q35.3 - Cleft soft palate

Q35.4 - Bilateral cleft hard and soft palate

Q35.5 - Cleft hard and soft palate

Q35.6 - Median cleft palate

Q35.7 - Cleft uvula

Q35.8 - Unspecified bilateral cleft palate

Q35.9 - Unspecified cleft palate

Q36 - Cleft lip

Q36.0 - Bilateral cleft lip

Q36.1 - Median cleft lip

Q36.9 - Unilateral cleft lip

Q37 - Cleft palate with cleft lip

Q37.0 - Cleft hard palate with bilateral cleft lip

Q37.1 - Cleft hard palate with unilateral cleft lip

Q37.2 - Cleft soft palate with bilateral cleft lip

Q37.3 - Cleft soft palate with unilateral cleft lip

Q37.4 - Cleft hard and soft palate with bilateral cleft lip

Q37.5 - Cleft hard and soft palate with unilateral cleft lip

Q37.8 - Unspecified cleft palate with bilateral cleft lip

Q37.9 - Unspecified cleft palate with unilateral cleft lip

1.5 Classification of the disease or condition

Clinical and anatomical classification of congenital cleft lip and palate:

1. unilateral cleft of the upper lip

1.1. latent

1.2. partial /incomplete/

1.3. complete

2. bilateral cleft of the upper lip (symmetrical, asymmetrical)

2.1. latent

2.2. partial /incomplete/

2.3. complete

3. unilateral cleft of the upper lip and alveolar process

3.1. partial /incomplete/

3.2. complete

4. bilateral cleft of the upper lip and alveolar process (symmetrical, asymmetrical)

4.1. partial /incomplete/

4.2. complete

5. unilateral complete cleft of the upper lip and palate

6. bilateral complete cleft of the upper lip and palate

7. cleft palate

7.1. submucous /hidden/

7.2. partial /incomplete/

7.3. complete

1.6 Clinical presentation of the disease or condition

A hidden cleft of the upper lip is characterized by the absence of disruption to the skin integrity of the lip. Lateral to the philtrum, there is a vertical skin depression beneath which splitting of the orbicularis oris muscle can be identified. The defect is especially noticeable during functional load.

An incomplete cleft is characterized by the presence of a split of the upper lip that does not reach the lower part of the outer nasal opening (nostril). The lip fails to fuse only in its lower parts, while at the base of the nose, there is a properly developed section of tissue ("bridge"). This pathology may be accompanied by nasal deformity.

In a complete isolated cleft of the upper lip, there is a splitting of the tissues of the upper lip throughout its entire length—from the vermilion border to the lower nasal meatus. This is always accompanied by a deformity of the skin and cartilage section of the nose. In unilateral cleft of the upper lip, flattening and stretching of the nasal ala are observed on the affected side. The tip of the nose is shifted toward the affected side, the nostril on this side is wide, and the nasal septum is curved toward the healthy side.

In a bilateral complete cleft of the upper lip, it is cleft to the right and left of the philtrum. The median part of the lip is shortened. The nasal septum is short, and due to this, the tip of the nose is flattened and pulled toward the upper lip. Both nasal alae are stretched and flattened so that the nose has a "negroid" shape.

Hidden clefts of the palate are clefts in which the congenital defect is subtle. Sometimes a depression of the mucous membrane between the two halves of the soft palate is noticeable. Non-fusion of the bony plates of the hard palate is noted. The palate is shortened. As a rule, there are no bite disorders. The child exhibits hypernasality (nasal tone of speech) and sound production (articulation) disorders. The speech of such children is sometimes accompanied by compensatory movements of the facial muscles of the face, nasal alae, and contraction of the frontal muscles.

Clefts of the soft palate can be complete and incomplete. Incomplete clefts do not reach the border with the hard palate. In a complete cleft, the defect of the soft palate reaches the posterior edge of the hard palate and is often accompanied by hidden underdevelopment of the posterior part of the hard palate or its hidden cleft. Shortening of the palate, widening of the pharyngeal ring, and underdevelopment of the soft palate muscles are noted. The occlusion is most often undisturbed.

In a complete cleft of the soft and hard palate, splitting of the palate is noted up to the incisive foramen. The base of the vomer lies freely, without connecting to the palatine plates. The nasal conchae are clearly visible. Their mucosa is usually hypertrophied. The palatine plates are underdeveloped. The soft palate is shortened. The pharyngeal ring is widened. Congenital underdevelopment of all parts of the upper jaw is possible.

In unilateral complete clefts of the upper lip and palate, clinically there is a complete cleft of the upper lip with deformity of the skin and cartilage section of the nose, followed by a cleft of the alveolar process of the maxilla (usually in the region of the second incisor), as well as a cleft of the hard palate on one side and a complete cleft of the soft palate. In addition, there is a typical deformity of the alveolar process of the maxilla due to the displacement of the small fragment toward the midline and posteriorly.

In bilateral complete clefts of the upper lip and palate, there is a complete bilateral cleft of the upper lip with typical nasal deformity, bilateral cleft of the alveolar process of the maxilla with anterior displacement of the premaxilla, a complete bilateral cleft of the hard palate, and a complete cleft of the soft palate. In this case, the base of the vomer on both sides does not fuse with the palatine processes of the maxilla.

2. Diagnostics, medical indications and contraindications for diagnostic methods

The diagnosis of cleft lip and palate is clinical and is established on the basis of visual examination, anthropometric, radiological, and ultrasound research methods.

2.1 Complaints and Anamnesis

It is recommended to take a hereditary history (maxillofacial pathology) and data on exposure to teratogens in the 1st trimester, as well as complaints from parents of children diagnosed with "unilateral and bilateral clefts of the upper lip, alveolar process, hard and soft palate" in order to identify potential hereditary factors and the presence of teratogenic exposure during pregnancy leading to the primary disease [13, 24].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

It is recommended to collect anamnesis and complaints from patients and/or parents of children diagnosed with "unilateral and bilateral clefts of the upper lip, alveolar process, hard and soft palate" regarding the presence of masticatory, speech, respiratory, hearing disorders, and aesthetic defects [13, 24].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: target age group – in all periods of postnatal development.

