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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_537_Библиотеки_им_академика_М_И_Перельмана

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providing an opportunity for the surgeon to simultaneously reconstruct any nasal defects. The resultant scarring is disguised within the recreated nasal philtrum using Millard’s technique. It should be noted that wider clefts are difficult to close with this technique, especially for inexperienced surgeons, and patients incur a risk of nostril stenosis as the soft tissues heal [3840].
Tennison’s triangular flap technique can be precisely pre-planned, and as such is relatively easier for inexperienced surgeons. Surgeons calculate the difference between the columellar base and Cupid’s Bow peak between the noncleft and cleft sides, which is equivalent to the cleft width. The width indicates how much lengthening of the lip is required, and two triangular flaps should be created (one on each side) when the cleft width is greater than 6 mm. In patients with cleft width <6mm, a triangular flap is created from the cleft side, which is then brought medially and sutured to the incised noncleft side. Wider clefts can be sufficiently reduced with this technique, although the results tend to be less optimal regarding aesthetic appearance. Nasal reconstruction is not accomplished within this plane, and the scar cannot be camouflaged between cosmetic subunits [41, 42] (Fig. 4).
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Fig. 4 An illustrative overview of Millard’s unilateral cleft lip repair [43]
3.3.2 Bilateral Cleft Lip
There are various techniques used for repair of the bilateral cleft lip, including two-stage procedures and one-stage procedures. Tennison’s triangular flap technique discussed previously can be utilized here as a one or two stage correction. Alternatively, Millard introduced a single-stage repair, which will be detailed here [29].
Incisions are marked along the vermilion border of the clefted lateral lip segments, which will be used to create bilateral muscle flaps containing oral mucosa and submucosa. The lip segments are dissected from the maxilla at their muscular attachments, allowing for medial advancement. At this point, the alar cartilages of the nose are sutured to the anterior nasal spine (ASA) medially to reconstruct the nostrils. Upper lip volume at the medial aspect is
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increased by excising and reattaching excess vermilion to the premaxillary tissues [44, 45].
The lateral lip flaps are fastened to the ASA just below the alar cartilages, and the orbicularis oris muscle layers are attached medially to create the perioral sphincter. After the muscles are reattached, a prolabial flap is advanced inferiorly and sutured deep to the sphincter, allowing for creation of the philtral dimple. Bilateral forked prolabial flaps are attached at the midline to create the nasal floor [44, 45].
After surgery, the incisions are cleaned with hydrogen peroxide and protected with antibiotic ointment. Elbow splints or arm restraints may be employed to prevent the patient from contaminating or causing harm to the surgical wound, and feeding should be performed with a bulb syringe to avoid suckling movement of the healing tissues. After 1week, the sutures can be removed [29].
3.3.3 Palatoplasty
Palatoplasty for isolated cleft palate repair and cleft lip/palate palatoplasty is generally performed at 9–15months of age, usually after the lip repair has been completed. There are a few techniques used, including Veau­Wardill-Killner V-Y pushback, straight line repair, and Furlow double Z­plasty. All of these techniques incorporate levator veli palatini (LVP) muscle reconstruction, which is of critical importance for its role in palatal elevation with swallowing. Additional caution is required to ensure the secure reattachment of LVP, as this helps to avoid velopharyngeal closure and resultant nasal reflux and speech defects [35, 46, 47].
The Veau-Wardill-Killner V-Y Pushback technique is performed to lengthen the palate using bilateral mucoperiosteal flaps, which are elevated and reapproximated along the midline. The nasal mucosa is left exposed and will close on its own by secondary intent [35, 47].
Straight Line Repair (Two-flap palatoplasty) uses intraoral incisions to reconstruct mucoperiosteal and mucosal flaps, which will be elevated and repositioned without lengthening the palate. Mucoperiosteal flaps on either side of the cleft are dissected from the vomer, and nasal and oral mucosal flaps are elevated away from the maxillary alveolus, leaving the soft palate intact. The flaps will be advanced posteriorly toward the soft palate and rotated medially, after which they are fixated in multiple sutured layers to the midline. LVP muscles should be dissected from the hard palate and
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reattached posteriorly to the soft palate to reconstruct the muscle sling [35,
47].
Furlow’s palatoplasty utilizes multiple palatal flap layers, allowing for recreation of the uvula with mucosal flap transposition and levator sling reconstruction with soft palatal muscle flaps. Hard palate reconstruction is accomplished using the previously discussed straight line technique. The transposition of multiple flap layers, also referred to as Z-plasty, allows for palatal lengthening [48].
After palatoplasty, patients should avoid bottle feeding until the wound is healed to prevent damage from the nipple. Breastfeeding and spoon or dropper feeding can commence four hours after the operation, and water is recommended after any meal to reduce food contamination at the suture line. Patients should be kept in a lateral or prone position while feeding in the early postoperative stages, and caregivers should monitor for aspiration, especially after isolated cleft palate repair [49, 50] (Fig. 5).
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Fig. 5 The various stages of bilateral cleft palate repair are shown in the images [51]
3.4 Outcomes and Complications
Cleft lip and palate repair involves subsequent reconstruction and intensive orthodontic work later on in life to maintain the surgical results. The patient’s age at the time of reconstruction is significant, and it is recommended to perform surgery before age 2 and/or the patient’s language acquisition [52].
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When performed in younger patients, the risk of palatine fistula development was relatively lower in some studies compared with individuals older than 2 [52]. Other studies associate greater degree of cleft laterality with fistula formation [53]. Palatine fistulas are a relatively common complication of palatoplasty and present with symptoms of nasal regurgitation, food particle lodging, and speech impairments. Fistulas increase the risk of conductive hearing loss compared with individuals that did not develop this complication [54] (Fig. 6).
