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3.4.12.9 the Temporal Bone of Patient Ip

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3.4.12.9 the Temporal Bone of Patient Ip 3.4.12.9 The Temporal Bone of patient Ip Distances (Figure 3.30(n)): Squamous Temporal Bone: The squamous temporal bone was not measurable in this child due to lack of visibility of the asterion (as), sphenion (spt) and the stylomastoid foramen (smÐ. Extemal Auditory Meatus: The configuration of the external auditory meatus was abnormal and asymmetrical, with increased distances recorded on both sides (eampl-pol, pol-eamal, eamir- eampr, eamar-eamir). Zygomatic Process: The length of the zygomatic arch (ztl-aul, ztr-aur) was decreased bilaterally. The articular fossa height (afl-ael, afr-aer) was normal, however, posteriorly the left EAM- articular fossa length (eamal-afl) was increased. Petrous Temporal Bone (Figure 3.30(o)): The prominence of the mastoid process (mal-jfl1, mar-jflr) was increased bilaterally compared with the experimental standard. The distances of the temporal bone showed the right jugular foramen to be narrowed (flr-jfmr) with an similar tendency on the left. The inferior petrous temporo-occipital suture (fml-ptsl, jfrnr-ptsr) was increased in length. Dimensions (Figure 3.30(o)): The petrous temporal ridge distance (petal-petpl, petar-petpr) was increased bilaterally. The dimensions between the temporal bones was increased between the external auditory meatus (pol-por). The angles of the auditory canal (pol-iamViamr-por), the petrous temporal bone angles (petpl-petaVpetar-petpr) and the zygoma projection (petal-aul-ztl, petar-aur-ztr) were not significantly different from the experimental standard. Discussion: Bony distortion was found at the external auditory meatus and the zygomatic arch which was reduced in length. The jugular foramen was nanowed while the temporal occipital suture medially and the distance to the mastoid laterally were increased. The overall length of the petrous ridge was also increased. This was reflected in the significantly increased distances between the external auditory canals. The distances between the internal auditory meatus and jugular foramen were relatively normal. There appeared to be lengthening of the petrous temporal bone at the expense of narrowing of the jugular foramen and this would be consistent with sutural involvement and compensatory bone lengthening. Page 276 Crouzon Syndrome Figure 3.30(n) Z Scores of the Distances of the Temporal Bone for Patient IP compared with the 2 Year Old Experimental Standard Z score Squamous External Auditory Meatus Zygomatic Process t4 L R L R L R t2 10 8 6 4 2 0 a 4 -6 LL Ê!! eØdç '=Àôd= Èo d ã çÐ!Èi çoÈÊ- *f E E Ê. b É ÀÉ tr = a:'=d f 133f f f 3_3. f À t s ås ü q,õäö9 dçlJe k oØà-ñ o d Ed 9Ë* Ñ N c Ri= tr= d ã E 9Ë É trtroÀtr ã:E È o ñd trtroÈtr o oo oodõ Figure 3.30(o) Z Scores of the Measurements of the Temporal Bone for Patient IP compared with the 2 Year Old Experimental Standard Z score Petrous Dimensions Angles 8.00 L R L R 6.00 4.00 200 0.00 -2.00 -4.00 dd.=ó d a .- d o.ã .aEzz e +ñ ñ 4ti+P BoäEä+'Edètli¿ !dr lñol LdùJ tr.'dd -B-x+ Ë çod É ã 3 Ë E 3.Ê 3" 3- '- .f?Ee3-8. KP õ. Chapter 3 Page 277 3.4.12.LO The Parietal Bone of patient Ip Distances: The key landmarks for the parietal bone for Patient IP were not visible due to suture fusion and could not be reliably estimated. Therefore the parietal bone was not measured and a pat[ern profile of Z scores was not generated for the parietal bone. The measurement data for the experimental standard are reported in Appendix 2. 3.4.12.11 The Occipital Bone of Patient IP Distances (Figure 3.30(p)): The medial temporo-occipital suture (inferior) (fml-ptsl, jfmr-prsr) was bilaterally significantly increased in length compared with the experimental standard. The left anterior foramen magnum distance (ba-fmll) and the right posterior foramen magnum distance (fmlr-o) were increased. The inferior spheno-occipital synchondrosis was normal (see sphenoid and cranial case sutures). Dimensions (Figure 3.30(p)): The dimensions were not significantly different from the experimental standard. Discussion: The occipital bone was involved medially along the sutures with the temporal bone and also laterally where the calvarial sutures were fused (see temporal bone). Figure 3.30(p) Z Scores of the Measurements of the Occipital Bone for patient rP compared with tlne 2 Year old Experimental standard Z score Distances 6.00 Dimensions L R 5.00 4.00 3.00 2.00 l.00 0.00 -1.00 _a -2.00 e?qË.È E i3 o -! o{ o. 'Y '1ì ;a q-i o- È =o¡o ,l Oo, oo Ø-l- !ËÊ'1". I E*EÉlEr! -o E - I Èo. tst€.- ¡- È ëË a -8.