2.2 Physical Examination

For patients diagnosed with cleft lip and palate, it is recommended to have an appointment (examination, consultation) with a pediatrician to assess the patient's general somatic status [84, 85, 101].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: the pediatrician assesses the patient's general condition, height and weight indicators, and identifies comorbid pathology. The vast majority of surgical interventions for the described diseases are elective, as a result of which the patient must be thoroughly examined, and all comorbid pathology must be compensated.

For patients diagnosed with cleft lip and palate, it is recommended to have an appointment (examination, consultation) with a maxillofacial surgeon for diagnosis and determination of the treatment plan [84, 101].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: the maxillofacial surgeon makes the diagnosis, determines the treatment plan, the need for additional diagnostic methods and consultations with related specialists, and provides treatment.

For patients diagnosed with cleft lip and palate, it is recommended to have an appointment (examination, consultation) with an otorhinolaryngologist to assess the condition of ENT organs [84, 86, 87, 101].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: the otorhinolaryngologist assesses the condition and prevents the development of complications from ENT organs.

Comments: the degree of anesthetic risk is assessed prior to any surgical intervention.

For patients diagnosed with cleft lip and palate, it is recommended to have an appointment (examination, consultation) with an orthodontist to assess the state of occlusion [86].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: congenital clefts of the upper lip, alveolar process, hard and soft palate are accompanied by malposition of the jaws and malocclusion. Surgical treatment of such patients is always accompanied by an orthodontic phase, during which the orthodontist assesses the jaw position and performs orthodontic correction both before and after surgery.

For patients diagnosed with cleft lip and palate, it is recommended to have an appointment (examination, consultation) with a geneticist to identify genetic pathology [84, 101].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comments: performed to identify the etiological cause of the disease, possible concomitant syndromes, and to plan future pregnancies.

2.3 Laboratory Diagnostics

The complex of laboratory diagnostics includes tests prescribed by a geneticist, as well as a preoperative set of tests for elective surgery prescribed by a pediatrician. No other specific laboratory diagnostics are provided.

2.4 Instrumental Diagnostics

It is recommended that pregnant women undergo an ultrasound screening examination at 19–21 weeks of gestation to assess prenatal fetal development in order to identify chromosomal abnormalities, malformations, risks of fetal growth restriction, premature birth, and preeclampsia (screening II) for the primary detection of maxillofacial developmental pathology in the child, in particular, unilateral and bilateral clefts of the upper lip, alveolar process, hard and soft palate, with the aim of a detailed study of the anatomical and physiological features of the face, brain, heart, and skeleton [88, 97].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

It is recommended that children diagnosed with "congenital unilateral and bilateral cleft of the upper lip, alveolar process, hard and soft palate" undergo pharyngoscopy (performed by a maxillofacial surgeon, otorhinolaryngologist) with consultation and subsequent conclusion by a medical speech therapist for medico-speech therapy research in dysarthria and the development of an individual speech therapy rehabilitation program [52].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

For patients diagnosed with cleft lip and palate, it is recommended to undergo follow-up and treatment by an otorhinolaryngologist, including tympanostomy when necessary, for the prevention and treatment of secretory otitis media and the preservation of auditory function [64].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

For patients diagnosed with cleft lip and palate, it is recommended to undergo rhinoscopy followed by an appointment (examination, consultation) with an otorhinolaryngologist to detect nasal septum deviation and the presence of communication between the oral and nasal cavities [98, 99, 100].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

Comment: it is advisable to perform rhinoscopy in school-age children (7 years and older).

It is recommended that early school-age children (6 to 11 years old) diagnosed with congenital unilateral and bilateral cleft lip, alveolar ridge, hard and soft palate undergo computed tomography (CT) of the maxillofacial region to assess the existing defect in the anterior hard palate, the position of the maxillary fragments, and to plan the required size of the bone graft, as well as to evaluate adjacent bone structures and develop a further comprehensive treatment plan [31].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

It is recommended that adolescent patients (12 to 18 years old) diagnosed with congenital unilateral and bilateral cleft lip, alveolar ridge, hard and soft palate undergo computed tomography of the maxillofacial region to assess the anatomical relationships of the upper and lower jaws and determine indications for orthognathic surgery [31, 34].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

2.5 Other Procedures

Section 2.4 describes diagnostic methods that most comprehensively cover all aspects of the described diseases; however, the pathology is so diverse, and the combination of symptoms in each specific case is so specific, that additional methods may be applied depending on the clinical manifestations. No other specific diagnostics not described above are provided.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

3.1 Conservative Treatment

It is recommended to use special orthodontic appliances (palatal plates) in children aged 0–3 months diagnosed with cleft lip and palate in order to facilitate feeding, separate the oral and nasal cavities, and correct jaw deformity prior to surgical treatment [25, 26, 33, 37].

GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)

Patients diagnosed with cleft lip and palate are recommended to undergo pre-surgical orthodontic treatment in order to prepare for subsequent surgical interventions [27].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Patients diagnosed with cleft lip and palate are recommended to undergo speech therapy correction to develop proper articulation skills and prevent speech disorders, starting from the preoperative period and continuing after uranosplasty [52, 53, 54].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

It is recommended to provide psychological counseling for families with children diagnosed with cleft lip and palate to reduce anxiety [91].

