CHEILOSCHIZIS engleza

7/15/2019 CHEILOSCHIZIS engleza

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CLEFT LIP

1. INTRODUCTION - Cleft lip and palate are the mostcommon congenital craniofacial anomalies in humans,affecting approximately 1 in 700 live births2.EMBRYOLOGy: It is generally accepted that facialtissues, including the lip and palate, arise from neural crestcells (6). These cells migrate from their original positionsat the margins of the neural fold, providing themesenchyme for the paired maxillary and mandibularprocesses (which are in turn derivatives of the first and

second branchial arches), and the central frontonasalprocess.Bilateral nasal pits develop at the inferolateral aspects

on either side of the frontonasal prominence. The tissueson the sides of the nasal pits become the medial and

lateral nasal processes.


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Normally, the medial nasal prominences fuse with themaxillary processes below the nasal pit to form the upperlip. Failure of fusion results in cleft lip. The frontonasalprocess does not contribute to the lip, but develops into

the primary palate, which is the portion of the palatecontaining the alveolar bone between the canine teeth,extending posteriorly to the incisor foramenThe secondary palate, containing the alveolus, hard

palate, and soft palate posterior to the primary palate,arises as bilateral outgrowths from the maxillaryprocesses.Cleft palate may also result from disturbances in shelfgrowth, defective shelf fusion, failure of medial edge celldeath, postfusion rupture, and failure of mesenchymalconsolidation and differentiation.


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Agents that can negatively affect the growthand development of the primary palate (i.e., a

cleft extending ventrally from the incisiveforamen) include Dilantin (phenytoin), alcohol,hypoxia, retinoids (member of the vitamin Afamily), and possibly dietary factors such asfolate vitamin deficiency.


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Incomplete Unilateral Cleft Lip


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Complete Unilateral Cleft Lip


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Bilateral Cleft Lip


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4. SURGICAL CLEFT CORRECTION

The cleft lip is always repaired first at 3 to 4 months ofage.

The historic rule of 10s is a goodguideline: 10 weeks of age, 10 grams

of hemoglobin, and 10 pounds of weight. The cleft palate is traditionally repaired after the lip and may be done

between 9 and 15 months of age depending on the surgeon'sphilosophy. Early surgical efforts are purported to result in better speechfunction.

The Millard rotation-advancement cleft lip repair, however, has becomethe most commonly performed procedure for repair of the unilateral cleftlip.

The concept involves an inferior rotation of the medial into thesubcolumellar space to join with the medial lip segment. This procedure

achieves a lengthening of the lip along the philtral line, reconstruction ofthe orbicularis muscle across the cleft, rotation of the displaced nasalbase medially, a slight lengthening of the columella, and establishmentof a labial sulcus.


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The bilateral cleft lip represents more than just a doublingof the problem of the unilateral cleft lip. The lackof a columella, splaying of the alar cartilages and nasalbases, and protrusion of the underlying premaxilla not only

make the initial lip repair different from a unilateralcleft but also ensure that subsequent operations will beneeded. Decisions regarding repair of the bilateral cleftinclude whether to repair both lip clefts simultaneously

or in stages, whether to adhese the lip elements before adefinitive lip repair, and how to manage the protrusivepremaxilla.


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CLEFT PALATE


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The fundamental goals of the procedure are a soft palatalmuscular reconstruction, a two-layer flaps, and bilateralvermilion tissue converge to form the bilateral (nasal and

oral) lining closure of both hard and soft palatal defects,and adequate palatal length. Although there remains nouniform palatal repair technique or approach, the operationmay be conceived as three basic types: straight-line closure,V-Y lengthening, or Z-plasty rearrangement.

Opening the mucosa along the cleft edges, mobilizing tissueat the subperiosteal level, and mobilizing of the palatal flapsmedially enables a straight-line repair to be completed intwo layers. This technique, known as the von Langenbeck

repair, is straightforward but may not provide adequate softpalatal length.


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PREAURICULAR PITS, SINUSESAND ACCESSORY AURICLES

Preauricular sinuses most ascending helical rim. Most ofthese lesions remain asymptomatic, but when problematicfrequently present with drainage which may occurspontaneously or following infection.

Infection should be treated in the first instance with oralor i.v. antibiotics and following recovery, the sinus tractshould be excised. Infected lesions may need to be incisedand drained before definitive therapy, which in this context

is complicated by a higher recurrence rate.

