More About Saethre-Chotzen Syndrome

Oct 05, 2004 17:25

Saethre-Chotzen Syndrome is a rare deformity which creates abnormal gene expression resulting in restriction in the development of the cranium, eye socket, jaw line and ear. Visual impairment is common. Out ward manifestations include a pronounced under-development of the mid-facial region further accentuated with shallow eye sockets and somewhat protuberant eyes which are additionally wide set. Mid-facial underdevelopment results furthermore in a low set hairline, drooping of the upper eyelids as well as under development of the eye control muscles associated with ordinary movement.
In close relationship to the breathing and nasal obstruction issues is the high incidence of inner ear infections which plague Saethre-Chotzen Syndrome children. Due to the fore shortening of the mid-facial region, the jaw line can mal-form or in certain cases express a cleft or break in the jaw line. Meanwhile, the smaller nose and raised high arched palate result in frequent nasal or breathing obstructions. All of these formative and developmental issues are addressed in surgical interventions beginning early in the child's life and continuing until physical maturity has been achieved, and where development risks associated with brain and cranial expansion no longer are deemed critical.
Because the primary symptom of Saethre-Chotzen Syndrome bears on the cranial development of the child, medical science has promoted the view to parents that the preliminary operations should be done during the first year of the child's life. In so doing, the operation creates the necessary conditions for near-normal cranial case expansion which, in turn, allows the brain to grow or expand during this critical growth stage.
In addition to these symptoms, the ear can also be subject to significant under development. These conditions can all be expressed or be partially expressed in either a unilateral or bilateral manner throughout the anatomy of the face and head.
With such pronounced deformities inter-playing simultaneously, the Saethre-Chotzen Syndrome child experiences developmental challenges in speech, eating, and hearing and vision. These challenges increase logarithmically as the child grows since the Saethre-Chotzen Syndrome results in both delayed and altered growth patterns across the cranial-facial area. If Saethre-Chotzen Syndrome is left untreated the developmental challenges in brain formation, speech, eating, and hearing and vision will extend to problems of breathing due to increasing impact from jaw deformity and dental malocclusion.
Saethre-Chotzen Syndrome reconstructive procedures necessary to create these appearance changes include mid facial advancement and jaw line surgery in order to provide adequate expansion volume for cranial growth, eye surgery, ear reconstruction surgery, and extensive dental surgery in order to facilitate speech. Accordingly, the planning for such Saethre-Chotzen Syndrome surgeries requires a team approach where the surgeon may cooperate with a pediatrician as well as a speech therapist, dentist, as well as eye and ear specialists in order to build an overall surgical plan to achieve optimal desired effects.
Because of the significant physiological and psychological impacts of Saethre-Chotzen Syndrome, families seek to mitigate these effects by exploring reconstructive surgery to address outward appearances as well as overall functionality. In the time period leading up to your child's scheduled Saethre-Chotzen Syndrome surgery you'll have interviewed a number of plastic surgeons. You'll be looking for replies such as Saethre-Chotzen Syndrome procedure or facelifts or eyelid surgery or whatever are the focal areas for your visit. The plastic surgeon you select will provide you with a certificate evidencing their good standing.
In order to provide ongoing room for cranial expansion associated with growth and development of the child, surgeons need to initially release the synostotic sutures restricting the brain case to allow for proper brain growth. This initial surgery is, therefore, the start to a series of related surgeries which will attend the child's life until maturity.
Jaw surgery is often performed as yet another surgical strategy aiming at volume enhancements for the cranium. . However, some general guidelines apply as regards overall surgery strategies. Because of the existence of profound jaw line abnormalities, especially under-sized jaw line (potentially upper and lower as well as bilateral or to both sides of the face), the surgeon has to plan for ways to synthetically extend the jaw line by introducing a rib graft or by introducing a distraction device in order to elongate or stretch the jaw line tissues.
Meanwhile, the other members of the surgical team have to prepare for eye muscle surgery in order to address the issues of shallow orbital eye sockets, poorly functioning eye muscles resulting in crossed eyes, as well as the congenital ptosis or drooping of the upper eyelids. Once again, a pediatric opthalmologist must perform these extensive surgeries in order to re-normalize the eye socket and eye functioning.
Each patient's requirements will be unique as the surgeon will clarify in the interviews. In view of the many aspects of the child's face requiring re-spacing and modification, it's no wonder that dental occlusion also represents a major and ongoing area requiring repeat surgery. Jaw line surgery must dove tail with the orthodontist's work on the teeth and bite in order to facilitate both eating and breathing. Saethre-Chotzen Syndrome patient preparations are important. As your child readies for the day of surgery, you need to make some practical plans to assist both yourself and the physician. In terms of food, ensure that your child does not ingest any food or water after 12AM in the night before your scheduled operation. When your child arises in the morning, continue to avoid food or water, however take a very thorough bath or shower. Your child needs to be as clean as possible in order to reduce the chance of infection.
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