Level of Evidence CGrade of Recommendation 5Level of Evidence C (reliability of evidence – 5)

3.2 Surgical Treatment

Patients diagnosed with cleft lip and palate are recommended to have the surgical treatment method selected based on the type of cleft, degree of hypoplasia, and deformity of the surrounding structures [23–29].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Patients diagnosed with cleft lip and palate are recommended to undergo restoration of the anatomical and functional integrity of the cleft area [30–35].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Patients diagnosed with cleft lip and palate are recommended to adhere to a staged treatment approach taking into account their somatic status [36–39].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

It is recommended to use a team approach (maxillofacial surgeon + orthodontist) to achieve optimal outcomes in patients diagnosed with cleft lip and palate [40–44, 85–87, 92–95].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Patients diagnosed with cleft lip and palate are recommended to undergo primary cheilorinoplasty to eliminate the upper lip defect and nasal deformity [87].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comment: The recommended age for primary cheilorinoplasty is older than 3–6 months during the first year of the child's life. Contraindications to early lip repair in a child include concomitant congenital malformations, birth trauma, postnatal encephalopathy, anemia, and malnutrition. In these cases, cheilorinoplasty is performed later once remission is achieved.

Patients diagnosed with secondary palate cleft are recommended to undergo tension-free uranosplasty [87].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comment: The recommended age for performing tension-free uranosplasty is 1–2 years.

Patients diagnosed with soft and hard palate cleft are recommended to undergo surgical intervention in two stages: the first stage is soft palate repair at the age of 1–2 years, and the second stage is tension-free uranosplasty after 3–4 years [87].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comment: The recommended age for performing the first stage (soft palate repair) is 1–2 years, and the second stage (tension-free uranosplasty) is after 3–4 years.

Patients diagnosed with cleft lip and palate, for the purpose of eliminating defects and deformities resulting from previously performed operations, are recommended to undergo reconstructive cheilorinoplasty, cheilorinoseptoplasty, or repeat uranosplasty (see tables 2–3 for details) [87, 92–95].

Level of Evidence CGrade of Recommendation 5Level of Evidence C (reliability of evidence – 5)

Comment: Corrective operations in the upper lip area can be performed at any age. The decision on the timing of corrective operations in the skin-cartilage part of the nose must be made individually, taking into account the severity of the pathology and the child's social adaptation. Full correction of the skin-cartilage part of the nose should be performed at 14–16 years of age, and if there is a need to correct a deformity of the nasal pyramid, the surgical intervention is postponed until 17 years of age.

Patients diagnosed with cleft lip and palate, for the purpose of developing proper speech, are recommended to undergo correction of velopharyngeal insufficiency after consultation with a medical speech therapist and an otorhinolaryngologist [26, 40].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comment: It is advisable to perform correction of velopharyngeal insufficiency starting from 6 years of age.

Patients diagnosed with cleft lip and palate, for the purpose of eliminating the alveolar ridge cleft of the upper jaw, are recommended to undergo alveolar bone grafting [26, 87].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Comment: The recommended age for performing alveolar bone grafting is from 8 to 10 years after orthodontic preparation.

Patients diagnosed with cleft lip and palate are recommended to undergo orthognathic surgery, secondary cheilorinoplasty/rhinoseptoplasty, repeat uranosplasty, correction of velopharyngeal insufficiency, alveolar bone grafting, and dental implantation after 18 years of age to eliminate postoperative and residual deformities of the upper lip and nose, the maxillary alveolar ridge defect, and velopharyngeal insufficiency (see tables 2–3 for details) [84, 86, 87, 92–95].

GDR CLE 5Grade of recommendation C (level of evidence – 5)

Table 2. Problems, tasks, and methods of surgical treatment for patients with unilateral cleft lip and palate.

Problems	Objectives	Solutions
The base of the columella and nasal septum are shifted toward the healthy side	Mobilization and repositioning of the anterior edge of the quadrangular cartilage.	Millard technique, Kozin technique Delaire technique Ficher
Deformity of the alar cartilage on the cleft side leading to asymmetry of the nasal tip, flattening of the alar wing, and varying nostril shapes	Mobilization of the alar cartilage, subperiosteal mobilization of the nasolabial muscle complex.	Millard technique, Kozin technique Delaire technique
Lateral displacement of the alar base on the cleft side	Mobilization of the alar wing, its medial displacement and fixation	Millard technique, Kozin technique Delaire technique
Deficiency of the nasal lining on the cleft side	Formation of the nasal passage floor	Millard technique, Kozin technique Delaire technique
Bundles of the orbicularis oris muscle running parallel to the edge of the cleft and attaching on the large fragment to the medial crus of the alar cartilage of the opposite side, and on the cleft side intertwining with the alar part of the nasalis muscle in the area of the alar base	Elimination of pathological fixation points of the orbicularis oris muscle with restoration of its sphincter function and formation of full myodynamic balance	Millard technique, Kozin technique Delaire technique
Disruption of upper lip continuity, distortion of Cupid's bow shape, preservation of only one philtral column	Restoration of upper lip continuity, restoration of the vermilion border contour	Millard technique, Kozin technique Delaire technique
Alveolar ridge defect of the upper jaw	Elimination of the bone defect	Skoog technique, Anderl technique Davydov-Bessonov technique Davis technique
Cleft of the hard and soft palate Defect of the hard and soft	Restoration of palatal integrity	Frolova technique, Delaire technique, Two-stage uranosplasty Ivanov-Ageeva technique Tension-free options of single- and/or two-flap hard palate repair Pavlovich-Ageeva technique
Shortening of the palate	Lengthening of the palate	Furlow Z-plasty, Soft palate repair by Frolova with narrowing of the pharyngeal ring
Dystopia of the muscles of the palatopharyngeal ring	Elimination of pathological attachment points with restoration of the muscular structure for velopharyngeal space occlusion	Ivanov- Ageeva technique Frolova technique Gentle options for uranoplasty according to Veau-Wardill-Killner Furlow Z-plasty Bardach two-flap palatoplasty method Two-stage uranosplasty

Table 3. Problems, objectives, and methods of surgical treatment in patients with bilateral cleft lip and palate.

