CLEFT LIPCLEFT LIP

11. . INTRODUCTION - Cleft lip and palate are the INTRODUCTION - Cleft lip and palate are the most common congenital craniofacial anomalies in most common congenital craniofacial anomalies in humans, affecting approximately 1 in 700 live humans, affecting approximately 1 in 700 live birthsbirths 2.EMBRYOLOGy: It is generally accepted that facial 2.EMBRYOLOGy: It is generally accepted that facial tissues, including the lip and palate, arise from tissues, including the lip and palate, arise from neural crest cells (6). These cells migrate from their neural crest cells (6). These cells migrate from their original positions at the margins of the neural fold, original positions at the margins of the neural fold, providing the mesenchyme for the paired maxillary providing the mesenchyme for the paired maxillary and mandibular processes (which are in turn and mandibular processes (which are in turn derivatives of the first and second branchial derivatives of the first and second branchial arches), and the central frontonasal process.arches), and the central frontonasal process.

Bilateral nasal pits develop at the Bilateral nasal pits develop at the inferolateral aspects on either side of the inferolateral aspects on either side of the frontonasal prominence. The tissues on the sides of frontonasal prominence. The tissues on the sides of the nasal pits become the medial and lateral nasal the nasal pits become the medial and lateral nasal processes. processes.

Normally, the medial nasal prominences fuse with the maxillary processes below the nasal pit to form the upper lip. Failure of fusion results in cleft lip. The frontonasal process does not contribute to the lip, but develops into the primary palate, which is the portion of the palate containing 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 maxillary processes. Cleft palate may also result from disturbances in shelf growth, defective shelf fusion, failure of medial edge cell death, postfusion rupture, and failure of mesenchymal consolidation and differentiation.

Agents that can negatively affect the growth and development of the primary palate (i.e., a cleft extending ventrally from the incisive foramen) include Dilantin (phenytoin), alcohol, hypoxia, retinoids (member of the vitamin A family), and possibly dietary factors such as folate vitamin deficiency.

Incomplete Unilateral Cleft LipIncomplete Unilateral Cleft Lip

Complete Unilateral Cleft LipComplete Unilateral Cleft Lip

Bilateral Cleft LipBilateral Cleft Lip

44. . SURGICAL CLEFT CORRECTIONSURGICAL CLEFT CORRECTION The cleft lip is always repaired first at The cleft lip is always repaired first at 3 to 4 months of 3 to 4 months of age.age. The historic rule The historic rule of 10s is a good of 10s is a good guideline: 10 weeks of age, 10 guideline: 10 weeks of age, 10

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

done between 9 and 15 months of age depending on the done between 9 and 15 months of age depending on the surgeon's philosophy. Early surgical efforts are purported to surgeon's philosophy. Early surgical efforts are purported to result in better speech function.result in better speech function.

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

The concept involves an inferior rotation of the medial into the The concept involves an inferior rotation of the medial into the subcolumellar space to join with the medial lip segment. This subcolumellar space to join with the medial lip segment. This procedure achieves a lengthening of the lip along the philtral procedure achieves a lengthening of the lip along the philtral line, reconstruction of the orbicularis muscle across the cleft, line, reconstruction of the orbicularis muscle across the cleft, rotation of the displaced nasal base medially, a slight rotation of the displaced nasal base medially, a slight lengthening of the columella, and establishment of a labial lengthening of the columella, and establishment of a labial sulcus.sulcus.

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

CLEFT PALATECLEFT PALATE

The fundamental goals of the procedure are a soft The fundamental goals of the procedure are a soft palatal muscular reconstruction, a two-layer flaps, palatal muscular reconstruction, a two-layer flaps, and bilateral vermilion tissue converge to form the and bilateral vermilion tissue converge to form the bilateral (nasal and oral) lining closure of both hard bilateral (nasal and oral) lining closure of both hard and soft palatal defects, and adequate palatal and soft palatal defects, and adequate palatal length. Although there remains no uniform palatal length. Although there remains no uniform palatal repair technique or approach, the operation may be repair technique or approach, the operation may be conceived as three basic types: straight-line closure, conceived as three basic types: straight-line closure, V-Y lengthening, or Z-plasty rearrangement.V-Y lengthening, or Z-plasty rearrangement.

