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    CHAPTER I

    INTRODUCTION

    There are 3 topics related to the alimentary and hepatobiliary system and

    disorders block that will be discussed in this paper; cleft lip and palate,

    leukoplakia, and intussusception. Each topic is further elaborated in its own

    chapter.

    Orofacial clefts, ie. cleft lip (CL), cleft lip and palate (CLP), are among the

    most common congenital anomalies. Approximately 1 case of orofacial cleft

    occurs in every 500-550 births. In the United States, 20 infants are born with

    an orofacial cleft on an average day, or 7500 every year.1 Children who have

    an orofacial cleft require several surgical procedures and complex medical

    treatments; the estimated lifetime medical cost for each child with an

    orofacial cleft is $100,000, amounting to $750 million for all children with

    orofacial cleft born each year in the United States. Also, these children and

    their families often experience serious psychological problems.2

    The correct diagnosis of a cleft anomaly is fundamental for treatment, for

    further genetic and etiopathological studies, and for preventive measures

    correctly targeting the category of preventable orofacial clefts.3

    The World Health Organization (WHO) first defined oral leukoplakia as a

    white patch or plaque that could not be characterized clinically or

    pathologically as any other disease; therefore, lichen planus, candidiasis, and

    white sponge nevus were excluded.4

    No etiologic factor can be identified for most persistent oral white plaques

    (ie, idiopathic leukoplakia). The histopathologic features are highly variable,

    ranging from hyperkeratosis and hyperplasia to atrophy and severe dysplasia.4

    Patients with idiopathic leukoplakia have the highest risk of developing

    cancer. In studies of these patients, 4-17% had malignant transformation of

    the lesions in less than 20 years. The risk of developing malignancies at

    lesion sites is 5 times greater in patients with leukoplakia than in patients

    without leukoplakia.

    4

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    Estimates of malignant transformation vary from 3-33% over a 10-year

    period. However, many innocuous leukoplakias are not always followed up in

    some centers, and the studies are often small. As many as 30% of

    leukoplakias can regress if habits are stopped.5

    Patients must be aware that lesions may recur. They should be able to

    monitor the lesions and report any changes. They should maintain excellent

    oral hygiene. Patients should avoid any causal factor, such as use of tobacco

    and alcohol. Leukoplakias can regress under these circumstances. Any degree

    of dysplasia in a lesion at a high-risk site must be taken seriously and the

    lesion should be removed. Occasionally, patients are treated by photodynamic

    therapy or topical cytotoxic agents. Patients should be examined regularly,

    probably at 3- to 6-month intervals.5

    Intussusception is a common cause of childhood intestinal obstruction,

    occurring more frequently in white children aged 6 months to 2 years and in

    more males than in females (3:1-2). When this condition occurs in neonates

    and in children older than 2 years, there is a high incidence of associated

    bowel abnormality that serves as an initiating lead point for intussusception

    (about 5% of patients are found to have a lead point). In addition,

    intussusception is known to occur with greater frequency in children who

    have undergone recent abdominal surgery, either intraperitoneal or

    retroperitoneal operations. It is thought that early adhesions or focal edema of

    the bowel wall create a lead point for the intussusception.6

    Invagination of a bowel segment (usually, the small bowel) into the lumen of

    the more distal bowel (usually, the colon) occurs. The invaginated segment

    (intussusceptum) is carried distally by peristalsis. Mesentery and vessels

    become involved with the intraluminal loop and are squeezed within the

    engulfing segment (intussuscipiens). Almost all occurrences are acute, and

    bowel obstruction is often the presenting sign of intussusceptions.7

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    CHAPTER II

    CLEFT LIP AND PALATE

    2.1 Risk Factor

    There are many risk factors associated with cleft lip and palate. Ethnicity,

    family history, maternal exposure to environmental factors, maternal diet that

    cause the formation of cleft lip as well as gender are predisposing factors.1

    Figure 1. Risk Factor of Cleft Lip and Palate1

    2.2 Etiology and Epidemiology

    There are various causes of cleft lip and palate. In general, any factor that

    could prevent the processes from reaching each other by slowing down

    migration, multiplication, or both of neural crest cells by stopping tissue

    growth and development for a time or by killing some cells that are already in

    that location would cause a persistence of a cleft. Also, the epithelium that

    covers the mesenchyme may not undergo programmed cell death, so that

    fusion of processes cannot take place.1 However, among those that have been

    identified are genetic factors and environmental factors. It is believed that

    genetic factors give the baby a predisposition for the disease, but the exposure

    to environmental factors trigger the occurrence.2

    RISK

    FACTOR

    S

    Family

    Histo

    ry

    Maternal

    Obesit

    y

    Race Sex

    Environmental

    Factors

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    Maternal exposure to hypoxia during pregnancy, cigarette smoke, alcohol

    and illicit drugs are also common risk factors are known environmental risk

    factors. Pesticide exposure has also been pointed out. Maternal diet and

    vitamin intake; retinoid, anticonvulsant drugs, nitrate compounds, organic

    solvents, parental exposure to lead and illegal drugs also contribute to the

    occurrence of cleft lip and palate.2

    Epidemiological data suggests that in general, all typical orofacial cleft types

    combined occur in white populations with a frequency of 1 per 500-550 live

    births. The prevalence rate of clefts in different racial groups is considerable.