Problems	Objectives	Solutions
Divergence of the alae nasi, flattening of the alar cartilages, absence of the nasal floor, widening of the nasal valve, shortening of the columella	Mobilization of the alar cartilages, subperiosteal mobilization of the nasolabial complex muscles, fixation, formation of the nasal tip and columella	Millard technique, Kozin technique Spina technique Manchester technique Delaire technique Davydov-Bessonov technique
Disruption of upper lip continuity, abnormal attachment of the orbicularis oris muscle, impairment of the oral fissure sphincter function	Restoration of the anatomical and functional integrity of the upper lip and orbicularis oris muscle	Millard technique, Kozin technique Spina technique Manchester technique Delaire technique Davydov-Bessonov technique
Reduction of the upper lip height	Increase of the upper lip height, philtrum formation	Millard technique, Kozin technique Spina technique Manchester technique Delaire technique Davydov-Bessonov technique Randell technique
Absence of the superior fornix of the oral vestibule	Formation of the superior fornix of the oral vestibule	Millard technique, Kozin technique Spina technique Manchester technique Delaire technique Davydov-Bessonov technique Randell technique
Bilateral alveolar bone defects of the maxilla	Elimination of the bone defect	Skoog technique, Anderl technique Davydov-Bessonov technique Davis technique
Cleft of the hard and soft palate Hard and soft palate defect	Restoration of palatal integrity	Frolova technique Two-stage uranosplasty Ivanov-Ageeva technique According to Dubov Gentle options for uranoplasty according to Veau-Wardill-Killner

3.3 Other treatment methods

No other specific treatment is provided for patients with the described pathology.

4. Medical Rehabilitation and Sanatorium-Resort Treatment

4.1 Ensuring adequate nutrition:

It is recommended to use special devices in newborns with isolated congenital maxillofacial malformations to maintain nutritional status [26, 101].

GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)

It is not recommended to use tube feeding in newborns with isolated congenital maxillofacial malformations to maintain nutritional status, due to the risk of suppressing the innate swallowing reflex and worsening the underdevelopment of the jaws and soft palate muscles [58].

GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)

Comment: tube feeding should be used under strict indications: in children with severe concomitant somatic pathology, in premature and immature newborns. The primary objective is to teach parents proper feeding with expressed breast milk/formulas and to prevent aspiration. Daily weight control is necessary in the first weeks of life.

For children with congenital maxillofacial malformations, in order to ensure conditions for their full social adaptation, surgical treatment is recommended to be performed in due time and in full volume [22, 45, 47, 48] – Tables 4-11.

GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)

Comment: medical rehabilitation is a long-term complex process (from birth to adolescence) and requires multidisciplinary participation of specialists: a neonatologist, maxillofacial surgeon, geneticist, pediatrician, orthodontist, medical speech-language pathologist, otorhinolaryngologist, and medical psychologist.

Table 4. Rehabilitation problems and their solutions during the neonatal period.

Problem	Solution strategy
Need for a multidisciplinary approach	Formation of a rehabilitation team for patients with cleft lip and palate
Cleft lip and palate	The specialist team assesses the cleft lip and palate, rules out other anomalies, and discusses the treatment plan with the family. Performing early orthodontic treatment, depending on the type of cleft.
Feeding difficulties and risk of weight deficit	Choice of feeding method, special feeding devices, regular (once a week) monitoring of growth and weight parameters
Diseases of the middle ear, nasopharynx, and oropharynx	The specialist team (otorhinolaryngologist and oto-rhino-laryngologist-audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing. Symptomatic treatment as indicated.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 5. Rehabilitation problems and their solutions in the period from 1 to 4 months.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the specialist team on preparing patients with cleft lip and palate for surgery
Cleft lip and palate	The specialist team determines the child's readiness for surgery, draws up a treatment plan, and discusses it with the family. Assessment of the effectiveness of previously performed orthodontic treatment. Regular replacement of the orthodontic appliance. Performing primary cheilorhinoplasty/cheilorhinoseptoplasty.
Weight deficit	Adjustment of the feeding method, special feeding devices, regular (once a week) monitoring of growth and weight parameters, correction of the identified weight deficit.
Diseases of the middle ear, nasopharynx, and oropharynx	The specialist team (otorhinolaryngologist and audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing. Symptomatic treatment as indicated. Identification of contraindications to surgery.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 6. Rehabilitation problems and their solutions in the period from 5 to 12 months.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the specialist team on surgical treatment and rehabilitation of patients with cleft lip and palate
Cleft lip, palate	Drawing up a surgical treatment plan, discussion with the family. Assessment of the effectiveness of previously performed orthodontic treatment. Performing primary cheilorhinoplasty/cheilorhinoseptoplasty. Preparation for single-stage uranoplasty or performing the first stage of palatoplasty in a two-stage procedure.
Weight deficit	Adjustment of the feeding method, special feeding devices, regular (once a week) monitoring of growth and weight parameters, correction of the identified weight deficit.
Diseases of the middle ear, nasopharynx, and oropharynx	The multidisciplinary team (otorhinolaryngologist and audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing in the postoperative period. Symptomatic treatment as indicated.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 7. Rehabilitation problems and their solutions for the period from 12 months to 2 years.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the specialist team on surgical treatment and rehabilitation of patients with cleft lip and palate
Cleft palate, alveolar ridge cleft	The multidisciplinary team performs the surgical intervention, informs about its scope, draws up a postoperative management plan, and discusses it with the family. Evaluation of the effectiveness of prior orthodontic treatment. Regular replacement of the orthodontic appliance. Performance of single-stage uranoplasty, or the second stage of palatoplasty in case of a two-stage surgery.
Diseases of the middle ear, nasopharynx, and oropharynx	The multidisciplinary team (otorhinolaryngologist-audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing in the postoperative period. Symptomatic treatment as indicated.
Teething and dental development disorders	Monitoring dental development. Oral hygiene training. Informing parents about expected dental issues.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 8. Rehabilitation problems and their solutions for the period from 2 to 5 years.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the multidisciplinary team on surgical treatment and rehabilitation of patients with cleft lip and palate.
Defects and deformities resulting from prior surgeries	Correction of velopharyngeal insufficiency, hard palate defects, reconstructive cheilorinoplasty/cheilorinoseptoplasty (as indicated). Evaluation of the effectiveness of prior orthodontic treatment. Regular replacement of the orthodontic appliance.
Teething and dental development disorders	Monitoring dental development. Oral hygiene monitoring. Dental sanation as indicated.
Diseases of the middle ear, nasopharynx, and oropharynx	The multidisciplinary team (otorhinolaryngologist-audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing in the postoperative period. Symptomatic treatment as indicated.
Speech dysfunction	Sessions with a medical speech therapist (logopedist), development of lower-costal breathing, phonemic hearing, and sound articulation training.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 9. Rehabilitation problems and their solutions for the period from 6 to 11 years.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the multidisciplinary team on the surgical treatment of patients with cleft lip and palate.
Residual deformity of the upper lip and nose, palate defects. Alveolar ridge cleft. Velopharyngeal insufficiency	The multidisciplinary team evaluates the effectiveness of prior surgical and orthodontic treatment, draws up a plan for further treatment and postoperative management, and discusses it with the family. Continuation of orthodontic treatment. Surgical treatment: correction of the alveolar ridge cleft, velopharyngeal insufficiency, cheilorinoplasty, uranoplasty.
Teething and dental development disorders	Monitoring dental development. Oral hygiene monitoring. Dental sanation as indicated.
Diseases of the middle ear, nasopharynx, and oropharynx	The multidisciplinary team (otorhinolaryngologist and audiologist) assesses the condition of the middle ear, oropharynx, nasopharynx, and hearing in the postoperative period. Symptomatic treatment as indicated.
Speech dysfunction	Sessions with a medical speech therapist (logopedist), development of lower-costal breathing, phonemic hearing, and sound articulation training.
Psychoemotional disorders	Identification of potential causes, implementation of measures to correct identified behavioral deviations.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 10. Rehabilitation problems and their solutions for the period from 12 to 18 years.

