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Given the severe limitations that it represents for those who suffer from Retinitis Pigmentosa, who are mostly young, a team of Cuban scientists, led by Prof. Dr. Orfilio Peláez Molina, has been dedicated for more than forty years to study the therapeutic possibilities for stop the advance of this disease.
Treatment for Retinitis Pigmentosa consists of performing a surgical technique that is combined with other therapies in addition to surgery.
The leading clinic for this treatment against Retinitis Pigmentosa in Cuba is the Camilo Cienfurgos International Center for Retinitis Pigmentosa.
It is a "revitalizing" eye surgery consisting of a transplant of the orbital vascular adipose tissue, pedunculated, located in the suprachoroidal space and which, through an angiogenesis mechanism, contributes to improving the function of the still active photoreceptors.
The surgical procedure for the treatment of Retinitis Pigmentosa is fundamental because it considers that it has a more effective action on the stabilization of the disease.
The surgical technique for the treatment of Retinitis Pigmentosa is indicated when there is a significant loss of visual field in patients with a diagnosis of Retinitis Pigmentosa, above what is expected annually, ie approximately 4%; or when there are restrictions of the visual field greater than 20%. Also in cases in which there is already central involvement associated with peripheral losses, determined by loss of visual acuity in 4 lines of the Snellen chart.
1.- Confirmatory diagnosis of Retinitis Pigmentosa.
2.- Assessment of the clinical stage, very important aspect to perform the surgical technique according to possible modifications.
3.- Assess the hemodynamic state of the patient.
4.- Analyze the state of the vision and the visual field.
5.- To know the antecedents of other ocular and systemic diseases.
6.- Assessment by the Internist, the Pediatrician, if the children are treated and by the team of anesthesiologists
Taking into account the result of the different assessments already mentioned is that we are able to carry out the surgical strategy, the treatment for Retinitis Pigmentosa is why we say that it is specific for each person.
After the measures of asepsis, antisepsis and preparation of the operative field, we will proceed according to the following steps:
1. Incision of the conjunctiva: it will cover approximately 60º, in the quadrant that has been selected temporarily. In some patients, due to difficulties in obtaining a good surgical field, more extensive incisions will be made. It is important to make an incision of the conjunctiva that allows the identification and opening of Tenon's capsule. It must be done meticulously to avoid difficulties in subsequent procedures, using a Kelly curved forceps to separate the visceral leaf of the Tenon capsule from the bulb of the eye, making Tenon's virtual suprasclerotic space real.
2. Identification and fixation of the muscles: the rectus muscles corresponding to the surgical area are identified, which will be fixed with 3-0 silk, which will pass below them. The fins that are tense will be released by means of a dampened applicator, to avoid muscular traumatisms.
3. Surgical position of the eye and identification of the anatomical references: the ends of the silk threads that fix the muscles move, in such a way that a movement occurs around the center of rotation of the eye, with which the posterior segment moves obliquely visualizing; and the previous segment is hidden. This is achieved by placing the threads crosswise, at an approximate angle of 60 º. We use a separator or recliner designed by Professor Peláez or also using a separator of Desmarres and strabismus hooks to separate redundant conjunctiva and expose the surgical field properly. The scleral insertion of the inferior oblique muscle, the emergence of the vasa vorticosa and the orbital vascular adipose tissue are localized.
4. Haemostasis: Possible traces of lax supraesclerotical connective tissue are released by an applicator. We proceed to cauterize the episcleral vessels in the area of ??the surgical area.
5. Sclerotomy: a non-perforating sclerotomy is performed in a line parallel to the limbus, located between the rectus muscles of the chosen quadrant, 3 mm posterior to its scleral insertion and 2 mm away from the scleral path. The incision will have an average extension of 10 mm, the extension varies according to the size of the eye and the location of the muscular insertions. In the future, the incision is deepened using the scalpel in an oblique position in such a way that the dark coloration of the choroid is visualized through the deeper scleral fibers and the fusca lamina. In this way, we have the cleavage plane in which we will work. Lateral incisions are made, parallel to each other and extending anteroposteriorly as the lamellar sclerotomy progresses, maintaining the desired epicoroid plane.