    The lowest rate is for blacks. A high prevalence of cleft lip with or without

    cleft palate was found for the Japanese population, and the highest prevalence

    was found for the North American Indian populations. In contrast, no

    remarkable variation among races was found in cleft palate. In particular, its

    prevalence did not significantly vary between black and white infants or

    between infants of Japanese and European origin in Hawaii.1

    The sex ratio in patients with clefts varies. In whites, cleft lip and cleft lip and

    palate occur significantly more often in males, and cleft palate occurs

    significantly more often in females. In cleft lip with or without cleft palate,

    the sex ratio correlates with the severity and laterality of the cleft. A large

    study of 8,952 orofacial clefts in whites found the male-to-female sex ratio to

    be 1.5-1.59:1 for cleft lip, 1.98-2.07:1 for cleft lip and palate, and 0.72-0.74:1

    for cleft palate.1

    2.3 Pathogenesis and Pathophysiology

    The embryological development of the upper lip and nose requires a sequence

    of complex, genetically programmed events. This involves fusion of the 5

    major facial prominences occurring between the 3rd and 8th week of

    gestation, with lip development between the 3rd and 7th weeks, and palate

    development between the 5th and 12th week.1

    The complexity of this cranio-facial developmental pathway and the

    numerous developmental points at which clefting could be induced is

    reflected in the heterogeneity of the phenotypic expression of the condition. 1

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    Cleft lip and/or palate: the maxillary, medial nasal and lateral nasal

    prominences converge through a complicated process of epithelial

    bridging, programmed cell death and sub-epithelial-mesenchymal

    penetration.Cleft lip and/or palate is likely to be secondary to a defect of

    epithelial fusion or mesenchymal growth, processes involving many

    possible genetic loci or intracellular signalling pathways.This results in

    interrupted fusion of the maxillary and median nasal prominences. In bi-

    lateral cleft lip with or without cleft palate, the arterial network and

    musculature of the lateral elements parallel that of the lateral segment of

    the uni-lateral deformity. The abnormal insertion of the cleft lip

    musculature follows the cleft margin up to the piriform aperture, and the

    prolabial segment receives its blood supply from the septal, columellar,

    and pre-maxillary vessels.

    Isolated uni-lateral cleft lip: the orbicularis oris (OO) is a ring of

    concentric muscle that constricts and puckers the sphincter of the mouth.

    In isolated uni-lateral cleft lip, the OO fibres on the cleft side insert into

    the nasal base, and the central (non-cleft) OO fibres abnormally insert

    into the nasal spine and septum. This causes the base of the nose to splay

    laterally when the infant smiles.

    Isolated cleft palate: the development of the palate involves fusion of the

    lateral palatal shelves and nasal septum in an anteroposterior direction

    from the incisive foramen to the uvula. A cleft palate is formed when

    normal palatal development is interrupted before the 12th week of

    gestation. The degree of clefting can range from a complete isolated cleft

    palate to a bifid uvula. Deformational cleft palate is seen in Pierre Robinsequence, where a small mandible (micrognathia) limits the space for the

    tongue, and the prominent tongue (glossoptosis) mechanically obstructs

    palatal fusion leading to the classic triad of micrognathia, glossoptosis

    and an isolated cleft palate.

    Midline clefts of the nose and/or lip: these are likely to arise from an

    interruption in the fusion of the paired median nasal prominences during

    embryological development. Most median facial deformities represent

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    developmental field defects, and are sporadic with multiple aetiological

    factors.

    In facial morphogenesis, neural crest cells migrate into the facial region,

    where they form the skeletal and connective tissue and all dental tissues

    except the enamel. Vascular endothelium and muscle are of mesodermal

    origin.

    The upper lip is derived from medial nasal and maxillary processes. Failure

    of merging between the medial nasal and maxillary processes at 5 weeks'

    gestation, on one or both sides, results in cleft lip. Cleft lip usually occurs at

    the junction between the central and lateral parts of the upper lip on either

    side. The cleft may affect only the upper lip, or it may extend more deeply

    into the maxilla and the primary palate. (Cleft of the primary palate

    includes cleft lip and cleft of the alveolus.) If the fusion of palatal shelves is

    impaired also, the cleft lip is accompanied by cleft palate, forming the cleft

    lip and palate abnormality. 1

    Cleft palate is a partial or total lack of fusion of palatal shelves. It can occur

    in numerous ways: 1

    Defective growth of palatal shelves

    Failure of the shelves to attain a horizontal position

    Lack of contact between shelves

    Rupture after fusion of shelves

    The secondary palate develops from the right and left palatal processes.