Problem	Solution strategy
Need for a multidisciplinary approach	Work of the multidisciplinary team on the surgical treatment and rehabilitation of patients with the consequences of cleft lip and palate treatment
Scar deformity of the upper lip and nose, palate defects. Alveolar ridge cleft. Velopharyngeal insufficiency. Combined jaw deformity.	The multidisciplinary team evaluates the effectiveness of prior surgical and orthodontic treatment, draws up a further surgical plan aimed at correcting jaw deformities and residual lip, nose, and palate defects and deformities, and discusses it with the family. Continuation of orthodontic treatment, preparation of dental arches for orthognathic surgery (as indicated). Surgical treatment: correction of combined jaw deformity, correction of alveolar ridge cleft, velopharyngeal insufficiency. Cheilorinoplasty.
Dental arch defects.	Intraosseous dental implantation. Fabrication of removable and fixed orthopedic appliances. Dental sanation as indicated.
Diseases of the middle ear, nasopharynx, and oropharynx	The otorhinolaryngologist evaluates the condition of the middle ear, oropharynx, and nasopharynx in the preoperative period. Symptomatic treatment as indicated.
Speech dysfunction	Sessions with a medical speech therapist (logopedist). Correction of speech and voice disorders.
Psychoemotional disorders	Identification of potential causes, implementation of measures to correct identified behavioral deviations.
Family's need for information and psychological support	Medical psychologist and social workers provide psychosocial support.

Table 11. Key provisions to avoid complications and accelerate the rehabilitation of patients with cleft lip and palate.

Position	Characteristic
Comprehensiveness	To provide comprehensive care and ensure complete rehabilitation of patients with congenital cleft lip and palate, coordinated work of a team of specialists is required: maxillofacial surgeon, orthodontist, geneticist, neonatologist, dentist, prosthodontist, otorhinolaryngologist-audiologist, pediatrician, neurologist, speech pathologist-logopedist, medical psychologist, as well as social workers.
Timeliness	Performing staged early surgical treatment, restoring the anatomical integrity and function of the upper lip, palate, and alveolar ridge with a minimal risk of facial skeleton growth disorders. Early speech correction and orthodontic treatment aimed at the proper formation of speech and occlusion. Timeliness of treatment helps avoid secondary deformities, reduce the number of surgical stages, and accelerate rehabilitation.
Consistency	Adherence to the stages of treatment ensures the continuity of work among team members. Continuity in the team approach increases the efficiency of each specialist, which significantly accelerates rehabilitation and reduces patient disability.
Administration	Regularity of follow-up observations makes it possible to monitor the quality of ongoing treatment throughout the entire rehabilitation period and make adjustments to the patient management plan in accordance with emerging changes.
Innovation	The application of modern surgical techniques grounded in the results of morphofunctional studies, the introduction of advanced computer technologies, and the use of the latest diagnostic methods and evaluation of treatment efficacy contribute to improving the quality of life of patients and their relatives, and serve as a basis for further improvement of the rehabilitation process.

5. Prevention and Dispensary Observation

It is recommended to conduct dispensary follow-up of patients diagnosed with cleft lip and palate from birth up to 18 years of age inclusive [87].

Level of Recommendation CLevel of Evidence 5Level of recommendation C (level of evidence – 5)

6. Organization of Medical Care

Type of medical care:

High-tech medical care.
High-tech medical care within the framework of compulsory health insurance.

Hospitalization is carried out according to the principle of staging and periodicity of treatment for these patients, in accordance with the algorithm.

Conditions of medical care: inpatient setting.

Form of medical care: elective.

