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Craniosynostosis and strabismus
- 1. STRABISMUS IN CRANIOSYNOSTOSIS Presenter: Dr.B A Maithri Moderator: Dr.Smita
- 2. Overview • Introduction to Craniosynostosis • Strabismus in CS • Evaluation • Management • Case Discussion
- 4. Craniosynostosis • Developmental craniofacial anomaly • Premature closure of cranial sutures Image source: Dr.Vasudha
- 5. CALVARIAL SUTURES Deposition of osteoid at the sutural margins Surface apposition and resorption (remodeling) of the bone Centrifugal displacement by the expanding brain Kajdic N, Spazzapan P, Velnar T. Craniosynostosis - Recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci. 2018;18(2):110-116. Published 2018 May 20. doi:10.17305/bjbms.2017.2083
- 6. Cells undergo transformation Migrate to the sutures Growth regulators FGF, TGF-β Mechanical signals Fusion of sutures Duramater Genetic Mutations/ Environmental causes Premature Closure of Sutures Kajdic N, Spazzapan P, Velnar T. Craniosynostosis - Recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci. 2018;18(2):110-116. Published 2018 May 20. doi:10.17305/bjbms.2017.2083
- 7. Premature closure Brain matter expands perpendicularly Abnormal skull shape - Craniosynostosis
- 9. Kajdic N, Spazzapan P, Velnar T. Craniosynostosis - Recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci. 2018;18(2):110-116. Published 2018 May 20. doi:10.17305/bjbms.2017.2083 Types Kimonis V, Gold JA, Hoffman TL, Panchal J, Boyadjiev SA. Genetics of craniosynostosis. Semin Pediatr Neurol. 2007;14:150–161
- 10. Ko JM. Genetic Syndromes Associated with Craniosynostosis. J Korean Neurosurg Soc. 2016;59(3):187-191. doi:10.3340/jkns.2016.59.3.187
- 11. In India • Most common – Coronal synostosis • Syndromic – Apert’s Sharma, Ramesh Kumar MCh, DNB Craniosynostosis in an Indian Scenario: A Long-term Follow-up, Plastic and Reconstructive Surgery - Global Open: March 2020 - Volume 8 - Issue 3 - p e2696
- 12. Images courtesy: Dr.Sumitha, Dr.Maithri
- 13. Strabismus in Craniosynostosis • 60% present with squint • Esodeviations - in Aperts • Exodeviations - in Crouzons • Exodeviations - in Non-syndromic • Pattern strabismus – V pattern > A pattern • Direction of deviation may change after cranio-facial surgery Lehman S. Strabismus in craniosynostosis. Curr Opin Ophthalmol. 2006 Oct;17(5):432-4. doi: 10.1097/01.icu.0000243017.90004.5b. PMID: 16932059.
- 14. Mechanism of strabismus Anatomic factors: • Shallow, exorotated orbits – poor support • Short orbital walls – arc of contact of EOM is altered • Disordered structure of cranial base • Complete absence of a muscle • Anomalous insertion Cheng H, Burdon MA, Shun-Shin GA, Czypionka S. Dissociated eye movements in craniosynostosis: a hypothesis revived. Br J Ophthalmol. 1993;77(9):563-568. doi:10.1136/bjo.77.9.563
- 15. Sensorimotor factors: Ex-cyclorotation of muscle cone Altered vectors of muscular action Under or overaction of muscles Cheng H, Burdon MA, Shun-Shin GA, Czypionka S. Dissociated eye movements in craniosynostosis: a hypothesis revived. Br J Ophthalmol. 1993;77(9):563-568. doi:10.1136/bjo.77.9.563
- 16. Ipsilateral orbital extorsion + superior lateral orbit elongation Globe extorsion – upward offset of MR + downward offset of LR MR – elevator in adduction LR – depressor in abduction Simulates IO overaction https://www.ophthalmologytimes.com/view/strabismus-alignment-surgical-challenge
- 17. Superior orbital rim retrusion Trochlea retrusion Superior Oblique underaction
- 18. Evaluation • History – Birth, Developmental, Family • Vision • Cycloplegic refraction • Examination of adnexa and slit lamp examination
- 19. Squint assessment: • Fusion – to be tested in preferred gaze before performing cover test • To be assessed and quantified in all gazes and head-tilts • Extra-ocular movements – both on ductions and versions • Torsion demonstrated on fundus examination
- 20. Other Ophthalmic features • Dystopia • Hypertelorism • Amblyopia • Nystagmus • Proptosis • Ptosis • Lagophthalmos • Corneal exposure Image source: google search
- 21. • Short stature • Syndactyly • Broad thumbs/great toe • Beaked nose or bifid nasal tip • Cleft lip/palate • Airway Obstruction • Hearing loss • Torticollis Image source: google search Systemic Features
- 22. Management Amblyopia Therapy • Glasses – after cycloplegic refraction • Occlusion therapy – care to be taken in proptosed eyes with lagopthalmos/corneal exposure • Prompt management of lid and corneal abnormalities
