1. INTRODUCTION - Cleft lip and palate are the most common congenital craniofacial anomalies in humans, affecting approximately 1 in 700 live births 2.EMBRYOLOGy: It is generally accepted that facial tissues, including the lip and palate, arise from neural crest cells (6). These cells migrate from their original positions at the margins of the neural fold, providing the mesenchyme for the paired maxillary and mandibular processes (which are in turn derivatives of the first and second branchial arches), and the central frontonasal process. Bilateral nasal pits develop at the inferolateral aspects on either side of the frontonasal prominence. The tissues on the sides of the nasal pits become the medial and lateral nasal 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 foramen The 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 Lip .

Complete Unilateral Cleft Lip .

Bilateral Cleft Lip .

10 grams of hemoglobin. The concept involves an inferior rotation of the medial into the subcolumellar space to join with the medial lip segment. The historic rule of 10s is a good guideline: 10 weeks of age. . a slight lengthening of the columella. The cleft palate is traditionally repaired after the lip and may be done between 9 and 15 months of age depending on the surgeon's philosophy. The Millard rotation-advancement cleft lip repair. Early surgical efforts are purported to result in better speech function. SURGICAL CLEFT CORRECTION      The cleft lip is always repaired first at 3 to 4 months of age. reconstruction of the orbicularis muscle across the cleft. and 10 pounds of weight. and establishment of a labial sulcus. has become the most commonly performed procedure for repair of the unilateral cleft lip.4. rotation of the displaced nasal base medially. This procedure achieves a lengthening of the lip along the philtral line. however.

The bilateral cleft lip represents more than just a doubling of the problem of the unilateral cleft lip. whether to adhese the lip elements before a definitive lip repair. Decisions regarding repair of the bilateral cleft include whether to repair both lip clefts simultaneously or in stages. splaying of the alar cartilages and nasal bases. and protrusion of the underlying premaxilla not only make the initial lip repair different from a unilateral cleft but also ensure that subsequent operations will be needed. . The lack of a columella. and how to manage the protrusive premaxilla.



known as the von Langenbeck repair.The fundamental goals of the procedure are a soft palatal muscular reconstruction. and adequate palatal length. and mobilizing of the palatal flaps medially enables a straight-line repair to be completed in two layers. and bilateral vermilion tissue converge to form the bilateral (nasal and oral) lining closure of both hard and soft palatal defects. mobilizing tissue at the subperiosteal level. is straightforward but may not provide adequate soft palatal length.  Opening the mucosa along the cleft edges. the operation may be conceived as three basic types: straight-line closure. This technique. Although there remains no uniform palatal repair technique or approach. V-Y lengthening. a two-layer flaps. or Z-plasty rearrangement. .


Infected lesions may need to be incised and drained before definitive therapy.PREAURICULAR PITS. Magnetic resonance imaging should be considered in all patients with a suspected first branchial cleft anomaly. the sinus tract should be excised. SINUSES AND ACCESSORY AURICLES    Preauricular sinuses most ascending helical rim. which in this context is complicated by a higher recurrence rate.v. antibiotics and following recovery. Infection should be treated in the first instance with oral or i. but when problematic frequently present with drainage which may occur spontaneously or following infection. Most of these lesions remain asymptomatic. .


  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. .

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

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


-In the latter case they can cause respiratory distress or even sudden infant death when located at the base of the tongue.DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS Thyreoglossal duct cysts are found in 60% of the cases in the midline at or below the hyoid bone. but not up or down.During palpation uninfected cysts are often ballotable and can be moved slightly from side to side. Due to their origin from the foramen cecum the thyroid cysts move upward during swallowing or when the tongue protrudes (this clinical sign is difficult to observe in small infants . . -24% are located above the hyoid and 8% intralingual.

lipomas. .submental lymphadenitis. dermoid cysts.complete ectopic thyroid gland or parts of the thyroid.sebaceous cysts.DIFFERENTIAL DIAGNOSIS The differential diagnosis includes : . .thymus cysts or tumor. . . .

Therapy Surgery of the thyreoglossal cyst or duct must always include resection of the middle part of the hyoid corpus whether or not the surgeon has the feeling that the duct ends at the 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 order to resect the hyoid bone. antibiotic therapy and a horizontal incision are recommended. If the pathology presents primarily as an abscess. . 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.


.These lesions form from endothelial cells in the walls of capillaries. they enter a stationary phase without growth before involuting at age three to five years.CONGENITAL VASCULAR LESIONS 1. such as vision or airway. They are flat or absent at birth. The exception is those lesions that impinge on function. This is apparently in response to secretion of a growth factor known as alphafibroblast. The lesions. Involution as late as 12 years has been reported.VASCULAR TUMORS HAEMANGIOMAS. which merit intervention.derived growth factor. but in the first few weeks of life they undergo a rapid proliferation. After 9–12 months. 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. as they are superficial and involve the skin (the traditional „strawberry naevus‟). Cutaneous lesions are often red.