7. Additional Information

Required qualifications of specialists.
Orthodontic preparation.
Presence of comorbid pathology.
Strict adherence to the attending physician's recommendations.

Criteria for Evaluating the Quality of Medical Care

Quality criteria	Evaluation criteria
A special orthodontic appliance (palatal plate) has been fabricated and fitted in a patient diagnosed with cleft upper lip and palate (0-3 months)	Yes/no
Primary cheilorinoplasty was performed in a patient with cleft lip and palate	Yes/no
Gentle uranoplasty was performed in a patient diagnosed with secondary palate cleft	Yes/no
Soft palate plastic surgery (first stage) and gentle uranoplasty (second stage) were performed in a patient diagnosed with hard and soft palate cleft	Yes/no
Psychological counseling of the family was conducted	Yes/no
A patient diagnosed with cleft lip and palate presenting with residual defects and deformities following previous surgeries underwent reconstructive cheilorinoplasty, cheilorinoseptoplasty, and secondary uranoplasty	Yes/no
A patient diagnosed with cleft lip and palate underwent correction of velopharyngeal insufficiency (following consultation with a medical speech-language pathologist and an otolaryngologist)	Yes/no
A patient diagnosed with a maxillary alveolar process cleft underwent alveolar bone grafting	Yes/no
A patient with a cleft of the upper lip, alveolar process, hard and soft palate presenting with residual defects and deformities following previous surgeries underwent orthognathic surgery, secondary cheilorinoplasty/rhinoseptoplasty, re-uranoplasty, correction of velopharyngeal insufficiency, alveolar bone grafting, and dental implantation (after 18 years of age)	Yes/no

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Stricker, P. A. Preoperative evaluation of children [Text] / P. A. Stricker, J. E. Fiadjoe // Pediatric Anesthesia. – 2018. – Vol. 28, № 11. – P. 940–946. – DOI: 10.1111/pan.13490.
Campbell, S. Prenatal Diagnosis: Clinical Cases and Imaging [Text] = Modern Guide to Prenatal Diagnosis / ed. by S. Campbell, C. Lees. – Cham : Springer, 2019. – 350 p. – ISBN 978-3-030-19270-2. – DOI: 10.1007/978-3-030-19270-2.
Kummer, A. W. Cleft Palate and Craniofacial Conditions: A Comprehensive Guide to Clinical Management [Text] / A. W. Kummer. – 4th ed. – Burlington : Jones & Bartlett Learning, 2020. – 550 p. – ISBN 978-1284178220
Stock, N. M. Prenatal Diagnosis of Cleft Lip and/or Palate: A Critical Review of the Literature and Recommendations for the Future [Text] / N. M. Stock, N. Rumsey // The Cleft Palate-Craniofacial Journal. – 2023. – Vol. 60, № 1. – P. 112–124. – DOI: 10.1177/10556656211052045
Current issues in the routing of patients with congenital clefts of the upper lip, palate, and alveolar process of the maxilla in the Astrakhan Region / E. V. Ryabykh, D. O. Ryabykh, L. A. Udochkina [et al.] // Caspian Journal of Medicine and Pharmacy. – 2024. – Vol. 5, № 2. – P. 52-59. – DOI 10.17021/2712-8164-2024-52-59. – EDN VQBKDQ
Plotnikov, N. A. The role of a multidisciplinary team in the rehabilitation of children with congenital cleft lip and palate / N. A. Plotnikov, T. V. Zueva // Russian Bulletin of Pediatric Surgery, Anesthesiology and Intensive Care. – 2020. – Vol. 10, № 3. – P. 45–56.
Pediatric Maxillofacial Surgery. Practical Guide: Textbook / ed. by O. Z. Topolnitsky, A. P. Gurgenadze. - 2nd ed. - Moscow : GEOTAR-Media, 2020. - 168 p. : ill. - ISBN 978-5-9704-5427-5 : 13440.00 KZT, 4058.00 KZT
Topolnitsky, O. Z. Pediatric Dentistry. Part 2. Surgery : Textbook / O. Z. Topolnitsky [et al.]. - in 3 parts. - Moscow : GEOTAR-Media, 2016. - 320 p. - ISBN 978-5-9704-3553-3. - Text : electronic // ELS "Student's Consultant" : [website]. - URL : https://www.studentlibrary.ru/book/ISBN9785970435533.html
Rogozhina, Yu. S., Blokhina, S. I., Bimbas, E. S., Galeyeva, K. V. Modern tactics of dynamic follow-up and treatment of patients with congenital maxillofacial pathology combined with anomalies of other organs and systems. Pediatric Dentistry and Prophylaxis 2023; 23(2): 92–103, https://doi.org/10.33925/1683–3031–2023–606.
Mamedov, A. A., Admakin, O. I., Nelyubina, O. V., Selitskaya, A. B., Blinder, Zh. A. Provision of specialized care to children with congenital cleft lip and palate // Sechenov Medical Journal. 2013. №1 (11). URL: https://cyberleninka.ru/article/n/okazanie-spetsializirovannoy-pomoschi-detyam-s-vrozhdennoy-rasschelinoy-verhney-guby-i-neba (date of access: 03.12.2025).
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Draft Order of the Ministry of Health of the Russian Federation "On Amendments to Order of the Ministry of Health of the Russian Federation No. 1130n of October 20, 2020, “On Approval of the Procedure for Providing Medical Care in the Field of ‘Obstetrics and Gynecology’”" (prepared by the Ministry of Health of Russia on 05.12.2024). – URL: https://www.garant.ru/ (18–21 weeks)
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Makhkamova, N. E., Nabieva, Zh. M., Yakubdzhanov, D. D., Nasretdinova, M. T. Condition of ENT organs in children with congenital cleft lip and palate // ISCMS. 2022. №1. URL: https://cyberleninka.ru/article/n/sostoyanie-lor-organov-u-detey-s-vrozhdyonnoy-rasschelinoy-guby-i-nyoba (date of access: 19.12.2025).
Bogoroditskaya, A. V., Sarafanova, M. E., Radtsig, E. Yu., Prityko, A. G. Condition of the nasal cavity and paranasal sinuses in children with congenital cleft lip and palate. Bulletin of Otorhinolaryngology. 2015;80(6):19–21.
Bogoroditskaya, A. V., Sarafanova, M. E., Radtsig, E. Yu., Prityko, A. G. An otorhinolaryngologist's perspective on the problems of children with occult cleft palate // RMJ. 2015. №15. URL: https://cyberleninka.ru/article/n/vzglyad-otorinolaringologa-na-problemy-detey-so-skrytoy-rasschelinoy-neba (date of access: 19.12.2025).
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Appendix A1. Working Group Composition