    Fusion of palatal shelves begins at 8 weeks' gestation and continues usually

    until 12 weeks' gestation. One hypothesis is that a threshold is noted beyond

    which delayed movement of palatal shelves does not allow closure to takeplace, and this results in a cleft palate. 1

    2.4 Clinical Manifestation

    Cleft lip can occur as a unilateral (on the left or right side) or as a bilateral

    anomaly. The line of cleft always starts on the lateral part of the upper lip and

    continues through the philtrum to the alveolus between the lateral incisor and

    the canine tooth, following the line of sutura incisiva up to the foramen

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    incisivum. The clefting anterior to the incisive foramen (ie, lip and alveolus)

    is also defined as a cleft primary palate. Cleft lip may occur with a wide

    range of severity, from a notch located on the left or right side of the lip to the

    most severe form, bilateral cleft lip and alveolus that separates the philtrum of

    the upper lip and premaxilla from the rest of the maxillary arch. 1

    When cleft lip continues from the foramen incisivum further through the

    sutura palatina in the middle of the palate, a cleft lip and palate (either

    unilateral or bilateral) is present.2

    A wide range of severity may be observed. The cleft line may be interrupted

    by soft (skin or mucosa) bridges, hard (bone) bridges, or both, corresponding

    to a diagnosis of an incomplete cleft. This occurs in unilateral and bilateral

    cleft lip and palate.2

    Several subtypes of cleft palate can be diagnosed based on severity. The

    uvula is the place where the minimal form of clefting of the palate is

    observed. However, a relatively high prevalence of this anomaly in the

    general population suggests that a certain proportion may represent the very

    far end of a normal variability. A more severe form is a cleft of the softpalate. A complete cleft palate constitutes a cleft of the hard palate, soft

    palate, and cleft uvula. The clefting posterior to the incisive foramen is

    defined as a cleft of secondary palate.3

    2.5 Diagnosis and Management

    Traditionally, the diagnosis is made at the time of birth by physical

    examination. Recent advances in prenatal diagnosis have allowed

    obstetricians to diagnose facial clefts in utero. Medical tests may be done to

    rule out other possible health conditions.3 Cleft lip can be easily diagnosed by

    performing ultrasonography in the second trimester of pregnancy when the

    position of the fetal face is located correctly. 1

    Cleft lip and palate is very treatable; however, the kind of treatment depends

    on the type and severity of the cleft.3

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    The cleft lip can be repaired by surgical therapy within the first 23 months

    after birth. While surgery to repair a cleft lip can be performed soon after

    birth, the often preferred age is at approximately 10 weeks of age, following

    the "rule of 10s" coined by surgeons Wilhelmmesen and Musgrave in 1969

    (the child is at least 10 weeks of age; weighs at least 10 pounds, and has at

    least 10g hemoglobin). If the cleft is bilateral and extensive, two surgeries

    may be required to close the cleft, one side first, and the second side a few

    weeks later. The most common procedure to repair a cleft lip is the Millard

    procedure pioneered by Ralph Millard. 3

    Often an incomplete cleft lip requires the same surgery as complete cleft.

    This is done for two reasons. Firstly the group of muscles required to purse

    the lips run through the upper lip. In order to restore the complete group a full

    incision must be made. Secondly, to create a less obvious scar the surgeon

    tries to line up the scar with the natural lines in the upper lip (such as the

    edges of the philtrum) and tuck away stitches as far up the nose as possible.

    Incomplete cleft gives the surgeon more tissue to work with, creating a more

    supple and natural-looking upper lip. 3

    In some cases of a severe bi-lateral complete cleft, the premaxillary segment

    will be protruded far outside the mouth. 3

    Nasoalveolar molding followed by surgery can improve long-term nasal

    symmetry among patients with complete unilateral cleft lip-cleft palate

    patients compared to surgery alone, according to a retrospective cohort study.

    Significant improvements in nasal symmetry were observed in the

    measurements of the projected length of the nasal ala, position of the

    superoinferior alar groove, position of the mediolateral nasal dome, and nasal

    bridge deviation. 3

    Cleft palate can also be corrected by surgery, usually performed between 6

    and 12 months. Approximately 20-25% only require one palatal surgery to

    achieve a competent velopharyngeal valve capable of producing normal, non-

    hypernasal speech. However, combinations of surgical methods and repeated

    surgeries are often necessary as the child grows. One of the new innovations

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    of cleft lip and cleft palate repair is the Latham appliance. The Latham is

    surgically inserted by use of pins during the child's 4th or 5th month. After it

    is in place, the doctor or parents turns a screw daily to bring the cleft together

    to assist with future lip and/or palate repair. 3

    If the cleft extends into the maxillary alveolar ridge, the gap is usually

    corrected by filling the gap with bone tissue. The bone tissue can be acquired

    from the patients own chin, rib or hip. 3

    After closing the cleft through surgical procedure, things to be considered are

    the speech and hearing ability of the patients. A tympanostomy tube is often

    inserted into the eardrum to aerate the middle ear. This is often beneficial for

    the hearing ability of the child. 3

    Speech problems are usually treated by a speech-language pathologist. In

    some cases pharyngeal flap surgery or augmentation pharyngoplasty is

    performed to reduce the escape of nasal airflow in speech sounds requiring

    oral air pressure, to improve the pronunciation of those sounds, and reduce

    nasality in those parts of speech that are not normally nasalized. The speech-

    language pathologist may also be called on to correct incorrect speakinghabits that the child developed before the cleft was corrected surgically. 3

