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Six days after hematoma removal and left blepharorrhaphy. Chemosis and proptosis resolved gradually after evacuating the hematoma and performing a left blepharorrhaphy.
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Rationale:
Crouzon syndrome is a craniofacial malformation caused by premature fusion of fibrous sutures in infants. It is one of the most common craniosynostosis syndromes, and surgery is the only effective treatment for correcting it. Postoperative complications such as encephalocele, infections, hematoma have been reported. We herein report a c...
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Context 1
... to incise the left upper eyelid and orbital periosteum on the 4th day postsurgery (Fig. 3). The hematoma was under the orbital periosteum and measured approximately 2 Â 2 Â 2 cm. The hematoma applied pressure on the left eyeball, occupied the orbital volume, and aggravated the exophthalmos. After hematoma evacuation and left blepharorrhaphy (Fig. 4), the chemosis and proptosis gradually resolved. The patient was discharged uneventfully 10 days after the remolding surgery and after receiving hemostasis and antiinfective agents as well as nutritional treatment. No other complications occurred during the follow-up period (12 months postsurgery), and CT scans confirmed that the ...
Citations
... Crouzon syndrome is a rare genetic disorder affecting craniofacial structures due to a mutation in one of the FGFR genes (FGFR2 and FGFR3). 1 The premature fusion of cranial sutures, including coronal and frontosphenoidal sutures and sphenoethmoidal synchondrosis in infants, causes the syndrome. 2,3 It affects about 1.6 per 100,000 people in the general population. 1 This disorder can cause several craniofacial malformations such as proptosis, hypertelorism, and midface hypoplasia, as well as the relative prognathism of the mandible, orbital hypoplasia, strabismus, crossbite, and open bite. 1,3 The clinical findings of this syndrome can be categorized into extraoral signs (e.g., high and large forehead, flattening of the occipital region, maxillary hypoplasia, exorbitism with hypertelorism, and maxillary hypoplasia with mandibular prognathism) and intraoral signs (e.g., Class III occlusion, the maxillary dental arch in V shape, spaced teeth, and congenital cleft in the palate). ...
Key Clinical Message
Vigilant monitoring for postoperative complications, including bleeding and dysrhythmia, is crucial in patients with craniosynostosis syndromes like Crouzon syndrome undergoing craniofacial surgery, with a thorough evaluation, including coagulation tests, assisting in diagnosing underlying conditions such as von Willebrand disease subtype 1 to inform appropriate management strategies.
Abstract
Crouzon syndrome is a rare genetic disorder affecting craniofacial structures. Its etiology is the premature fusion of cranial sutures. The LeFort III advancement surgery is a commonly used approach to correct malformations related to midface hypoplasia. Complications following surgical treatment of craniosynostosis and craniofacial syndromes can include both intracranial and extracranial problems. Reporting of this syndrome and the surgery complications, in addition to consideration of other differential diagnoses, can help improve the treatment plan and surgery outcomes. The aim of the article is to report a 14‐year‐old female with Crouzon syndrome who underwent the modified LeFort III osteotomy and developed unexpected massive bleeding during the surgery. Post‐surgery, she experienced complications including dysrhythmia, hypothermia, and cyanosis. Treatment included fluid therapy, blood transfusions, and antibiotic therapy for suspected septic shock. Differential diagnosis was disseminated intravascular coagulation but was ruled out. Post‐discharge, coagulation tests suggested von Willebrand disease subtype 1 as the diagnosis. Excessive bleeding during surgery for craniosynostosis syndromes is a significant and concerning issue in the surgical management of Crouzon syndrome. For patients with von Willebrand disease who are candidates for elective surgeries, von Willebrand factor concentrates or recombinant von Willebrand factor can be used.
... Craniofacial anomalies are frequently present at birth and might get worse over time. An infant with CS may present mildly, with a slight midface deficiency, or more severely, with fused numerous cranial sutures, and eye issues (16,17). Acute respiratory distress can be brought on by upper airway obstruction (18,19). ...
Crouzon syndrome(CS) is the most common craniosynostosis condition.This report presents a rare case of a 25-year-old male CS patient referred for orthodontic treatment with the chief complaint of severe irregularities in the arrangement of teeth and abnormal facial appearance.The clinical,cephalometric features and initial orthodontic management of this patient are discussed