- 23. Before considering surgery - • Complete systemic evaluation by pediatrician • Genetic evaluation • Imaging – CT/ MRI • Re-modelling helmets - infants • Cranio-facial surgery Kajdic N, Spazzapan P, Velnar T. Craniosynostosis - Recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci. 2018;18(2):110-116. Published 2018 May 20. doi:10.17305/bjbms.2017.2083 Sharma, Ramesh Kumar MCh, DNB Craniosynostosis in an Indian Scenario: A Long-term Follow-up, Plastic and Reconstructive Surgery - Global Open: March 2020 - Volume 8 - Issue 3 - p e2696
- 24. Imaging of brain and orbit - • Orbital anatomy • Extraocular muscle position • Excyclorotation of EOM • Dysgenesis
- 25. • Harlequin shaped orbit • Absent/hypoplastic muscles • Abnormal positioning of trochlea Abdelrahman M. Elhusseiny, Elisah M. Huynh & Linda R. Dagi (2020) Evaluation and Management of V pattern Strabismus in Craniosynostosis, Journal of Binocular Vision and Ocular Motility, 70:1, 40-45
- 26. Patch test: • Torticollis – ocular or non-ocular Abdelrahman M. Elhusseiny, Elisah M. Huynh & Linda R. Dagi (2020) Evaluation and Management of V pattern Strabismus in Craniosynostosis, Journal of Binocular Vision and Ocular Motility, 70:1, 40-45
- 27. Timing of surgery Before or after Craniofacial surgery? Diamond et al • Change in squint seen in only 9/120 patients after cranio-facial surgery. • Advocates early squint correction Morax et al • Change in squint seen in 9/10 patients after cranio-facial surgery • Advocates squint surgery after primary surgery Diamond GR, Katowitz JA, Whitaker LH, et al. Ocular alignment after craniofacial reconstruction. Am J Ophthalmol. 1980;90(2):248–250. Morax S. Change in eye position after cranio-facial surgery. J Maxillofac Surg. 1984;12(2):47–55
- 28. Pre-operative counselling • Realistic expectations • Pattern may not be fully corrected • Surgery will not change shape of globe/face • Need for multiple surgeries • Surgery may improve binocularity (in very young children) but not vision
- 29. Surgical Approach • FDT and exaggerated traction test – SO and IO function • SO tuck or IO weakening • Recession/Resection of Horizontal Recti with vertical displacement • Recession/Resection of Horizontal recti with slanting
- 30. • Conjunctival incision – Limbal incision for recti Fornix incision for obliques • Radial relaxing incisions recommended • Identify muscles correctly before hooking – For IO, identify/hook LR and IR first – direct viewing better than MRI/CT Jean D A Carruthers, Strabismus in Craniosynostosis syndromes. Clinical Strabismus Management – Rosenbaum Santiago, pages 393-403
- 31. • If LR and MR at approximate normal horizontal meridian – true IO over action – requires IO weakening procedures • Severe IO overaction – Anterior transposition of IO ( Bell’s phenomenon to be noted pre-op) • No IO over-action and no displacement of LR – SO underaction – SO tuck Jean D A Carruthers, Strabismus in Craniosynostosis syndromes. Clinical Strabismus Management – Rosenbaum Santiago, pages 393-403
- 32. For V pattern EXotropia : MR resection with down-shift LR recession with up-shift MR LR LE For V pattern ESotropia: B/L MR Recession – Lower margin recessed more than upper margin Vertical Displacement: Slanting:
- 33. IO Weakening Procedures • IO Recession – upto 14 mm • Anterior transposition – Alters path of muscle Stager D Jr, Dao LM, Felius J. Uses of the Inferior Oblique Muscle in Strabismus Surgery. Middle East Afr J Ophthalmol. 2015;22(3):292-297. doi:10.4103/0974-9233.159723
- 34. • Anterior Nasal transposition – Transposed to a location over the nasal half of the inferior rectus muscle • Myectomy – Removal of a segment of IO without detaching from insertion
- 37. Case 1 • 6 year old • Uneventful perinatal and developmental history • Tilting of head to watch TV • Using glasses and had undergone amblyopia therapy • Facial asymmetry with right- sided forehead depression • Left head tilt • VA: 20/20 and 20/25 with - 1.50D cyl X 150˚ • Stereopsis : 400 secs of arc • 12 PD right hypertropia increasing in LG and RHT • Right IOOA • Extorsion in fundus in RE Matalia J, Kasturi N, Brodsky MC. Synostotic anterior plagiocephaly: a cause of familial congenital superior oblique muscle palsy. Am Orthopt J. 2013;63:80-4. doi: 10.3368/aoj.63.1.80. PMID: 24141756.
