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cleft palate

SINA INSTITUTE OF NURSING AND ALLIED HEALTH SCIENCES Quetta

Assignment Topic: cleft palate

G-BSN Session 2021-2025(3rd batch- 5th semester)

Subject: pediatrics

Submitted to: Sir Adnan George

Student name: Shiraz khan

Date: 18-3-2024
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Table of contents

I. Abstract ------------------------------------------------------------------------------------------ 3

II. Introduction ------------------------------------------------------------------------------------- 4-5

III. Discussion -------------------------------------------------------------------------------------- 6

IV. What is cleft lip and causes and Risk factors ---------------------------------------------- 7

V. Diagnosis and management treatment ------------------------------------------------------ 8

VI. Surgery treatment ------------------------------------------------------------------------------ 9

VII. Conclusion ------------------------------------------------------------------------------------- 10

VIII. References -------------------------------------------------------------------------------------- 11


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Abstract

Infantile hemangioma (IH) with concurrent cleft lip and palate is a rare case. Surgical management is often

considered as the best management for infantile hemangioma with concurrent cleft lip and palate. However,

consideringthefunctionality aspectandaesthetic appearance, aplastic surgeon can also consider non-surgical

managementwithoutinterrupting the surgical timeline forthe cleft lip and palate. This case report aimed to describe

the role of oral propranolol and oral methylprednisolone for infantile hemangioma with concurrent cleft lip and

palate alongside the surgical management for cleft lip and palate.

A 2-month-old presented with complaints of swelling in her right upper nose and cheek along with cleft lip

and palate. She was treated with oral propranolol and oral methylprednisolone. Labioplasty was performed

whenshe was three months old. Palatoplasty and nasorraphy were done when she was one year old. A significant

reduction of the hemangiomawasseen and the corrective procedures showed a good result.

The use of propranolol and methylprednisolone for infantile hemangioma in our patient shows a good

result even when combined with labioplasty, palatoplasty, and nasorraphy for cleft lip and palate.

The management of infantile hemangioma with concurrent cleft lip and palate using oral propranolol and

oral methylprednisolone shows a good result with noside effects and can be elaboratedwith labioplasty,

palatoplasty, and nasorraphy, and will not interrupt the cleft lip and palate surgical timeline.

(Rifqi Fauzi 25- 2- 25)


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Introduction

These are the most severe of congenital anomalies which affect the mouth and related structures. The roof is

shaped from the palate and flooring from the constructions at the floor of the mouth. Laterally, it's bounded from

the cheeks. A cleft is a congenital abnormal space or gap in the upper lip, alveolus, or palate. The colloquial term

for this condition is harelip. The use of this term should be discouraged due to its demeaning connotation of

inferiority. The more appropriate terms are cleft lip, cleft palate or cleft lip and palate. So cleft lip and cleft palate

can be defined as:

Cleft lip: The failure of fusion of the frontonasal and maxillary processes, resulting in a cleft of varying extent

through the lip, alveolus, and nasal floor (an incomplete cleft does not extend through the nasal floor, while a

complete cleft implies lack of connection between the alar base and the medial labial element).

Cleft palate: The failure of fusion of the palatal shelves of the maxillary processes, resulting in a cleft of the hard

and/or soft palates. Clefts arises during the fourth developmental stage. Exactly where they appears is determined

by locations at which fusion of various facial processes failed to occur, this in turn is influenced by the time in

embryologic life when some interference with development occurred dromes. In the developed world, most

scientists believe that clefts occur due to a combination of genetic and environmental factors (e.g., maternal

illness, drugs, malnutrition). In developed countries, CL/P is typically identified before birth by ultrasonography.

Early detection allows time for parental education about the potential causes of the CL/P and procedures that the

child may need after birth. Consequently, due to the widespread access to medical care in developed countries,

although beliefs unsupported by science (e.g., superstitious beliefs) do exist, scientific causal beliefs are the most

commonly endorsed.
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In contrast, in developing countries where prenatal care is less advanced or less available, a CL/P is usually

unexpected and families rely less on medical explanations for the cleft from doctors and rely more on religion and

folklore to explain the deformity. For example, individuals in India who practice Hinduism believe that a CL/P is

the result of sins from a past life (Weatherley-White et al., 2005). Other religious and cultural beliefs regarding

causation of clefts include witchcraft, God's will, and engaging in a behaviour associated with causal power (e.g.,

looking at a child with a facial deformity when pregnant). Overall incidence of cleft lip and palate is

approximately 1 in 600 to 800 live births (1.42 in 1000) and isolated cleft palate occurs approximately in 1 in

2000 live births. Thus, the typical distribution of cleft types are.