    2.6 Prognosis

    Although treatment may continue for several years and require several

    surgeries, most children with a cleft lip and palate can achieve normal

    appearance, speech, and eating. However, some people may have continued

    speech problems. 3

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    CHAPTER III

    LEUKOPLAKIA

    3.1 Risk Factor

    Leukoplakia is a white lesion that, unlike oral candidiasis, cannot be removed

    by rubbing the mucosal surface. The areas of leukoplakia are usually small

    but may be several centimeters in diameter. Histologically, they are often

    hyperkeratoses occurring in response to chronic irritation (e.g., from tobacco

    and dentures); about 2-6%, however, represent either dysplasia or early

    invasive squamous cell carcinoma.4

    There are some risk factors that contribute to the occurrence of leukoplakia:

    a. Age

    Those with age older than 65 have increased risk of having leukoplakia.

    b. Sex

    More men than women get leukoplakia. In women, the condition more

    often develops into cancer.

    c. Lifestyle

    Tobacco (especially smokeless tobacco) and long-time alcohol use

    increase the vulnerability of getting leukoplakia.

    d. Conditions

    Incidence of leukoplakia increase in those with diminished immune

    system, such as in HIV-positive patients.

    3.2 Etiology and Epidemiology

    No etiologic factor can be identified for most persistent oral leukoplakias

    (idiopathic leukoplakia). Known causes of leukoplakia include the

    following:5

    Trauma (eg, chronic trauma from a sharp or broken tooth or from mastication

    may cause keratosis)

    Tobacco use: Chewing tobacco is probably worse than smoking.

    Alcohol

    http://www.empowher.com/node/43341#chttp://www.empowher.com/node/22069http://www.empowher.com/node/43341#chttp://www.empowher.com/node/22069
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    Infections (eg, candidosis, syphilis, Epstein-Barr virus infection): Epstein-

    Barr virus infection causes a separate and distinct nonpremalignant

    lesion termed hairy leukoplakia.

    Chemicals (eg, sanguinaria)

    Immune defects: Leukoplakias appear to be more common in transplant

    patients.

    Epidemiological study shows that leukoplakia is uncommoon, possibly

    occurring in less than 1% of adults. An increased prevalence is observed in

    communities and races with hibh tobacco use, such as Southeast Asia. Males

    have the highest incidence of leukoplakias. Leukoplakias are usually seen in

    adults older than 40.5

    3.3 Pathogenesis and Pathophysiology

    No etiologic factor can be identified for most persistent oral white plaques

    (ie, idiopathic leukoplakia). The histopathologic features are highly variable,

    ranging from hyperkeratosis and hyperplasia to atrophy and severe dysplasia.

    Patients with idiopathic leukoplakia have the highest risk of developing

    cancer. In studies of these patients, 4-17% had malignant transformation of

    the lesions in less than 20 years. The risk of developing malignancies at

    lesion sites is 5 times greater in patients with leukoplakia than in patients

    without leukoplakia.5

    Dysplastic lesions do not have any specific clinical appearance; however,

    where erythroplakia is present, dysplasia is likely. Dysplasia is evident in 17-

    25% of biopsy samples of leukoplakias. Erythroleukoplakias, verrucous

    leukoplakias, and nodular leukoplakias show an increasing frequency of

    dysplastic histologic changes or aneuploidy. 5

    Leukoplakias that are speckled, or erythroleukoplakic, are usually dysplastic

    or frank carcinomas. Nodular or verrucous lesions are also sinister, but

    homogenous leukoplakias are far less likely to be potentially malignant. 5

    Most idiopathic leukoplakias are homogenous leukoplakias and show little

    evidence of dysplastic histologic changes or aneuploidy. However, studies

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    have revealed carcinoma or severe dysplasia in the excision specimens of

    approximately 5% of leukoplakias excised when the diagnostic biopsy

    specimens had revealed no dysplasia. 5

    Carcinoma in situ is a controversial term used for severe dysplasia in which

    the abnormalities extend throughout the thickness of the epithelium. All the

    cellular abnormalities characteristic of malignancy may be present; only

    invasion of the underlying connective tissue is absent. Top-to-bottom

    epithelial dysplasia, like other dysplastic lesions, has no characteristic clinical

    appearance, although erythroplasia often proves to be carcinoma in situ or

    early invasive carcinoma. 5

    3.4 Clinical Manifestation

    Leukoplakia has a varied clinical appearance and its appearance frequently

    changes over time. Change or progression over time accounts for yet another

    unique aspect of leukoplakia, it is one of the few diseases in which long

    duration is not evidence of harmless future behavior. Lesions of long duration

    have a greater risk of malignant transformation than those of short duration,

    and the older a leukoplakia the worse is its prognosis.4

    Leukoplakias are white lesions that cannot be removed with a gauze swab.