Opening the mucosa along the cleft edges, Opening the mucosa along the cleft edges, mobilizing tissue at the subperiosteal level, and mobilizing tissue at the subperiosteal level, and mobilizing of the palatal flaps medially enables a mobilizing of the palatal flaps medially enables a straight-line repair to be completed in two layers. straight-line repair to be completed in two layers. This technique, known as the von Langenbeck This technique, known as the von Langenbeck repair, is straightforward but may not provide repair, is straightforward but may not provide adequate soft palatal length. adequate soft palatal length.

PREAURICULAR PITS, PREAURICULAR PITS, SINUSES AND ACCESSORY SINUSES AND ACCESSORY AURICLESAURICLES

Preauricular sinuses most ascending helical rim. Most Preauricular sinuses most ascending helical rim. Most of these lesions remain asymptomatic, but when of these lesions remain asymptomatic, but when problematic frequently present with drainage which problematic frequently present with drainage which may occur spontaneously or following infection.may occur spontaneously or following infection.

Infection should be treated in the first instance with Infection should be treated in the first instance with oral or i.v. antibiotics and following recovery, the oral or i.v. antibiotics and following recovery, the sinus tract should be excised. Infected lesions may sinus tract should be excised. Infected lesions may need to be incised and drained before definitive need to be incised and drained before definitive therapy, which in this context is complicated by a therapy, which in this context is complicated by a higher recurrence rate.higher recurrence rate.

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

Accessory auricles are situated in the line of fusion between maxillary an mandibular processes of the first branchial arch and are managed by complete excision

Surgery can be safely postponed until after infancy.

RANULARANULA

Ranula is a term applied to a Ranula is a term applied to a retention cyst of the sublingual retention cyst of the sublingual gland. It results from partial gland. It results from partial obstruction of the sublingual obstruction of the sublingual salivary duct leading to dilatation salivary duct leading to dilatation of the more proximal duct. The cyst of the more proximal duct. The cyst is round or oval in shape and is round or oval in shape and located beneath the tongue. It may located beneath the tongue. It may occur congenitally and be evident occur congenitally and be evident on antenatal ultrasound scans. on antenatal ultrasound scans. When large they interfere with When large they interfere with respiration and feeding. respiration and feeding.

Treatment of ranula consists of Treatment of ranula consists of marsupialization of the cyst.marsupialization of the cyst.

THYREOGLOSSAL THYREOGLOSSAL CYSTSCYSTS

ETIOLOGYETIOLOGY – – The thyroid anlage (primordium) is a part of the second branchial arch located in the midline floor of the pharynx. It descents as a pouch from the foramen cecum down to the neck during the third week of foetal live passing anterior, through or posterior to the hyoid bone. The cells differentiate at their final position into the thyroid gland anterior to the thyroid cartilage . If duct cells persist they can form a cyst or a sinus, which is connected to the foramen cecum at the base of the tongue, but rarely a fistula with an external opening in the middle of the neck, only after perforation of a cyst.

DIAGNOSIS AND DIFFERENTIALDIAGNOSIS AND DIFFERENTIALDIAGNOSISDIAGNOSIS

Thyreoglossal duct cysts are found in 60% of the cases in the 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 or even sudden infant deathwhen located at the base of the tongue.- During palpation uninfected cysts are often ballotableand 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

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

thyroid,- dermoid cysts,- lipomas,- sebaceous cysts,- submental lymphadenitis,- thymus cysts or tumor.

TherapyTherapySurgery 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 duct ends at the bone. In order to resect the hyoid bone, the upper and lower rim have to be freed from the straight neck muscles – omohyoid and sternohyoid muscle – and the middle 1–2 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 to the base of the tongue including high ligature of the fistula.If the pathology presents primarily as an abscess, antibiotic therapy and a horizontal incision are recommended.

CONGENITAL VASCULAR CONGENITAL VASCULAR LESIONSLESIONS

1.VASCULAR TUMORSHAEMANGIOMAS- These lesions form from endothelial cells in the walls of capillaries.They are flat or absent at birth, but in the first few weeks of life they undergo a rapid proliferation. This is apparently in response to secretion of a growth factor known as alphafibroblast- derived growth factor. After 9–12 months, they enter a stationary phase without growth before involuting at age three to five years. Involution as late as 12 years has been reported. Cutaneous lesions are often red, as they are superficial and involve the skin (the traditional ‘strawberry naevus’).The lesions, however, may be placed more deeply and may not discolour the skin at all.The conventional approach to haemangiomas is simply to observe the lesion and await spontaneous involution. The exception is those lesions that impinge on function, such as vision or airway, which merit intervention.