Kulakov, A. A. – Academician of the Russian Academy of Sciences, MD, Professor, President of the Association of Maxillofacial Surgery Specialists LLC.
Braylovskaya, T. V. – MD, Associate Professor, Executive Secretary of the Association of Maxillofacial Surgery Specialists LLC.
Bayrikov, I. M. – Corresponding Member of the Russian Academy of Sciences, MD, Professor, Member of the Association of Maxillofacial Surgery Specialists LLC.
Ivanov, S. Yu. – Corresponding Member of the Russian Academy of Sciences, MD, Professor, Board Member of the Association of Maxillofacial Surgery Specialists LLC.
Yaremenko, A. I. – MD, Professor, Vice President of the Association of Maxillofacial Surgery Specialists LLC.
Roginsky, V. V. – MD, Professor, Member of the Association of Maxillofacial Surgery Specialists LLC.
Topolnitsky, O. Z. – MD, Professor, Board Member of the Association of Maxillofacial Surgery Specialists LLC.
Durnovo, E. A. – MD, Professor, Board Member of the Association of Maxillofacial Surgery Specialists LLC.
Epifanov, S. A. – MD, Associate Professor, Member of the Association of Maxillofacial Surgery Specialists LLC.
Bagnenko A.S. – Candidate of Medical Sciences, Associate Professor, Member of the "Association of Specialists in Maxillofacial Surgery".
Loginopulo O.V. – Candidate of Medical Sciences, Associate Professor at the Department of Pediatric Maxillofacial Surgery, Russian University of Medicine of the Ministry of Health of the Russian Federation.
Yakovlev S.V. – Candidate of Medical Sciences, Associate Professor at the Department of Pediatric Maxillofacial Surgery, Russian University of Medicine of the Ministry of Health of the Russian Federation.
Lopatin A.V. – Doctor of Medical Sciences, Professor, Deputy General Director for Scientific and Clinical Work at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.
Ivanov A.L. – Candidate of Medical Sciences, Leading Researcher, Associate Professor at the Educational Activities Management Department, National Medical Research Center of Dentistry and Maxillofacial Surgery.
Pavlovich V.A. – Candidate of Medical Sciences, Senior Researcher at the Scientific and Methodological Department of the Scientific and Organizational Management, National Medical Research Center of Dentistry and Maxillofacial Surgery.
Ostanin A.V. – Candidate of Medical Sciences, Associate Professor at the Department of Dentistry and Maxillofacial Surgery with a Postgraduate Training Course, Astrakhan State Medical University of the Ministry of Health of the Russian Federation.
Verbo E.V. – Doctor of Medical Sciences, Professor, Plastic and Maxillofacial Surgeon, Head of the Reconstructive Surgery Department, Member of the Russian Society of Plastic, Reconstructive and Aesthetic Surgeons (RSPRAS).
Korchak V.V. – Candidate of Medical Sciences, Plastic Surgeon, Member of the Russian Society of Plastic, Reconstructive and Aesthetic Surgeons (RSPRAS).
Korableva N.P. – Doctor of Medical Sciences, Professor at the Department of Surgical Diseases, Mechnikov North-Western State Medical University, Member of the Russian Society of Plastic, Reconstructive and Aesthetic Surgeons (RSPRAS).
Glushko A.V. – Candidate of Medical Sciences, Plastic and Maxillofacial Surgeon, Head of the Department of Maxillofacial and Plastic Surgery at the LANCET Clinic, Peoples' Friendship University of Russia (IPHIK), Moscow, Member of the Russian Society of Plastic, Reconstructive and Aesthetic Surgeons (RSPRAS).
Sharobaro V.I. – Doctor of Medical Sciences, Professor, Head of the Department of Plastic Surgery, I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Member of the Russian Society of Plastic, Reconstructive and Aesthetic Surgeons (RSPRAS).

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience for these clinical guidelines:

Maxillofacial surgeons;

Orthodontists;

Pediatric dentists;

Dentists;

Oral and maxillofacial surgeons;

Otorhinolaryngologists;

Pediatricians;

Medical speech-language pathologists;

Medical psychologists.

Methods used for collecting/selecting evidence: electronic database search.

Description of methods used for evidence collection/selection: the evidence base for the guidelines includes publications from the Cochrane Library, EMBASE, MEDLINE, PUBMED databases, collections of the State Central Medical Library (including the dissertation collection), the B.N. Yeltsin Electronic Library, the Central Scientific Medical Library of I.M. Sechenov First Moscow State Medical University. The search depth was 10 years.

Methods used for assessing the quality and strength of evidence: expert consensus, significance assessment in accordance with the rating scheme (Tables App. 1, App. 2, App. 3).

Table App. 1. Scale for assessing levels of evidence (LE) for diagnostic methods (diagnostic interventions).

LoE	Definition
1	Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis
2	Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis
3	Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies
4	Non-comparative studies, case report
5	Only justification of mechanism of action or expert opinion is available

Table App. 2. Scale for assessing levels of evidence (LE) for prevention, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, therapeutic, and rehabilitation interventions).