    Most leukoplakias are smooth, white plaques (homogeneous leukoplakias),

    occur on the lip, the buccal mucosae, or the gingivae. Some leukoplakias are

    white and warty (verrucous leukoplakia), some are mixed white and red

    lesions (erythroleukoplakias or speckled leukoplakias). Dysplastic lesions do

    not have any specific clinical appearance; however, where erythroplasia is

    present, dysplasia, carcinoma in situ, and frank carcinomas are more likely to

    be seen. The site of the lesion is relevant; leukoplakias on the floor of the

    mouth or on the ventrum of the tongue and the lip are sinister. The size of the

    lesion appears to be irrelevant. Even small dysplastic lesions may lead to

    multiple carcinomas and a fatal outcome.5

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    3.5 Diagnosis and Management

    The first diagnostic method should be performed is physical examination. A

    systematic intraoral examination including the lateral tongue, floor of the

    mouth, gingiva, buccal area, palate, and tonsillar fossae and palpation of the

    neck for enlarged lymph nodes should be part of any general physical

    examination, especially in patients over the age of 45 who smoke tobacco or

    drink immoderately.4

    Oral biopsy is performed to detect the dysplastic cells. Intraoral staining with

    1% toluidine blue may aid in selection of the most suspicious biopsy site.4

    The recently introduced, computer-assisted, oral brush biopsy is a detection

    tool providing evidence of cellular abnormalities in precancerous and

    cancerous lesions. With the aid of a highly specialized, neural, network-

    based, image-processing system specifically designed to detect oral epithelial

    precancerous and cancerous cells, the pathologist can detect as few as 1 or 2

    abnormal individual cells in several hundred thousand cells. The detection of

    1 or 2 such abnormal cells is sufficient to warrant a histologic specimen

    obtained by scalpel biopsy.5

    The histopathologic features are highly variable, ranging from hyperkeratosis

    and hyperplasia to atrophy and severe dysplasia. The histologic assessment of

    oral epithelial dysplasia is notoriously unreliable. Many studies show

    interpathologist and intrapathologist variation in diagnosing dysplasia.

    Besides the fact that the criteria for diagnosing dysplasia are ill defined,

    another serious problem exists. A tissue specimen from a biopsy may not be

    representative of the whole lesion. Latent carcinomas may be missed.5

    Molecular and genetic analysis of premalignant and malignant tissue has

    produced increasing evidence of genetic instability (including microsatellite

    instability, cell cycle-regulatory gene P16 and P14 deletions and

    hypermethylation, and mutations in P53); and clonal alterations, such as loss

    of retinoic acid -receptor expression, occur during the early stage of

    aerodigestive tract carcinogenesis. These molecular and epidemiologic

    studies provide the foundation on which clinical trials have been designed to

    evaluate the role of retinoids and other compounds in the reversal of

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    premalignancy and the possible reduction in the 4-5% annual rate of second

    primary tumors.4

    Once the diagnosis is established, thorough management should be done. The

    objective of care is to detect and to prevent malignant change. Several

    management regimens have been suggested; however, no large trials have

    shown a definitive, reliable treatment. Yet, possible courses of action include

    medical therapies (eg, anti-inflammatory agents, vitamins, cytotoxic agents)

    and surgical removal (eg, scalpel, laser, cryoprobe, electrosurgery,

    photodynamic therapy).5

    Patients should avoid any causal factor, such as use of tobacco and alcohol.

    Leukoplakias can regress under these circumstances. Any degree of dysplasia

    in a lesion at a high-risk site must be taken seriously and the lesion should be

    removed. Occasionally, patients are treated by photodynamic therapy or

    topical cytotoxic agents. Patients should be examined regularly, probably at

    3- to 6-month intervals. A diet rich in fresh fruits and vegetables may help

    prevent cancer.5

    A number of clinical trials have suggested a role for beta-carotene, vitamin E,and retinoids in producing regression of leukoplakia and reducing the

    incidence of recurrent SCCs. Retinoids suppress head and neck and lung

    carcinogenesis in animal models and inhibit carcinogenesis in individuals

    with premalignant lesions. They also seem to reduce the incidence of second

    primary cancers in head and neck and lung cancer patients previously treated

    for a primary.4 Retinoids appear to be very effective but can have severe

    adverse effects on liver function and may cause teratogenicity. 5

    3.6 Prognosis

    Some leukoplakias are potentially malignant. Dysplasia currently appears to

    be the best predictor of malignant potential. As many as 25% of leukoplakias

    are dysplastic at the first visit. Malignant change appears to be more frequent

    among nonsmokers than among smokers.5

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    Estimates of malignant transformation vary from 3-33% over a 10-year

    period. However, many innocuous leukoplakias are not always followed up in

    some centers, and the studies are often small. As many as 30% of

    leukoplakias can regress if habits are stopped. A poorer prognosis is noted in

    females, nonsmokers, moderate or severe epithelial dysplasia, and lesions in

    high-risk sites, such as the floor of the mouth.5

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    CHAPTER IV

    INTUSSUSCEPTION

    3.1 Risk Factor

    Intussusception is a process in which a segment of intestine invaginates into

    the adjoining intestinal lumen, causing bowel obstruction.6 There are some

    conditions that can increase the risk of intussusception, including:6

    a.Age

    Children are much more likely to develop intussusception than adults are.