Magnetic resonance imaging should be considered in allpatients with a suspected first branchial cleft anomaly.


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Accessory auricles aresituated in the line of fusionbetween maxillary anmandibular processes of the

first branchial arch and aremanaged by completeexcision

Surgery can be safelypostponed until after infancy.


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RANULA

Ranula is a term applied to a retentioncyst of the sublingual gland. It resultsfrom partial obstruction of the sublingualsalivary duct leading to dilatation of the

more proximal duct. The cyst is round oroval in shape and located beneath thetongue. It may occur congenitally andbe evident on antenatal ultrasoundscans. When large they interfere withrespiration and feeding.

Treatment of ranula consists ofmarsupialization of the cyst.


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THYREOGLOSSAL CYSTS

ETIOLOGYThe thyroid anlage (primordium) is a part ofthe second branchial arch located in the midline floor ofthe pharynx. It descents as a pouch from the foramen

cecum down to the neck during the third week of foetallive passing anterior, through or posterior to the hyoidbone. The cells differentiate at their final position intothe thyroid gland anterior to the thyroid cartilage . If

duct cells persist they can form a cyst or a sinus, whichis connected to the foramen cecum at the base of thetongue, but rarely a fistula with an external opening inthe middle of the neck, only after perforation of a cyst.


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DIAGNOSIS AND DIFFERENTIALDIAGNOSIS

Thyreoglossal duct cysts are found in 60% of the cases inthe midline at or below the hyoid bone.-24% are located above the hyoid and 8% intralingual.

-In the latter case they can cause respiratory distress oreven sudden infant deathwhen located at the base of the tongue.- During palpation uninfected cysts are often ballotable

and can be moved slightly from side to side, butnot up or down. Due to their origin from the foramencecum the thyroid cysts move upward during swallowingor when the tongue protrudes (this clinicalsign is difficult to observe in small infants


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DIFFERENTIAL DIAGNOSIS

The differential diagnosis includes :- complete ectopic thyroid gland or parts of the

thyroid,

- dermoid cysts,

- lipomas,- sebaceous cysts,

- submental lymphadenitis,

- thymus cysts or tumor.


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Therapy

Surgery of the thyreoglossal cyst or duct must alwaysinclude resection of the middle part of the hyoid corpuswhether or not the surgeon has the feeling that the ductends at the bone. In order to resect the hyoid bone, the

upper and lower rim have to be freed from the straightneck muscles omohyoid and sternohyoid muscle andthe middle 12 cm can be excised with a strong scissor.In some cases a clear continuity of the duct behind the

bone can be seen which then has to be resected up tothe base of the tongue including high ligature of thefistula.If the pathology presents primarily as an abscess,antibiotic therapy and a horizontal incision arerecommended.


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CONGENITAL VASCULARLESIONS

1.VASCULAR TUMORSHAEMANGIOMAS- These lesions form from endothelial cells in the walls ofcapillaries.

They are flat or absent at birth, but in the first few weeks of life they undergo a rapidproliferation. This is apparently in response to secretion of a growth factor known asalphafibroblast- derived growth factor. After 912 months, they enter a stationaryphase without growth before involuting at age three to five years. Involution as lateas 12 years has been reported. Cutaneous lesions are often red, as they aresuperficial and involve the skin (the traditional strawberry naevus).

The lesions, however, may be placed more deeply and may not discolour the skin atall.The conventional approach to haemangiomas is simply to observe the lesion andawait spontaneous involution. The exception is those lesions that impinge on function,such as vision or airway, which merit intervention.


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Haemangiomas often reduce in size when treated with corticosteroids. This effectmay be temporary. If treatment is required, then a choice must be made betweenprolonged steroid treatment and surgical intervention. Prolonged steroid treatmentmay have major side effects, with weight gain, hypertension and all the featuresof Cushing syndrome.Oral prednisone is used at a dose of 2 to 3 mg/kg/day. Doses up to 5 mg/kg/dayhave been administered for life-threatening complications with large hemangiomascausing airway obstruction or heart failure. The overall response rate is 80% to90%, with initial improvement in the color and tension of the mass usually notedwithin 1 week.