LoE	Definition
1	Systematic review of randomized controlled trials with meta-analysis
2	Individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis
3	Non-randomized comparative studies, including cohort studies
4	Non-comparative studies, case report or case series, case-control study
5	Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available

Table App. 3. Scale for assessing grades of recommendation (GR) for prevention, diagnostic, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, diagnostic, therapeutic, and rehabilitation interventions).

GoR	Definition
A	Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent)
C	Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent)
C	Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent)

Procedure for updating clinical guidelines

The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

"Procedure for the provision of medical care in the profile of 'maxillofacial surgery', approved by the Ministry of Health of the Russian Federation on June 14, 2019, No. 422n (registered by the Ministry of Justice of the Russian Federation on August 30, 2019, registration No. 55783).

Appendix B. Physician Action Algorithms

Table App. 4. Patient model.

Appendix C. Patient Information

Cleft palate and cleft lip are congenital facial malformations.

A cleft lip appears as a slit-like opening or tear in the skin and mucosa of the upper lip. It often extends into the nasal region and can also involve the bones of the upper jaw.

A cleft palate is an opening in the palate. It can involve the entire palate (often extending to the alveolar processes of the maxilla) or be limited to the soft palate (the soft posterior part of the palate).

There are many different forms of clefts. These include cleft lip and alveolar process, with or without cleft palate; and isolated cleft palate. Clefts can be unilateral or bilateral, as well as complete and incomplete.

Cleft lip and palate is the most common congenital facial deformity (1/700 newborns), which has been a surgical challenge since ancient times. Chinese documents dated 390 BC contain a report of an Emperor's official who was operated on for an upper lip deformity. Other historical documents describe similar surgical interventions in the era of ancient Greeks and Romans.

Causes of cleft lip and palate

In most cases, the exact cause of these defects is unknown, and therefore it is impossible to prevent these deformities. It is believed that cleft lip and palate result from a combination of genetic and adverse environmental factors. If this anomaly has been detected in a child's parents, relatives, or siblings, the risk of its occurrence in the fetus is 15–33%.

Cleft palate and cleft lip can also result from the exposure of the fetus to viruses, chemicals, alcohol, drugs, certain medications (e.g., antiepileptic drugs), or vitamin deficiencies during the periods of upper lip and palate formation (5th–6th and 10th–11th weeks of pregnancy).

How is the diagnosis made?

Cleft lip and palate are easily diagnosed as they manifest with obvious defects. Ultrasound examination during pregnancy sometimes helps determine if an unborn child has a cleft. If deformities were not detected during prenatal ultrasound, the diagnosis is confirmed following a physical examination of the oral cavity, nose, and palate after the child's birth. Sometimes deformities may be detected during examinations aimed at identifying other developmental anomalies.

Associated problems

Feeding difficulties

In cleft palate, food and liquids can pass from the oral cavity into the nasal cavity. There are specially designed baby bottles and nipples that help direct liquids downward toward the esophagus.

Dental problems

Children with cleft palate or lip are more prone to dental caries and often have supernumerary, crooked, or displaced teeth that require dental and orthodontic intervention. In addition, such children often have a defect of the upper alveolar process (the bony part of the gums where teeth grow). An alveolar process defect can:

• provoke displacement, protrusion, or rotation of permanent teeth;

• hinder the normal eruption of permanent teeth;

• impede the normal formation of the alveolar ridge.

These problems can usually be managed by a maxillofacial surgeon and an orthodontist.

Speech problems

Children with cleft lip and palate in most cases may experience speech problems. The voice has a "nasal" quality, and understanding their speech can often be difficult. Not all children develop these problems. With surgical intervention, they can be completely eliminated in some patients. In other cases, the child will need to work with a medical speech-language pathologist—a specialist who will help the child overcome speech difficulties.

Who cares for children with cleft lip and palate?

An entire team of specialists is involved in correcting these defects. Such a team typically includes:

• a maxillofacial surgeon — to evaluate and perform necessary surgical procedures on the lip and/or palate, as well as the alveolar process;

• an orthodontist — to align and reposition teeth, preparing the child for surgery;

• a pediatric dentist, who will provide necessary dental and oral hygiene care;

• a prosthodontist — for the fabrication of artificial teeth and dental appliances that will improve appearance, restore normal speech function, and facilitate feeding;

• a medical speech-language pathologist, who will work with the child to develop and improve speech quality;

• an otolaryngologist-audiologist — a specialist in communication disorders caused by hearing loss or impairment — to evaluate and test hearing quality;

• a pediatrician — to ensure ongoing monitoring of the child's health and development;

• a social worker and/or medical psychologist — to provide psychological support to the family and assess any adaptation issues;

• a geneticist — to help parents and adult patients understand the chances of having future children with these deformities.

Stages of treatment for cleft lip and palate

Treatment of cleft lip is primarily surgical and performed in stages. The first stage is cheiloplasty and rhinoplasty. This surgery is performed during the first four to six months of life. Its goal is to eliminate the cosmetic defect and restore the normal anatomy of the upper lip and nose. The second stage is uranosplasty. This surgery is performed in one or two stages starting from 13 months of age. The third stage, alveolar bone grafting of the maxilla, is performed starting from 7 years of age, following special orthodontic preparation. Thus, by the time the child starts school and begins an active social life, treatment is nearly complete. Sometimes additional cosmetic surgeries are required at an older age to definitively eliminate postoperative scar deformity of the lip and nose. In some cases, if there is a significant combined jaw deformity that is refractory to orthodontic treatment, orthognathic surgery may be required after 16 years of age.

Expected Outcomes

Treatment of facial deformities can span several years and require multiple surgical procedures (depending on the severity of the deformity) combined with functional (orthodontic and speech therapy) outpatient care; however, the majority of children have a high chance of achieving a normal appearance, intelligible speech, and no feeding difficulties at an early age before starting school.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

Not applicable.

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