    It is the most common cause of bowel obstruction in children between the

    ages of 3 months and 6 years, with the majority of cases occurring in

    children younger than 1 year.

    b.Sex

    Intussusception affects boys more often than girls.

    c.Abnormal Intestinal Formation at Birth

    Malrotation, a condition present at birth (congenital) in which the

    intestine doesnt develop correctly, also is a risk factor for

    intussusception.

    d.Prior History of Intussusception

    The risk of developing intussusception increases in those who have prior

    history of this disease.

    3.2 Etiology and Epidemiology

    The cause of most intussusception is unknown. It is postulated that

    gastrointestinal infection or the introduction of new food proteins results in

    swollen Peyer patches in the terminal ileum. The prominent mounds of tissue

    lead to mucosal prolapse of the ileum into the colon, thus causing an

    intussusception. In 2-8% of patients, recognizable lead points for the

    intussusception are found, such as a Meckel diverticulum, intestinal polyp,

    neurofibroma, intestinal duplication, hemangioma, or malignant conditions

    such as lymphoma. Lead points are more common in children older than 2

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    year of age. Intrauterine intussusception is associated with the development

    of intestinal atresia.7

    The true prevalence of intussusception is difficult to be obtained because of a

    wide geographic variation in incidence of intussusception among countries

    and cities within a country. There is no significant difference in the incidence

    of intussusception is reported between races. Most series report a slight

    preponderance of males, with a male-to-female ratio of approximately 3:2.6

    Two thirds of children with intussusception are younger than 1 year; most

    commonly, intussusception occurs in infants aged 5-10 months. Although

    extremely rare, intussusception has been reported in the neonatal period.

    Intussusception can account for as many as 25% of abdominal surgical

    emergencies in children younger than 5 years, exceeding the incidence of

    appendicitis. Intussusception is the most common cause of intestinal

    obstruction in patients aged 5 months to 3 years.6

    3.3 Pathogenesis and Pathophysiology

    The pathogenesis of intussusception is believed to be secondary to an

    imbalance in the longitudinal forces along the intestinal wall. This imbalance

    can be caused by a mass acting as a lead point or by a disorganized pattern of

    peristalsis (eg, an ileus in the postoperative period). Electrolyte derangements

    associated with various medical problems can produce aberrant intestinal

    motility, leading to its invagination. Recent experimental studies in animals

    showed that abnormal intestinal release of nitric oxide, an inhibitory

    neurotransmitter, caused relaxation of the ileocecal valve predisposing to

    ileocecal intussusception. Other studies have demonstrated that the use of

    certain antibiotics leads to ileal lymphoid hyperplasia and intestinal

    dysmotility with resultant intussusception.6

    As a result of the imbalance, an area of the intestinal wall invaginates into the

    lumen, with the rest of the intestine following. The invaginating portion of

    the intestine (ie, intussusceptum) completely invaginates into the receiving

    portion of the intestine (ie, intussuscipiens). This process continues and more

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    proximal areas follow, allowing the intussusceptum to proceed along the

    lumen of the intussuscipiens. 6

    If the mesentery of the intussusceptum is lax and the progression is rapid, the

    intussusceptum can proceed to the distal colon or sigmoid and even prolapse

    out the anus. The mesentery of the intussusceptum is invaginated with the

    intestine, leading to the classic pathophysiologic process of any bowel

    obstruction. 6

    Early in this process, lymphatic return is impeded; then, with the rise in the

    pressure within the wall of the intussusceptum, venous drainage is impaired.

    Finally, the pressure reaches a point at which arterial inflow is inhibited, and

    infarction ensues. The mucosa is most sensitive to ischemia because it is

    farthest away from the arterial supply. Ischemic mucosa sloughs off, which

    initially leads to the heme-positive stools and then the classic "currant jelly

    stool" (a mixture of sloughed mucosa, blood, and mucus). If untreated, the

    process progresses to transmural gangrene and perforation of the leading edge

    of the intussusceptum. 6

    3.4 Clinical Manifestation

    In typical cases there is sudden onset, in a previously well child, of severe

    paroxysmal colicky pain that recurs at frequent intervals and is accompanied

    by straining efforts with legs and knees flexed and loud cries. The infant may

    initially be comfortable and play normally between the paroxysms of pain;

    but if the intussusception is not reduced, the infant becomes progressively

    weaker and lethargic. At times, the lethargy is out of proportion to the

    abdominal signs. Eventually a shocklike state may develop with fever. The

    pulse becomes weak and thready, the respirations become shallow and

    grunting, and the pain may be manifested only by moaning sounds. Vomiting

    occurs in most cases and is usually more frequent early. In the later phase, the

    vomitus becomes bile stained. Stools of normal appearance may be evacuated

    during the first few hours of symptoms. After this time, fecal excretions are

    small or more often do not occur and little or no flatus is passed. Blood

    generally is passed in the first 12 hr but at times not for 12 days and

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    infrequently not at all; 60% of infants pass a stool containing red blood and