È-3È oo q: ÈÈ Page 278 Crouzon Syndrome 3.4.L2.12 The Cranial Base Sutures of Patient IP Anterior Cranial Fossa (Figure 3.30(q)): The majority of distances measured were abnormal. The spheno-ethmoid synchondrosis (es-cppl, es-cppr) was increased in length. The spheno- frontal suture in the anterior cranial fossa (cppl-spal, cppr-spar) was of normal length, while in the orbit, the suture (spal-zfsl, spar-zfsr) was reduced in length. The spheno-zygomatic suture (zfsl-gwll, zfsr-gwlr) length was increased. Middle Cranial Fossa (Figure 3.30(q)): The lateral spheno-squamous temporal suture (gwll- fisl) was increased in length on the left side. Posterior Cranial Fossa (Figure 3.30(q)): The medial temporo-occipital suture (inferior) Cfml- ptsl, jfmr-ptsr) and the occipital mastoid suture (superior) fiflI-petpl, jflr-peþr) were lengthened. The superior and inferior parts of the spheno-occipiøl synchondrosis (petal-petar, ptsl-ptsr) were normal, while the lateral part of the synchondrosis was increased in height (ptsl- petal, ptsr-petar). Discussion: The cranial base sutures were abnormal anteriorly and laterally. Medially the spheno-occipital synchondrosis was deformed. The larger number of abnormal sutural findings in this patient are reflected by the more severe clinical deformity. Figure 3.30(q) Z Scores of the Dimensions of the Cranial Base Sutures for Patient IP compared with the 2 Year Old Experimental Standard Zscore Anterior Cranial Fossa Middle Cranial Fossa Posterior Cranial Fossa 20.00 r 5,00 l0.00 5.00 0.00 -5.00 L_L-L-L !-!-L 9-r-!qL-L o.aLcdd.ØØ-J ØØdclØØ ã. È* Y+ir)a)ô,o- EÉesggsess ,h,h'^XEã-lIFrl-ú F A â\ \ ,åLO.O.r..r U O ñcúF.F.-L= * BÉ EöOÞO¿!r.Ekç E + *Z á iiågÊþËå-iÞ:rÈÈ,¡EËSä: s F F * ää- B.-o.o- Chopter 3 Pctge 279 3.4.12.13 The craniofacial Dimensions and Angles of patient rp Dimensions and Angles (Figure 3.30(r)): The facial heights were increased, however the patient's mouth was open, and the findings were not valid. The SNA angle was significantly reduced. (SNB was not valid for reasons stated previously). The cranial base angle (ba-s-n) was more obtuse. The cranial base dimensions were increased generally. Discussion: This patient exhibits platybasia of the cranial base, and demonstrates the corresponding reduction in facial angle as a result of the distorted cranial base and the lack of forward projection of the maxilla (see maxilla). Figure 3.30(r) Z Scores of the Craniofacial Dimensions and Angles for patient rP compared with the 2 Year otd Experimental standard Zscorc Facial heights Facial Angles Cranial Base 8.00 SNA SNB Angle 6.00 Dimensions 4.00 o 2.00 0.00 -2.00 -4.00 -6.00 -8.00 -10.00 -t2.00 É k ào o E bo bo I Ø I I I ¿ I -o Page 280 Crouzon Syndrome 3.4.13 Clinical and Radiographic Findings for Patient LW Clinical Features Thischildpresented aged 5 years and 6 months (Figure 3.5). There was no family history of Crouzon syndrome. A sagittal synostectomy had been performed elsewhere when he was 3 months of age. On examination, the calvarial shape was oxycephalic. He had proptosis and hypertelorism. Ptosis of the left eyelid, left amblyopia and astigmatism were also present. Mild bilateral papilloedema was found. He suffered chronic nasal obstruction and had bilateral middle ear effusions and hyponasal speech. Maxillary hypoplasia was evident and he had a class III occlusal relationship. Lateral, Antero-Posterior and Basal Radiographs Lateral, antero-posterior, and basal views showed fusion of all sutures with a copper-beaten appearance of the calvaria. The spheno-occipital synchondrosis was patent (best seen on the post-operativeradiographs in Figure 3.5). The sella had an additional depression anteriorly in the region of the jugum sphenoidale. The lesser wings were swept up lateraliy producing the harlequin-mask appearance on the antero-posterior radiograph. The orbits were mildly dystopic with the left lower than the right. The second dentition was appearing in the hypoplastic maxilla. 3D CT Reconstruction Calvarial bones: The head shape was mildly oxycephalic and scaphocephalic with complete calvarial suture fusion, including the zygomatico-spheno-temporal complex. A bilateral ridge along the sagittal suture was present, possibly a result of the previous craniectomy. Cranial base: The cribriform plate was thin in the anterior cranial fossa. Small bony defects were seen above the orbits and in the region of the jugum sphenoidale and ethmoid spine. The lesser wings of the sphenoid were swept up and the greater wings were protruding into the orbits. Depression of the jugum sphenoidale anteriorly produced the appearance of double depression to the sella. The spheno-occipital synchondrosis was not visible as being patent on the 3D CT reconstruction.
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