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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_537_Библиотеки_им_академика_М_И_Перельмана
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MRI imaging is indicated, and CT should be performed only when
necessary to avoid early radiation exposure.
Craniosynostosis has a variable presentation depending on the site of
premature closure. Premature sagittal suture closure is most common,
followed by metopic or coronal insults. Lambdoid craniosynostosis is
considerably rare, occurring in 5% or fewer cases [74, 80]. Various areas of
early fusion and resulting deformities are illustrated in the images in Figs.
10, 11, 12 and 13 [81]:
Fig. 10 Scaphocephaly with early sagittal suture fusion [81]
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Fig. 11 Bradycephaly with bicoronal suture deformity [81]
Fig. 12 Anterior plagiocephaly with unicoronal suture deformity [81]
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Fig. 13 Trigonocephaly with metopic suture deformity [81]
Patients presenting with syndromic craniosynostosis require a more
flexible approach to reconstruction, depending on the coexisting defects and
their relative urgency. Similar surgical reconstruction timeline is indicated
with syndromic and non-syndromic synostosis, barring any patient-specific
variations. The presence of facial microsomia (maxillary or midfacial
hypoplasias), hypertelorism (overly projected interocular structures), ear
displacement, and/or beaked nose are a few common syndromic symptoms
to be aware of when assessing patients [82–86].
The Glasglow protocol indicates repair of metopic, unicoronal, and
bicoronal synostosis at 10–14months, using fronto-orbital advancement
(unilateral or bilateral) and anterior remodeling. Bicoronal synostosis may
additionally be repaired with posterior vault distraction (also known as
helmet therapy), which involves external stabilization of the internal
osseous structures and continuous externally applied forces which maintain
adequate space between the bones as they grow normally. Sagittal
synostosis can be corrected earlier (5–7months) with total vault
remodeling, and reconstruction is recommended before 6months whenever
possible [81, 87]. It should be noted that surgical repair is contraindicated
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for patients younger than 3months of age due to the anesthesiologicassociated risks.
4.3 Surgical Techniques
Prior to surgery, it is recommended to take additional precautions with
infants and younger children due to their increased risk of blood loss and
lower capacity for thermoregulation. Infants are usually indicated for blood
transfusion if a lengthy operating time is estimated, and arterial and venous
lines are placed, in addition to bladder catheterization and internal
temperature monitoring. Prophylactic antibiotics and steroids are
administered as indicated. Additional caution is taken to protect the eyes,
usually with ophthalmic ointment and clear plastic dressings or corneal
shields. Tarsorrhaphy sutures are placed, if necessary, as an alternative
protective measure. The scalp is prepared preoperatively with hair removal
at the incision site (shaving) and the skin on the face and scalp is cleaned
with povodione-iodine and antiseptic scrub solution [88–90].
Except for occipital deformities, most synostosis reconstruction
maneuvers allow the patient to remain supine throughout the procedure.
Diluted vasoconstricting agents (preferably 1:200,000 or 1:400,000
epinephrine) are injected prior to incision to minimize blood loss and
improve visibility. The use of tranexamic and aminocaproic acid as
antifibrinolytics are well utilized in the maintenance of blood clotting and
are shown to be effective in reducing blood loss during craniosynostosis
repair [88–90].
Previously described techniques for correction of craniosynostosis
include suturectomy (removal of bony sutures) with additional
morselization of incised bone for pansynostosis. However, these approaches
are relatively outdated and do not provide patients with permanent
correction [91, 92]. Endoscopic techniques have been documented in the
literature and are shown to be well utilized in the repair of craniosynostosis
[93–95]. With the endoscopic approach, orthotic molding is used to
maintain the distance between separated cranial bones via tension from an
external helmet (scaffold) on internally fastened pins. Interested readers are
encouraged to investigate the surgical techniques used in endoscopic repair
using the articles cited here [96, 97].
It is important to note that younger patients (3–4months of age) are the
ideal candidates for endoscopic repair, and it is mainly performed for
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individuals less than 6months of age [98, 99]. Patients older than 6months
are indicated for open cranial vault reconstruction. Open surgical techniques
for the common craniosynostosis malformations are described below.