Haemangiomas often reduce in size when treated with corticosteroids. This effect may be temporary. If treatment is required, then a choice must be made between prolonged steroid treatment and surgical intervention. Prolonged steroid treatment may have major side effects, with weight gain, hypertension and all the features of Cushing syndrome.Oral prednisone is used at a dose of 2 to 3 mg/kg/day. Doses up to 5 mg/kg/day have been administered for life-threatening complications with large hemangiomas causing airway obstruction or heart failure. The overall response rate is 80% to 90%, with initial improvement in the color and tension of the mass usually noted within 1 week.Intralesional corticosteroids are used for hemangiomas that cause local deformity or 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) are typically given at intervals of 6 to 8 weeks.Surgical excision will leave a scar. Indications are the following: (1) when it is obvious that resection would be necessary sooner or later; (2) when the surgical scar would be identical, regardless of timing of operation; and (3) when the surgical scar is easily hidden.Finally, for the difficult-to-treat and life-threatening large hemangiomas, especially in the liver, angiographic embolization may be required to manage high-output cardiac failure. There is an emerging role for laser treatment of haemangiomas.

TUFTED ANGIOMA AND KAPOSIFORM HEMANGIOENDOTHELIOMA-Both types of tumor are typically present at birth. Unlike infantile hemangioma, KHE and TA 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 . Ecchymosis appears over and around the tumor in association with generalized petechiae and may falsely raise concern for child abuse. Thrombocytopenia unresponsive to platelet transfusion can be profound (<10,000 mm3), but coagulation values (partial thromboplastin time and prothrombin time) are normal to mildly elevated. The child with Kasabach-Merritt thrombocytopenia is at risk for intracranial, pleural-pulmonic, intraperitoneal, or gastrointestinal hemorrhage with an associated mortality of 20–30%.

VASCULAR VASCULAR MALFORMATIONSMALFORMATIONS

Vascular malformations are localized or diffused errors of Vascular malformations are localized or diffused errors of embryonic development which may affect any segment of the embryonic development which may affect any segment of the vascular tree including arterial, venous, capillary, and lymphatic vascular tree including arterial, venous, capillary, and lymphatic vessels. It is useful to subcategorize vascular malformations on the vessels. It is useful to subcategorize vascular malformations on the predominant type of channel abnormality and fl ow characteristics.predominant type of channel abnormality and fl ow characteristics.

According to this distinction, two major categories exist: (1) slow-fl According to this distinction, two major categories exist: (1) slow-fl ow anomalies (capillary malformations, lymphatic malformations, ow anomalies (capillary malformations, lymphatic malformations, and venous malformations) and (2) fast-fl ow anomalies (arterial and venous malformations) and (2) fast-fl ow anomalies (arterial malformations [e.g. aneurysm, coarctation, ectasia, and stenosis], malformations [e.g. aneurysm, coarctation, ectasia, and stenosis], AVMs, and arteriovenous fistulasAVMs, and arteriovenous fistulas

Capillary MalformationCapillary Malformation

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

Lymphatic Lymphatic MalformationMalformation

Historically termed lymphangioma or cystic hygroma, slow-fl ow vascular anomalies of the lymphatic system consist of localized or diffuse malformations of lymphatic channels best characterized as microcystic, macrocystic or both. Lymphatic malformations (LMs) most commonly appear as ballotable masses with normal overlying skin, although a blue hue may result if large underlying cysts are present. The two strategies for treating lymphatic anomalies are sclerotherapy and surgical resection. Sclerotherapy works through obliteration of the lymphatic lumen byendothelial destruction with subsequent sclerosis/fibrosis. Ethanol is widely considered 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 is necessary 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 as necessary.

Venous MalformationVenous Malformation

Venous malformations are the most common of all vascular anomalies and are frequently 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 small cutaneous or oromucosal VMs, injection with 1–3% 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 some locations, staged subtotal surgical removal can be accomplished without preoperative sclerotherapy.

Arteriovenous Arteriovenous MalformationsMalformations

Most often arteriovenous malformations are latent during infancy and childhood and expand during adolescence manifesting as a warm, pink patch in the skinand an underlying thrill or bruit. Later, cutaneous consequences may include ischemic changes, ulceration, pain and intermittent bleeding. The usual strategy is arterial embolization for the temporaryocclusion of the nidus 24–72 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 resectedcompletely.