Fig. 6 Development of fistula after primary cleft palate repair, indicating a secondary reconstruction for this patient [55]
The detrimental impact of cleft palate repair on the mid-facial or maxillary growth is measured by the decreased maxillary arch growth and circumference trends. It should be noted that the V-Y pushback method provides patients with relatively successful midfacial growth [56, 57].
Many patients that have undergone surgical repair of cleft lip and/or palate require subsequent follow up to assess for related functional
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impairments, including difficulty with speech and feeding, hearing loss, and poor dentition [58, 59]. Criteria used to assess postoperative outcomes are centered around the patient’s functional abilities, as well as aesthetic appearance. Earlier studies have focused on the clinician’s assessment to determine surgical success, although the psychosocial impact on the patient and their family is equally important [60]. As of 2016, the cleft lip and palate group of the ICHOM (International Consortium for Health Outcomes Measurement) introduced specific outcome measures (CLEFT-Q) to assess the patient and caregiver’s reported outcomes after surgery, which have been implemented in various care facilities to date [58, 6164].
CLEFT-Q contains parent-reported and patient-reported subsets of questions to assess facial function (eating, drinking, breathing), speech, psychosocial function, health-related quality of life, and appearance. It is used in conjunction with other surveys, including the NOSE questionnaire (Nasal Obstructive Symptom Evaluation), Intelligibility in Context Scale/ICS to assess speech difficulties, and the Child Oral Health Impact Profile-Oral Symptoms Scale (COHIP-OSS) to assess oral health [6567].
The difficulty of these surveys is related to the young age of patients and their potential inability to accurately answer the survey questions. However, there is some benefit to these assessments when used as a checklist to identify postoperative issues as opposed to determination of patient satisfaction [28] 57. From a clinician standpoint, the optimal postoperative goals of cleft lip and palate repair are to close the open communication between oral and nasal cavities and provide patients with sufficient postoperative speech ability (construction of a functional velum) [68].
Generally, about 15% of patients will experience complications, usually hypertrophic scarring with cleft lip repair and oronasal fistula with cleft palate repair. Most patients achieve satisfactory postoperative function and appearance, although the latter is much more difficult to assess [6972]. Patients who undergo cleft lip and palate repair at a younger age (less than 18months of age) generally achieve better speech function and cosmetic outcomes. On the other hand, later repairs tend to allow for increased maxillary growth despite less than ideal functional and aesthetic outcomes [72]. Figures 7 and 8 show the preoperative, intraoperative, and postoperative images of patients with complete cleft palate.
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Fig. 7 Postoperative results of complete cleft palate repair are shown in image C, with the preoperative appearance (a) and intraoperative view (b) included for reference [73]
Fig. 8 Similar to Fig. 7, perioperative images of a patient with complete cleft palate are shown in the figure [73]
4 Surgical Reconstruction of Craniosynostosis
As discussed in Sect. 4.2, craniosynostosis occurs when the cranial sutures between the skull bones fuse prematurely. The resultant perpendicular growth of the osseous tissues causes increased intracranial pressure (ICP), incurs damage to respiratory and neurologic systems, and produces visible aesthetic deformities in affected patients [74, 75].
4.1 Review of Relevant Anatomy
The skull is divided structurally into the upper calvaria and lower base, which are both composed of multiple bones attached at suture joints. The calvaria is composed of anterior frontal bone, which is fused posteriorly to bilateral parietal bones at the coronal suture. A sagittal suture fuses the paired parietal bones superiorly along the midline, and temporal bones are fused at the inferior aspect via squamosal sutures. Posteriorly, lambdoid
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sutures fix the parietal bones to the occipital bone. Cranial bones and their connecting suture joints are labeled in Fig. 9.
Fig. 9 Cranial bone anatomy [76]
Sphenoid and ethmoid bones form the skull base, along with inferior portions of the occipital, temporal, and frontal bones. The pterion is an osseous landmark significant for the articulation of sphenoid, temporal, frontal, and parietal bones, while the asterion contains bony articulations of occipital, parietal, and temporal bones [77].
4.2 Preoperative Patient Assessment
Generally, 75% of craniosynostosis cases will present nonsyndromically, although a thorough patient history and examination is warranted to rule out
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syndromic conditions and confirm diagnosis. Some of the vital information to be obtained is detailed below.
Surgeons should obtain a thorough maternal gynecological history to assess for previous in-utero exposures (teratogens) and prior complications (abnormal fetal position, intrauterine restraints), in addition to any difficulties encountered in the intrapartum period. This information should mainly be obtained for the affected child, although any available information from previous maternal gestation or birth history may be helpful in assessing the risk of future occurrence. The developmental history will also be discussed at the preoperative visit with significance placed on any delayed milestones encountered in the affected child, as well as feeding difficulty or prior diagnosis of obstructive sleep apnea (OSA). OSA presents similarly in neonates when compared to adults and can be observed as loud breathing or snoring followed by apneic periods (no breathing). The obstructive symptoms may be accompanied by daytime fatigue or failure to thrive (FTH), the former of which is somewhat difficult to assess in newborns [75, 78].
The key aspects of the physical examination are detailed in the bullet points below [75, 79]:
Visual examination: Assess for asymmetry, deformity, and head shape. Calculation of cephalic index using measured head circumference.
– Cephalic index=(maximal skull circumference * 100)/(maximal skull
length).
Palpation of the skull and facial bones.
– Assess for ridges at the suture lines, large blood vessels, indentations,
or contour deformities.
Measure and examine fontanelles. Ophthalmologic examination: Assess for papilledema (presents with ICP) and corneal exposure.
– Abnormally elevated intracranial pressure often presents in neonates
with inconsolable crying, distress and/or headbanging, which tends to worsen at night when lying flat.
Airway examination.
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