1. Cleft lip alone – 15%

2. Cleft lip and palate – 45%

3. Isolated cleft palate – 40%.

The potential problems of the condition include social handicaps such as impaired suckling and resultant failure to

thrive, speech impediment, deafness, malocclusion, gross facial deformity and severe psychological problems.

The clefting of lip and/or palate occurs at such a strategic place in the orofacial region, at such a crucial time

(before birth) that it becomes a complex congenital deformity. Patient with oro-facial cleft deformity needs to be

treated at right time and at right age to achieve functional and aesthetic well-being. The treatment process is

complex, multidisciplinary and interdisciplinary approach. Successful management of the child born with a cleft

lip and palate requires coordinated care provided by a number of different specialties including oral/maxillofacial

surgery, otolaryngology, genetics/dysmorphology, speech/language pathology, orthodontics, prosthodontics, and

other. This successful reconstruction routinely requires multiple phase of surgical intervention.
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(Nelson et al., 2009)

Discussion

IH with concurrent cleft lip and palate is a rare case and possesses a particular challenge for clinicians. The role of

propranolol to accelerate the involution phase of hemangioma was discovered unexpectedly by Léauté-Labrèze et

al. when they initiated propranolol for infants with obstructive hypertrophic cardiomyopathy with hemangioma.

Propranolol is a non-selective β1‐ and β2‐adrenoceptor antagonist which causes vasoconstriction, impeding the

proangiogenic signals, and stimulating apoptosis in endothelial cells’ proliferation in regards to IH . Still, the

potential side effects of propranolol such as hypotension, bradycardia, and hypoglycemia, should also be

monitored.

Prior to the use of propranolol as IH treatment, corticosteroids were considered as the drug of choice for

hemangioma. Corticosteroids were also found to be an effective treatment for thrombocytopenia in a child with

hemangioma and prevented complications such as hemorrhagic events .However, long-term use of corticosteroids

has several side effects such as hypertension, glucose intolerance, wound complications, increased risk of

infections, etc. Nonetheless, corticosteroids are still included in the treatment of IH. The exact method for

reducing corticosteroid doses in IH patients is unknown. From the previous study, adequate time for treatment is

around 6–8 weeks . In addition to the therapeutic period, propranolol administration for IH was recommended for

a duration of 6 months and up to 12 months.Until now, hemangioma management is still controversial.

Traditionally, observation has been a therapeutic option in the hope that the lesions disappear spontaneously. This

choice arises when surgical excision or other therapies may worsen the condition. However, with changing

techniques, surgery can be the first-choice therapy, especially in the location of lesions with significant cosmetic
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or functional defects. Unfortunately, after we gave a choice for surgery or conservative therapy, the parents of the

patient preferred conservative treatment.

However, in our case, we did not observe any side effects of both propranolol and methylprednisolone in our

patient. Moreover, the use of propranolol and methylprednisolone for IH in our patient shows a good result even

when combined with labioplasty, palatoplasty, and nasorraphy for cleft lip and palate. (Rifqi Fauzi 25- 2- 25)

What is Cleft Lip?

The lip forms between the fourth and seventh weeks of pregnancy. As a baby develops during pregnancy, body

tissue and special cells from each side of the head grow toward the center of the face and join together to make

the face. This joining of tissue forms the facial features, like the lips and mouth. A cleft lip happens if the tissue

that makes up the lip does not join completely before birth. This results in an opening in the upper lip. The

opening in the lip can be a small slit or it can be a large opening that goes through the lip into the nose. A cleft lip

can be on one or both sides of the lip or in the middle of the lip, which occurs very rarely. Children with a cleft lip

also can have a cleft palate.

Causes and Risk Factors


The causes of orofacial clefts among most infants are unknown. Some children have a cleft lip or cleft palate

because of changes in their genes. Cleft lip and cleft palate are thought to be caused by a combination of genes

and other factors, such as things the mother comes in contact with in her environment, or what the mother eats or

drinks, or certain medications she uses during pregnancy.

Smoking―Women who smoke during pregnancy are more likely to have a baby with an orofacial cleft than

women who do not smoke.2-3


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Diabetes―Women with diabetes diagnosed before pregnancy have an increased risk of having a child with a cleft

lip with or without cleft palate, compared to women who did not have diabetes.5

Use of certain medicines―Women who used certain medicines to treat epilepsy, such as topiramate or valproic

acid, during the first trimester (the first 3 months) of pregnancy have an increased risk of having a baby with cleft

lip with or without cleft palate, compared to women who didn’t take these medicines.6-7

Diagnosis

Orofacial clefts, especially cleft lip with or without cleft palate, can be diagnosed during pregnancy by a routine

ultrasound. They can also be diagnosed after the baby is born, especially cleft palate. However, sometimes certain

types of cleft palate (for example, submucous cleft palate and bifid uvula) might not be diagnosed until later in

life.