- 38. CT scan: • Right- sided frontal plagiocephaly • Lateral displacement of the superior rectus muscle • Medial displacement of the inferior rectus muscle on the affected side Right inferior oblique anterior transposition under GA
- 39. CASE 2 • 2 year old • Uneventful perinatal and developmental history • Head tilt to right since 1 year • Facial asymmetry with left sided flattening of forehead • Right head tilt • TAC: 20/84 at 84 cm • left hypertropia - increasing on RG and LHT • Left IOOA • Extorsion in fundus in LE Left inferior oblique myectomy under GA
- 40. 1 year later : • Intermittent outward deviation of eyes • Visual acuity: OU: 20/25 • IDS of 15 PD with B/L DVD • Extra-ocular movements: Full • Treated with glasses After 1 more year: • IDS – 30 PD with poor control with B/L DVD B/L Lateral Rectus recession with half tendon width infra-displacement
- 41. Discussion • Synostotic plagiocephaly - indistinguishable from neurogenic congenital superior oblique palsy • Older patients - facial asymmetry simulate longstanding superior oblique palsy. However, it involves the forehead and lower face. • Though unable to evaluate the presence or absence of trochlear retrodisplacement on orbital images, heterotopias of muscles was confirmed. Matalia J, Kasturi N, Brodsky MC. Synostotic anterior plagiocephaly: a cause of familial congenital superior oblique muscle palsy. Am Orthopt J. 2013;63:80-4. doi: 10.3368/aoj.63.1.80. PMID: 24141756.
- 42. • Anatomical aberrations could necessitate a different surgical approach • Excellent responses to weakening of the inferior oblique muscle in these and other patients with unilateral coronal synostosis Matalia J, Kasturi N, Brodsky MC. Synostotic anterior plagiocephaly: a cause of familial congenital superior oblique muscle palsy. Am Orthopt J. 2013;63:80-4. doi: 10.3368/aoj.63.1.80. PMID: 24141756.
- 43. Our Case 35 year old male Squint since birth Using glasses since 20 years Facial asymmetry Ptosis
- 44. Image credits: Dr.Bhanumathi Hirschberg: 15 ° LXT Cover test: LXT – AXT with R/L with DVD PBCT : 35 PD BI + 25 PD BD (in front of RE) in all gazes and headtilts EOMs: B/L SO and IO over action MRI Brain and Orbit: Fusion of coronal sutures on right side Frontal Plagiocephaly
- 45. Plan: To tackle horizontal muscles first and if needed to operate obliques/vertical muscles as a second procedure Left Eye MR Resection + LR Recession under MAC Post op at 1 month: 10 PD BD for near 25 PD BD with 8 PD BI for distance Convergence Exercises Patient happy with Outcome
- 46. Take Home Message • Strabismus in craniosynostosis is difficult to treat • Differentiate from true muscle palsies and true muscle over-action • CT/MRI before considering surgery • Surgical approach can be complicated and can achieve varied results
- 47. THANK YOU Rocking Crouzon’s in Style!
Editor's Notes
- Take a look at these pictures. As soon as you see this baby, you notice that he isnt a normal looking child. But what are you actually seeing? Keep these pictures in mind and we will get back to them in a while.
- A craniosynostosis is a developmental anomaly which occurs as a consequence of abnormal and non-physiological sutural fusion. When one or more sutures are prematurely closed, the compensatory growth starts perpendicular to the patent sutures since the brain still grows and expands in the direction of lower resistance. The result is an abnormally shaped skull and also, in more severe cases, increased intracranial pressure (ICP), as well as sensory, respiratory and neurological dysfunction
- The fusion of the sutures is mainly regulated by the dura mater, which interacts with the overlying tissues of the cranial vault. The dura mater provides many important regulators of growth, such as intercellular signals (for example, signaling mediated by fibroblast growth factor [FGF] and transforming growth factor beta [TGF-β] have been suggested to be vital in this process), mechanical signals, and cells which undergo transformation and migrate to the sutures. This complex signaling cascade can be disrupted by a large number of genetic mutations, leading to an abnormal development of the cranial sutures [9-14]. Finally, this may result in a premature fusion. Genes most commonly mutated in craniosynostosis are
- Craniosynostoses are classified according to the sutures and the frequencies of these different types of craniosynostoses are as follows: Sagittal (≈60%), coronal (≈25%), metopic (≈15%), and lambdoid. The sagittal suture (40–55%) is the most commonly affected, followed by the coronal (20–25%), metopic (5–15%), and lambdoid (<5%) sutures
- Non-syndromic – 70%
- doi: 10.1097/GOX.0000000000002696
- Pictures of B/o Fouziya – 9 days old, 3rd deg CM, FTND, no NICU admission, frontal bossing – acrocephaly, depressed nasal bridge, syndactyly of hands and feet, proptosis, lagophthalomos - ?? Aperts syndrome. Does this baby have strabismus? We will have to wait a while to know since fixation is yet to develop.