    mucus, the currant jelly stool. Some patients have only irritability and

    alternating or progressive lethargy.7

    Palpation of the abdomen usually reveals a slightly tender sausage-shaped

    mass, sometimes ill defined, which may increase in size and firmness during

    a paroxysm of pain and is most often in the right upper abdomen, with its

    long axis cephalocaudal. If it is felt in the epigastrium, the long axis is

    transverse. About 30% of patients do not have a palpable mass. The presence

    of bloody mucus on the finger as it is withdrawn after rectal examination

    supports the diagnosis of intussusception. Abdominal distention and

    tenderness develop as intestinal obstruction becomes more acute. On rare

    occasions, the advancing intestine prolapses through the anus. This prolapse

    can be distinguished from prolapse of the rectum by the separation between

    the protruding intestine and the rectal wall, which does not exist in prolapse

    of the rectum. 7

    Ileoileal intussusception may have a less typical clinical picture, the

    symptoms and signs being chiefly those of small intestinal obstruction.

    Recurrent intussusception is noted in 58% and is more common after

    hydrostatic than surgical reduction. Chronic intussusception, in which the

    symptoms exist in milder form at recurrent intervals, is more likely to occur

    with or after acute enteritis and may arise in older children as well as in

    infants. 7

    3.5 Diagnosis and Management

    The clinical history and physical findings are usually sufficiently typical for

    diagnosis. Plain abdominal radiographs may show a density in the area of the

    intussusception. A barium enema shows a filling defect or cupping in the

    head of barium where its advance is obstructed by the intussusceptum. A

    central linear column of barium may be visible in the compressed lumen of

    the intussusceptum, and a thin rim of barium may be seen trapped around the

    invagination intestin in the folds of mucosa within the intussuscipiens

    (coiled-spring sign), especially after evacuation. Retrogression of the

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    intussusceptum under the pressure of the enema and gaseous distention of the

    small intestine from obstruction are also useful radiographic signs. Ileoileal

    intussusception is usually not demonstrable by barium enema but is suspected

    because of gaseous distention of the intestine above the lesion. The use of

    air enemas in the diagnosis and treatment of intussusception has supplanted

    hydrostatic reduction. Reflux of air into the terminal ileum and the

    disappearance of the mass at the ilocecal valve document successful

    reduction. Air reduction is associated with fewer complications and lower

    radiation exposure than traditional hydrostatic techniques.7

    Laboratory investigation is usually not helpful in the evaluation of patients

    with intussusception. Leukocytosis can be an indication of gangrene if the

    process is advanced. Dehydration is depicted by electrolyte imbalances.6

    Ultrasonography is a noninvasive modality that can aid in making the

    diagnosis of intussusception. Its accuracy reaches 100%. Hallmarks of

    ultrasonography include depiction of the intussusceptum and its mesentery

    within the intussuscipiens. Ultrasonography is highly operator dependent;

    therefore, interpret result with caution.(medscape) The diagnostic findings of

    intussusception include a tubular mass in longitudinal views and a doughnut

    or target appearance in transverse images. Ultrasonography is also useful in

    demonstrating reduction of the intussusception by hydrostatic or air

    techniques.7

    In histologic examination, typical findings in a segment of intestine which is

    resected at the time of operative reduction are intestinal obstruction with

    edema, congestion, lymphocytic infiltration, and transmural infarction.6

    The differential diagnoses of intussusception can be appendicitis, colic, cyclic

    vomiting syndrome, gastroenteritis, and volvulus.6

    Reduction of an acute intussusception is an emergency procedure and

    performed imediately after diagnosis in preparation for possible surgery. In

    patients with prolonged intussusception with signs of shock, peritoneal

    irritation, intestinal perforation, or pneumatosis intestinalis, reduction should

    not be attempted.7

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    The success rate of radiologic reduction under fluoroscopic or ultrasonic

    guidance is approximately 50% if symptoms are present longer than 48 hr

    and 70-90% if reduction is done within the first 48 hr. bowel perforatons

    occur in 0,5-2,5% of attempted barium and hydrostatic (saline) reductions.

    The perforation rate with air reduction ranges from 0,1-0,2%.7

    An ileoileal intussusception is best demonstrated by abdominal

    ultrasonography. Reduction by instillation of barium, saline, or air may not be

    possible. Such intussusceptions may develop insidiously after bowel surgery

    and require reoperation if they do not spontaneously reduce. If manual

    operative reduction is impossible or the bowel is not viable, resection of the

    intussusception is necessary, with end-to-end anastomosis. 7

    3.6 Prognosis

    Untreated intussusception in infants is almost always fatal; the chances of

    recovery are directly related to the duration of intussusception before

    reduction. Most infants recover if the intussusception is reduced within the

    first 24 hr, but the mortality rate rises rapidly after this time, especially after

