J Korean Neurosurg Soc.  2015 Jul;58(1):79-82. 10.3340/jkns.2015.58.1.79.

Cranioplasty Using a Modified Split Calvarial Graft Technique in Cleidocranial Dysplasia

• Jung YT
• Cho JI
• Lee SP

• KMID: 2067110
• DOI: http://doi.org/10.3340/jkns.2015.58.1.79

Abstract

Cleidocranial dysplasia is a well-documented rare autosomal dominant skeletal dysplasia characterized by hypoplastic/aplastic clavicles, brachycephalic skull, patent sutures and fontanelles, midface hypoplasia, and abnormalities of dentition. Patients with cleidocranial dysplasia often complain about undesirable esthetic appearance of their forehead and skull. Notwithstanding many studies of molecular, genetics and skeletal abnormalities of this congenial disorder, there have been very few written reports of cranioplasty involving cleidocranial dysplasia. Thus, we report a rare case of successful cranioplasty using a modified split calvarial graft technique in patient with cleidocranial dysplasia.

Keyword

Cleidocranial dysplasia; Congenital calvarial defect; Cranioplasty; Split calvarial graft

MeSH Terms

Clavicle
Cleidocranial Dysplasia*
Dentition
Forehead
Genetics
Humans
Skull
Sutures
Transplants*

Figure

• 

  Fig. 1 Note a large head with a midline forehead groove, including a depression of the anterior fontanelle and a depressed nasal bridge, some degree of hypertelorism and a small maxilla (A). A three-dimensional computed tomographic image demonstrating an opened anterior fontanelle, sagittal and metopic suture. Note the high and narrow orbital openings, hypoplasia of the nasal bone and the anteriorly-inclined mandible (B). A chest radiography showing a cone-shaped thorax and hypoplasia of the bilateral clavicles (C).

• 

  Fig. 2 The midline calvarial defect areas on the metopic suture and the anterior fontanelle are exposed (A). The bilateral frontal bossing areas are split into the outer and inner table flaps with a thin cutting tool (Midas Rex Legend EHS Stylus High-Speed Surgical Drill, Medtronics). This thin cutting tool can minimize bone losses while splitting the craniotomy flap. The outer table flaps are gained for craniotomy of the calvarial defect area as autografts (B). The bone flour is collected with a bone collector (a widely used generalized sputum collector which was connected to the intraoperative closed suction system) during bone sawing (C and D).

• 

  Fig. 3 Note the multiple rectangular-shaped outer tables are put on to the skull defect areas. The areas filled-up with bone flaps and bone flour are covered with a tailored titanium mesh and low profile screws (A). The obtained bone flour and hydroxyapatite cement are molded together in the calvarial defect site. The margins of the bone harvested area are smoothly drilled out with a high speed drill and filled up with hydroxyapatite cement and bone flours for cosmesis (B).

• 

  Fig. 4 A post operative frontal photograph of patient. Note the even and flat forehead compared with the preoperative one (A). On the 6 month follow up brain computed tomographic scan demonstrates the bone fusion is noticeable (B).

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