Individuals presenting with trigonocephaly, bradycephaly, and anterior
plagiocephaly are all affected by excessive anterior constriction caused by
premature fusion of metopic and/or coronal sutures. This anterior
constriction is repaired using the same concepts with open reconstruction of
all three variations. These are detailed in the bullet points below, for ease of
comparison with scaphocephaly repair discussed next [82, 100]:
Bicoronal incisions are made well into the hair-bearing scalp through
which the cutaneous tissues are dissected from underlying bone. The
dissection should be discontinued just before the superior orbit.
Bur holes drilled along preplanned markings are made to allow for
osteotomy access.
The frontal bone is removed completely, along with the anomalous
suture, using an osteotome or Piezotome.
Frontal bandeau is removed with direct visualization through the superior
orbit, and removal should be performed using cuts in an outward
direction from the anterior cranial fossa.
The cranial bones are remodeled as necessary and secured in the new
position, using polydioxanone or dissolvable plates.
Patients who present with scaphocephaly tend to have an elongated
cranial shape and lateral constriction, secondary to premature fusion of the
longitudinal sagittal suture. This anomaly requires a different surgical
approach, which is detailed in the bullet points below [82, 100]:
A midcranial incision is made within the hairline to the calvarium,
through which the soft tissues are dissected to the eyebrows anteriorly
and occipital base posteriorly.
Excess posterior occipital bone is removed, and the remaining osseous
tissues are sliced centrifugally into rectangular segments (barrel staving)
from the open occipital calvarium.
Fused sagittal sutures are removed with osteotomy, and longitudinal
barrel staving is performed as far anteriorly as possible into the parietal
bone.
The occipital bone is secured in place, and the patient is repositioned in
the supine direction for frontal remodeling.
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Once supine, the anterior portion of fused sagittal suture is removed, and
barrel staving will be completed from the anterior aspect.
The frontal bone is secured in place, at which point any bony gaps can be
filled in as needed with polydioxanone or dissolvable plates.
4.4 Complications and Outcomes
When repair is not initiated, untreated craniosynostosis can restrict brain
development, which results in increased intracranial pressure (ICP),
developmental delays, and tissue damage. Generally, individuals with
sagittal synostosis have a lower risk of intellectual disability compared with
other synostoses (metopic, unicoronal, lambdoid). Additional consideration
should be placed on education accessibility and symptom management in
these cases [75, 99].
The endoscopic approach greatly reduces the risk of bleeding and
hematoma formation when compared to open techniques. Overall, the most
common surgical complication is postoperative hyperthermia, although
there are additional risks of seizures, cerebrospinal fluid leakage, and severe
infection (meningitis). Careful technique is important to avoid aggressive
movement and vascular damage, and it is especially important to prepare
for blood transfusion with such a young patient [74, 75].
Long term follow-up is required for patients with syndromic
craniosynostosis, as they may require multiple operations in the future to
maintain the reconstructed calvarium [75]. Aside from these complications,
this procedure is relatively successful and may be the only required therapy
in some cases. It should be noted that this does not usually apply to
syndromic cases, which frequently require additional treatments for
associated malformations.
The long-term aesthetic defects of craniosynostosis are well published
in the literature, with patients experiencing treatment-related scarring, facial
asymmetry, and atypical head shape. These aesthetic impacts are often
more severe in syndromic cases. Patients are described to have a less
pleasing appearance, and this has been associated with reported social
difficulties in romantic relationships through adulthood [101].
Despite the previously mentioned psychosocial impact, craniosynostosis
patients do not express more severe depressive symptoms when compared
to their unaffected counterparts. Additional exploration indicates a lack of
correlation between facial asymmetry and low self-esteem in adulthood,
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which proposes a lower than assumed psychological impact [102] (Figs. 14,
15 and 16).
Fig. 14 The figure above shows a patient with syndromic craniosynostosis secondary to Crouzon
syndrome at various time points after cranial remodeling [81]
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Fig. 15 Various views of a young male patient with metopic synostosis are shown, which were taken
prior to cranial remodeling interventions [81]
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Fig. 16 The postoperative appearance of the patient is shown in Fig 16-c below [81]
References
1. Wan, D. C., Kwan, M. D., Kumar, A., et al. (2009). Craniofacial surgery, from past pioneers to
future promise. Journal of Oral and Maxillofacial Surgery, 8, 348–356.