Intralesional corticosteroids are used for hemangiomas that cause local deformityor ulceration, especially for facial lesions of the eyelid, nose, cheek, or lip.41 Atotal of three to five injections (at a dose of 3 to 5 mg/kg per injection) aretypically given at intervals of 6 to 8 weeks.Surgical excision will leave a scar. Indications are the following: (1) when it isobvious that resection would be necessary sooner or later; (2) when the surgical

scar would be identical, regardless of timing of operation; and (3) when thesurgical scar is easily hidden.Finally, for the difficult-to-treat and life-threatening large hemangiomas, especiallyin the liver, angiographic embolization may be required to manage high-outputcardiac failure.There is an emerging role for laser treatment of haemangiomas.


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TUFTED ANGIOMA AND KAPOSIFORM HEMANGIOENDOTHELIOMA-Bothtypes of tumor are typically present at birth. Unlike infantile hemangioma, KHE andTA affect both genders equally, are unifocal, and generally involve the trunk,shoulder, thigh, or retroperitoneum.

The overlying skin is deep red-purple in color, tense, and shiny . Ecchymosisappears over and around the tumor in association with generalized petechiae andmay falsely raise concern for child abuse. Thrombocytopenia unresponsive toplatelet transfusion can be profound (


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VASCULAR MALFORMATIONS

Vascular malformations are localized or diffused errors of embryonicdevelopment which may affect any segment of the vascular tree includingarterial, venous, capillary, and lymphatic vessels. It is useful to subcategorizevascular malformations on the predominant type of channel abnormality and

fl ow characteristics. According to this distinction, two major categories exist: (1) slow-fl ow

anomalies (capillary malformations, lymphatic malformations, and venousmalformations) and (2) fast-fl ow anomalies (arterial malformations [e.g.aneurysm, coarctation, ectasia, and stenosis], AVMs, and arteriovenousfistulas


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Capillary Malformation

Still commonly referred to as port-wine orclaret stains, capillary malformations (CMs)are dermal vascular anomalies that arereported to occur in 0.3% of newbornswith an even gender distribution.


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Lymphatic Malformation

Historically termed lymphangioma or cystic hygroma, slow-fl ow vascularanomalies of the lymphatic system consist of localized or diffuse malformations oflymphatic channels best characterized as microcystic, macrocysticor both.Lymphatic malformations (LMs) most commonly appear as ballotable masses with

normal overlying skin, although a blue hue may result if large underlying cysts arepresent.The two strategies for treating lymphatic anomalies are sclerotherapy and surgicalresection. Sclerotherapy works through obliteration of the lymphatic lumen byendothelial destruction with subsequent sclerosis/fibrosis. Ethanol is widelyconsidered to be the most effective sclerosing agent for low flow malformations.Other sclerosant agents include doxycycline, sodium tetradecyl sulfate and OK-432.Resection is the only way to potentially cure LM. Often staged excision isnecessary and total excision is often possible. In each resection a surgeon shouldfocus on a defi ned anatomic region, attempt to limit blood loss, perform as

thorough a dissection as possible and to be prepared to operate as long asnecessary.


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Venous Malformation

Venous malformations are the most common of all vascular anomalies and arefrequently misdiagnosed as hemangiomas or mislabeled cavernous

hemangiomas. While present at birth, they are not always immediately evident.The typical description of a VM is of a blue, soft, and compressible mas.Therapy for venous malformations is sclerotherapy and surgical resection. For smallcutaneous or oromucosal VMs, injection with 13% sodium tetradecyl sulfate isoften successful. Ethanol is also used as described for lymphatic malformations.Venous anomalies have a propensity for recanalization and recurrence.

Excision of a VM is usually successful for small, well localized lesions. In somelocations, staged subtotal surgical removal can be accomplished withoutpreoperative sclerotherapy.


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Arteriovenous Malformations

Most often arteriovenous malformations are latent during infancy and childhoodand expand during adolescence manifesting as a warm, pink patch in the skinand an underlying thrill or bruit. Later, cutaneous consequences may includeischemic changes, ulceration, pain and intermittent bleeding.

The usual strategy is arterial embolization for the temporaryocclusion of the nidus 2472 h prior to surgical resection.If the arteries are tortuous or if the feeding arterieshave been ligated, sclerotherapy may play a rolein conjunction with local arterial and venous occlusion.Whenever possible, the lesion should be resected

completely.

CHEILOSCHIZIS engleza

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