If treatment is required. Prolonged steroid treatment may have major side effects. Intralesional corticosteroids are used for hemangiomas that cause local deformity or ulceration. regardless of timing of operation. .Haemangiomas often reduce in size when treated with corticosteroids.41 A total 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. especially in the liver. Indications are the following: (1) when it is obvious that resection would be necessary sooner or later. hypertension and all the features of Cushing syndrome. There is an emerging role for laser treatment of haemangiomas. or lip. (2) when the surgical scar would be identical. nose. then a choice must be made between prolonged steroid treatment and surgical intervention. cheek. This effect may be temporary. with weight gain. Oral prednisone is used at a dose of 2 to 3 mg/kg/day. Surgical excision will leave a scar. with initial improvement in the color and tension of the mass usually noted within 1 week. Doses up to 5 mg/kg/day have been administered for life-threatening complications with large hemangiomas causing airway obstruction or heart failure. and (3) when the surgical scar is easily hidden. especially for facial lesions of the eyelid. Finally. for the difficult-to-treat and life-threatening large hemangiomas. The overall response rate is 80% to 90%. angiographic embolization may be required to manage high-output cardiac failure.


Unlike infantile hemangioma. The child with Kasabach-Merritt thrombocytopenia is at risk for intracranial. or gastrointestinal hemorrhage with an associated mortality of 20–30%. . The overlying skin is deep red-purple in color. and generally involve the trunk. thigh. or retroperitoneum. Ecchymosis appears over and around the tumor in association with generalized petechiae and may falsely raise concern for child abuse. tense. shoulder.TUFTED ANGIOMA AND KAPOSIFORM HEMANGIOENDOTHELIOMA-Both types of tumor are typically present at birth. but coagulation values (partial thromboplastin time and prothrombin time) are normal to mildly elevated. Thrombocytopenia unresponsive to platelet transfusion can be profound (<10. pleuralpulmonic. intraperitoneal. and shiny . KHE and TA affect both genders equally.000 mm3). are unifocal.

aneurysm. According to this distinction. venous. ectasia. two major categories exist: (1) slow-fl ow anomalies (capillary malformations. capillary. and stenosis]. and venous malformations) and (2) fast-fl ow anomalies (arterial malformations [e. It is useful to subcategorize vascular malformations on the predominant type of channel abnormality and fl ow characteristics. and lymphatic vessels.g. AVMs.VASCULAR MALFORMATIONS   Vascular malformations are localized or diffused errors of embryonic development which may affect any segment of the vascular tree including arterial. coarctation. lymphatic malformations. and arteriovenous fistulas .

.Capillary 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 newborns with an even gender distribution.

Resection is the only way to potentially “cure” LM. The two strategies for treating lymphatic anomalies are sclerotherapy and surgical resection. slow-fl ow vascular anomalies of the lymphatic system consist of localized or diffuse malformations of lymphatic channels best characterized as microcystic. Sclerotherapy works through obliteration of the lymphatic lumen by endothelial destruction with subsequent sclerosis/fibrosis. Lymphatic malformations (LMs) most commonly appear as ballotable masses with normal overlying skin. . Ethanol is widely considered to be the most effective sclerosing agent for low flow malformations. attempt to limit blood loss. Often staged excision is necessary and total excision is often possible.Lymphatic Malformation Historically termed lymphangioma or cystic hygroma. perform as thorough a dissection as possible and to be prepared to operate as long as necessary. sodium tetradecyl sulfate and OK432. although a blue hue may result if large underlying cysts are present. Other sclerosant agents include doxycycline. macrocystic or both. In each resection a surgeon should focus on a defi ned anatomic region.


Venous Malformation Venous malformations are the most common of all vascular anomalies and are frequently misdiagnosed as hemangiomas or mislabeled “cavernous hemangiomas. injection with 1–3% sodium tetradecyl sulfate is often successful. staged subtotal surgical removal can be accomplished without preoperative sclerotherapy. soft. The typical description of a VM is of a blue.” While present at birth. For small cutaneous or oromucosal VMs. Excision of a VM is usually successful for small. they are not always immediately evident. and compressible mas. Therapy for venous malformations is sclerotherapy and surgical resection. . well localized lesions. In some locations. Venous anomalies have a propensity for recanalization and recurrence. Ethanol is also used as described for lymphatic malformations.


cutaneous consequences may include ischemic changes. Whenever possible. pain and intermittent bleeding. the lesion should be resected completely. If the arteries are tortuous or if the feeding arteries have been ligated. sclerotherapy may play a role in conjunction with local arterial and venous occlusion. ulceration. pink patch in the skin and an underlying thrill or bruit. Later.Arteriovenous Malformations Most often arteriovenous malformations are latent during infancy and childhood and expand during adolescence manifesting as a warm. The usual strategy is arterial embolization for the temporary occlusion of the nidus 24–72 h prior to surgical resection. .

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