2. Larsen, W. J. (1993). Human Embryology. Churchill Livingstone.
3. Lin, I. C., Slemp, A. E., Hwang, C., et al. (2004). Immunolocalization of androgen receptor in
the developing craniofacial skeleton. The Journal of Craniofacial Surgery, 15, 922–927.
discussion 928–929.
4. Whitaker, L. A., Pashayan, H., & Reichman, J. (1981). A proposed new classification of
craniofacial anomalies. The Cleft Palate Journal, 18, 161–176.
5. Enlow, D. H. (1990). Facial growth (3rd ed.). Saunders Philadelphia.
6.
Ansari, A., & Bordoni, B. (2022). Embryology, face. In StatPearls [internet]. StatPearls
https://t.me/medicina_free
Publishing.
7. Som, P. M., & Naidich, T. P. (2013). Illustrated review of the embryology and development of
the facial region, part 1: Early face and lateral nasal cavities. American Journal of
Neuroradiology, 34(12), 2233–2240.
8. Cuervo, R., Valencia, C., Chandraratna, R. A., & Covarrubias, L. (2002). Programmed cell
death is required for palate shelf fusion and is regulated by retinoic acid. Developmental
Biology, 245, 145–156.
9. Martinez-Alvarez, C., Tudela, C., Perez-Miguelsanz, J., et al. (2000). Medial edge epithelial
cell fate during palatal fusion. Developmental Biology, 220, 343–357.
10. Rice, D. P. (2005). Craniofacial anomalies: From development to molecular pathogenesis.
Current Molecular Medicine, 5, 699–722.
11. Shuler, C. F., Guo, Y., Majumder, A., & Luo, R. Y. (1991). Molecular and morphologic changes
during the epithelial-mesenchymal transformation of palatal shelf medial edge epithelium in
vitro. The International Journal of Developmental Biology, 35, 463–472.
12. Vyas, T., Gupta, P., Kumar, S., Gupta, R., Gupta, T., & Singh, H. P. (2020). Cleft of lip and
palate: A review. Journal of Family Medicine and Primary Care, 9(6), 2621–2625. https:// doi.
org/ 10. 4103/ jfmpc. jfmpc_ 472_ 20
[Crossref]
13. Kohli, S. S., & Kohli, V. S. (2012). A comprehensive review of the genetic basis of cleft lip and
palate. Journal of Oral and Maxillofacial Pathology: JOMFP, 16(1), 64–72. https:// doi. org/ 10.
4103/ 0973-029X. 92976
[Crossref]
14. Leslie, E. J., & Marazita, M. L. (2013). Genetics of cleft lip and cleft palate. American Journal
of Medical Genetics. Part C, Seminars in Medical Genetics, 163C(4), 246–258. https:// doi. org/
10. 1002/ ajmg. c. 31381
[Crossref]
15. Trokovic, N., Trokovic, R., Mai, P., & Partanen, J. (2003). Fgfr1 regulates patterning of the
pharyngeal region. Genes & Development, 17, 141–153.
16. Dode, C., Levilliers, J., Dupont, J. M., et al. (2003). Loss-of-function mutations in FGFR1
cause autosomal dominant Kallmann syndrome. Nature Genetics, 33, 463–465.
17. Moss, M. L. (1959). The pathogenesis of premature cranial synostosis in man. Acta Anatomica,
37, 351–370.
18. Virchow, R. (1989). Cellular pathology. As based upon physiological and pathological
histology. Lecture XVI–atheromatous affection of arteries. 1858. Nutrition Reviews, 47, 23–25.
19. Jabs, E. W., Muller, U., Li, X., et al. (1993). A mutation in the homeodomain of the human
MSX2 gene in a family affected with autosomal dominant craniosynostosis. Cell, 75, 443–450.
20.
Warman, M. L., Mulliken, J. B., Hayward, P. G., & Muller, U. (1993). Newly recognized
autosomal dominant disorder with craniosynostosis. American Journal of Medical Genetics, 46,
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