Management and Treatment

Services and treatment for children with orofacial clefts can vary depending on the severity of the cleft; the child’s

age and needs; and the presence of associated syndromes or other birth defects, or both.

Surgery to repair a cleft lip usually occurs in the first few months of life and is recommended within the first 12

months of life. Surgery to repair a cleft palate is recommended within the first 18 months of life or earlier if

possible.8 Many children will need additional surgical procedures as they get older. Surgical repair can improve

the look and appearance of a child’s face and might also improve breathing, hearing, and speech and language

development. Children born with orofacial clefts might need other types of treatments and services, such as

special dental or orthodontic care or speech therapy.4,8


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With treatment, most children with orofacial clefts do well and lead a healthy life. Some children with orofacial

clefts may have issues with self-esteem if they are concerned with visible differences between themselves and

other children. Parent-to-parent support groups can prove to be useful for families of babies with birth defects of

the head and face, such as orofacial clefts.

June 28, 2023

Surgery Treatment

Unlike the artistic nature of the cleft lip repair, the cleft palate repair is very functional in nature. A team approach

has decreased the morbidity and secondary deformities caused by the cleft and mostly focuses quality of

speech.Soft palate repair techniques may be used in isolation or combined with hard palate procedures, as

necessary. Most surgeons today perform either some modification of an intravelar veloplasty, vs. A two flap

palatoplasty with double opposing z-plasty to achieve levator muscular repositioning.Maxillary distraction is

increasingly used for the correction of severe maxillary retrusion in patients with cleft lip and palate. Cleft lip and

palate children benefit from team approach special treatment requirements. Such a team lead by the plastic

surgeon should include a speech therapist and orthodontist having ready access to pediatric, ENT and dental

treatment facilities.Esenlik et al. Reviewed the literature on nasoalveolar molding (NAM) with an eye to both

benefits and limitations. A review of the literature suggests that NAM Cleft lip and palate children benefit from

team approach special treatment requirements. Such a team lead by the plastic surgeon should include a speech

therapist and orthodontist having ready access to pediatric, ENT and dental treatment facilities does not alter

skeletal facial growth when compared with the samples that did not receive PSIO (Presurgical infant orthopedics).

Nevertheless, the published studies on NAM show evidence of benefits to the patient, caregivers, the surgeon, and

society. These benefits include documented reduction in severity of the cleft deformity prior to surgery and as a
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consequence improved surgical outcomes, reduced burden of care on the caregivers, reduction in the need for

revision surgery and consequent reduced overall cost of care to the patient and society.Robotic cleft surgery is a

new and exciting field that holds numerous advantages to both patients and surgeons. Previous research in allied

health specialities has paved the way to the feasibility studies of robotic cleft surgery. Finally, the use of surgical

robots at present introduces economic challenges to implementation because of increased operative time and high

capital and operating costs and it is hoped that over time, costs will reduce and performance will increase as more

systems are developed in the future.

( J Family Med Prim Care. 2020 Jun)

Conclusion

The perfect thing to do for CLP is certainly to prevent its occurrence in the first place. The primary aim in CLP is

to educate parents and future mothers and fathers. Cleft lip and palate are both birth defects that affect different

structure and function such as speech difficulty, aesthetic, eating, nutrition etc. Patients with oro-facial cleft

deformity needs to be treated at right time and at right age to achieve functional and aesthetic well-being. The

mental status of patients with CLP should be considered and supported by psychological rehabilitation and their

morale should always be bolstered. Extensive dental treatment may be requires but it should not be made more

extensive or complex than is necessary to achieve a reasonable standard of dental perfection. The

multidisciplinary approach towards this problem led to a steady improvement in its end results.
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References

https://www.sciencedirect.com/science/article/pii/S2049080120301540

https://www.sciencedirect.com/science/article/pii/S2049080121006579

https://www.google.com/url?sa=t&source=web&rct=j&opi=89978449&url=https://www.cdc.gov/ncbddd/

birthdefects/

cleftlip.html&ved=2ahUKEwjenIqmg_2EAxXghf0HHcnMDdUQFnoECDEQAQ&usg=AOvVaw18DxVCCBvW

OPYznI1A6oHo

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7491837/

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