    the second day. Spontaneous reduction during preparation for operation is not

    uncommon. 7

    Recurrence rate of intussusception after nonoperative reduction is usually less

    than 10% but has been reported to be as high as 21%. Most intussusceptions

    recur within 72 hours of the initial event; however, recurrences have been

    reported as long as 36 months later. More than one recurrence suggests the

    presence of a lead point. A recurrence is usually heralded by the onset of the

    same symptoms as appeared during the initial event. Provide similar

    treatment for a recurrence unless the suggestion of a lead point is very strong;

    in which case, contemplate surgical exploration. The recurrence rates after air

    enema and barium enema are 4% and 10% respectively. Recurrences respond

    to nonoperative reduction in almost 95% of cases. 6

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    CHAPTER V

    SUMMARY

    Cleft lip and palate is common among congenital anomalies. Several risk factors of

    this disorder are ethnicity, family history, maternal exposure to environmental

    factors, maternal diet that cause the formation of cleft lip as well as gender. The

    genetic factor gives predisposition to this disease, but exposure to environmental

    factors trigger the occurrence. Epidemiological data suggests that in general, all

    typical orofacial cleft types combined occur in white populations with a frequency of

    1 per 500-550 live births. Failure of merging between the medial nasal and maxillary

    processes at 5 weeks' gestation, on one or both sides, results in cleft lip.If the fusion

    of palatal shelves is impaired also, the cleft lip is accompanied by cleft palate,

    forming the cleft lip and palate abnormality. Traditionally, the diagnosis is made at

    the time of birth by physical examination.Cleft lip and palate is very treatable;

    however, the kind of treatment depends on the type and severity of the cleft. Speech

    problems are usually treated by a speech-language pathologist. After surgical

    intervention, most children with a cleft lip and palate can achieve normal appearance,

    speech, and eating.

    Leukoplakia is a white lesion that, unlike oral candidiasis, cannot be removed by

    rubbing the mucosal surface. Several predisposing factor to this disorders are age

    older than 65, male, tobacco use and alcohol consumption, and state of diminished

    immune system. No etiologic factor can be identified for most persistent oral

    leukoplakias (idiopathic leukoplakia), but several causes have been identified, which

    are related to the predisposing factors. Patients with idiopathic leukoplakia have the

    highest risk of developing cancer. In studies of these patients, 4-17% had malignant

    transformation of the lesions in less than 20 years. Leukoplakia has a varied clinical

    appearance and its appearance frequently changes over time. Most leukoplakias are

    smooth, white plaques (homogeneous leukoplakias), occur on the lip, the buccal

    mucosae, or the gingivae. Diagnostic method includes physical examination and oral

    biopsy. Some leukoplakias are potentially malignant. A poorer prognosis is noted in

    females, nonsmokers, moderate or severe epithelial dysplasia, and lesions in high-

    risk sites, such as the floor of the mouth.

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    Intussusception is a process in which a segment of intestine invaginates into the

    adjoining intestinal lumen, causing bowel obstruction. Several risk factors contribute

    to occurrence of this disease,ie. children younger than 1 year, boys, abnormal

    intestinal formation at birth, and prior history of intussusception. The cause of most

    intussusception is unknown. It is postulated that gastrointestinal infection or the

    introduction of new food proteins results in swollen Peyer patches in the terminal

    ileum. Two thirds of children with intussusception are younger than 1 year; most

    commonly, intussusception occurs in infants aged 5-10 months. There is no

    significant difference in the incidence of intussusception is reported between races.

    Most series report a slight preponderance of males, with a male-to-female ratio. The

    pathogenesis of intussusception is believed to be secondary to an imbalance in the

    longitudinal forces along the intestinal wall. In typical cases there is sudden onset, in

    a previously well child, of severe paroxysmal colicky pain that recurs at frequent

    intervals and is accompanied by straining efforts with legs and knees flexed and loud

    cries. The clinical history and physical findings are usually sufficiently typical for

    diagnosis. Another tests are plain abdominal radiography (or with contrast) and

    ultrasonography. Reduction of an acute intussusception is an emergency procedure

    and performed imediately after diagnosis in preparation for possible surgery.

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    REFERENCES

    1. Marie M Tolarova. Cleft Lip and Palate. [cited 2009 March]. Available from:http://emedicine.medscape.com/article/205231-overview

    2. Kirschner R E, LaRossa D. Cleft lip and palate. Otolaryngol Clin North Am2000; 33: 1191-1215.

    3. Cohen MM. Etiology and pathogenesis of orofacial clefting. In: Oral andMaxillofacial Surgical Clinics of North America. Vol 12. 2000:379-97.

    4. Stephen J. McPhee and Maxine A. Papadakis. Current Medical Diagnosisand Treatment. McGraw Hill 2007. Chapter 8.

    5. Crispian Scully. Oral Leukoplakia. eMedicine. [cited 2010 April]. Availablefrom: http://emedicine.medscape.com/article/ 1075448-overview

    6. Felix C Blanco.Intussusception. eMedicine. [cited 2010 February]. Availablefrom: http://emedicine.medscape.com/article/196411-overview

    7.Richard E Behrman & Robert M Kliegman. 4

    th

    Edition Essentials ofPediatrics. Philadelphia. W.B. Saunders Company.