- Refractive errors are a major cause for visual impairment after strabismus. Hypermetropia more common in syndromic and astigmatism more in nonsyndromic. 20 –35 % amblyopic. Optic atrophy seen in 7 %.
- Excyclorotation, defined as the rotation of the / j upper pole of the globe away from the midline, would explain the pattern of dissociated movements simply by invoking Hering's law without the need of assuming over or underaction of - certain muscles. (counterclockwise rotation of the rectus muscles of the right eye and/or clockwise rotation of the rectus muscles of the left eye
- Coronal synostosis
- While requesting for MRI/CT – mention orbital cuts and to look for EOM specifically. MRI not possible if metal implants have been used for craniofacial surgery
- 1. Harlequin orbit – retrusion of upper lateral margin, coronal suture on one side fused,
- (Kushner) Nischal has indicated that many of these patients have an abnormally tilted union between the base of the skull and the first cervical vertebra. This results in a persistent non-ocular head tilt even after strabismus has been corrected
- Diamond et al - evaluated the ocular misalignment after craniofacial reconstruction. They reported that out of 120 patients who underwent complete ocular examination pre-and postoperatively, only nine patients developed a change in their primary position ocular alignment and this was persistently more than 10 prism diopters in only 3 patients. the current trend toward earlier craniofacial intervention (by 4 months of age for endoscopic procedures and close to year of age for cranial vault expansion) makes awaiting completion of the primary craniofacial repair reasonable. In our scenario, such cases are rare and infantc with CS brought to us for squint surgery is rarer still. Amblyopia therapy takes priority and getting consistent measurement of deviation in infants is almost impossible – hence the question of whether to operate before before cranio-facial surgery or not, does not arise.
- Lax SO – tuck, IOOA - weaken
- Some muscles may be inserted closer to limbus than normal
- If Bells is strong/good pre-op, lesser chance of corneal exposure post-op as antr transposition of IO can lead to lagophthalmos
- Vertical transposition of the horizontal recti involves either recessing or resecting the muscle and then re-inserting it to the sclera with a vertical offset. In slanting procedures, In V pattern esotropia, bilateral medial rectus muscle recessions are performed, with the upper margin of the muscle being recessed to correct the deviation in upward position and the lower margin being recessed a greater amount according to the downward deviation. In A pattern esotropia the deviation is greater in upward gaze and so more recession is performed on the upper margin than on the lower margin of the muscle. The difference in recession was between 2 and 3 mm in accordance with the differences in measurements.
- IO recession: 1.b anterior border is placed 5 mm posterior to the temporal insertion of the inferior rectus muscle Antr transposition : postoperative limitation of elevation during abduction – IOOA of opp eye Myectomy: faster than a recession. In addition, there is less risk of surgical intraoperative complication because there is no scleral pass required to reattach the muscle. The disadvantage is the possibility that the muscle may reattach and lead to recurrent overaction.
- Antr Nasal trans: 2 mm nasal to the nasal border of the inferior rectus muscle and 2 mm posterior to the inferior rectus insertion. Makes the inferior oblique muscle into a tonic depressor Myectomy: faster than a recession. In addition, there is less risk of surgical intraoperative complication because there is no scleral pass required to reattach the muscle. The disadvantage is the possibility that the muscle may reattach and lead to recurrent overaction.
- IO recession: 1.b anterior border is placed 5 mm posterior to the temporal insertion of the inferior rectus muscle Antr transposition : postoperative limitation of elevation during abduction – IOOA of opp eye Antr Nasal trans: 2 mm nasal to the nasal border of the inferior rectus muscle and 2 mm posterior to the inferior rectus insertion. Makes the inferior oblique muscle into a tonic depressor
- Dr.Jyoti matalia has published an article in the American orthoptic journal in 2013 written along with Prof Brodsky. They examined two siblings with familial synostotic plagiocephaly and simulated congenital superior oblique palsy who were successfully managed with weakening of the ipsilateral inferior oblique muscles. Let’s take a look at these cases.
- Postoperatively, her inferior oblique overaction, hypertropia, and head tilt resolved.
- Synostotic plagiocephaly - secondary orbital aberration involving the extraocular muscles, leading to a clinical picture that is otherwise. SO palsy involves deformation of lower 2/3rds of face