To advance it is used as aid, firstly the scalpel and then Vannas scissors and scarifier. The incision near the inferior oblique muscle passes about 2 mm from it and ends at 2-4 mm from its posterior end. The lateral incision that enters in relation with the vasa vorticosa, changes its direction and approaches the center of the scleral flap to avoid vessel injury and extends towards the posterior pole until it is positioned about 6 mm from the posterior end of the insertion of the inferior oblique muscle and vasa vorticosa. The lamellar sclerotomy is finished in the direction of the vicinity of the oblique (inferior) muscle insertion, where care must be taken because of its relationship with the macular area.
6. Microincisions: with the scalpel in a vertical position, microincisions are made in the epicoroid bed, which must be parallel to each other and crossed obliquely to create a lattice.
7. Identification of the orbital adipose tissue, dissection and selection of the fragment to be grafted: in direct relation with the bulb of the eye and in a posterior direction, the adipose orbital tissue covered by the capsule remains. This makes a certain protrusion between the oblique muscle that goes towards its orbital insertion and the end of the scleral flap that is more posterior and close to the vorticus. This tissue is clamped and separated from the surrounding by blunt dissection by Kelly applicator and clamp, when pulling it is careful not to interest area near the vasa vorticosa or muscle, so as not to injure them, for this incisions are made in the portion of its fibrous capsule that is placed towards the bulb of the eye, in the fibrous tracts that go towards the interior of the adipose tissue and simultaneously, 2 lateral incisions are made parallel, separated by 6-9 mm, which allows the tissue can move from its posterior position to the line of the initial sclerotomy. In this way it is joined by a pedicle constituted by its capsule and subsequent extensions, in which the tissue and vascular continuity is maintained.
8. Fixation of the orbital adipose vascular graft to the sclerochoroidal bed with sutures: the vascular fatty pedicle is fixed by virgin silk points 7.0 or 8.0, to the posterior most edge of the anterior lip of the initial sclerotomy; is fixed to the angles that form the initial incision and the incisions in the anterior posterior direction, from these two ends it is preferred to start at the end that is farthest from the origin of the pedicle, to ensure its proper extension on the epicoroid bed and at the same time it is verified that its length is appropriate to be free of tensions. It will then be sutured to the posterior border of the sclerotomy bordering the oblique muscle at its most posterior end. With this the tissue is extended on the epicoroid bed and it is easily observed that the vascular adipose tissue consists of the following parts: neck, body, head.
9. Replacement and fixation of the scleral fragment: the scleral flap extends over the graft. The sclerotomy is closed with loose stitches or continuous surget. It is preferred to start at the ends and then suture the edge in relation to the inferior oblique muscle, so that scleral stresses are more appropriate. Care must be taken that the vascular adipose tissue is completely covered by the sclera as a prevention of possible postoperative fibrosis, for which it must invaginate into the flap of the adipose graft, when necessary.
10. Closure of the conjunctiva: conjunctiva is closed by surget.
11. Use of local or subconjunctival antibiotic and cycloplegic mydriatic eye drops, if there is no contraindication.
12. Occlusion of both eyes for a period of approximately 24 - 72 h.
The technique is indicated when there is a loss of visual field or visual acuity in patients with a diagnosis of Pigmentary Retinosis with visual field restrictions greater than 20º and central involvement that decreases visual acuity to 0.6 together with peripheral visual field losses .
This surgery may be useful in the treatment of other retinal dystrophies and conditions caused by retinal ischemia.
1. Operated retinal detachment, which hinders the performance of surgery.
2. Untreated retina detachment.
3. Staphyloma, which by its extension or by the degree of thinning of the sclera may have the risk of perforation.
4. Active inflammatory processes of the eye.
5. Vitreorretinian hemorrhages.
6. Infectious processes of the eye
7. People with significant systemic diseases that compromise the patient's life or the result of eye surgery.
8. In children under 8 years, assess the anteroposterior diameter of the eye.
Its application may vary according to the age of the patient, systemic and ocular alterations associated with Retinitis Pigmentosa, and the indication, concentration and sessions are not constant.
Its route of administration in the patient with Retinitis Pigmentosa can be: rectal and intravenous (autohemotherapy), administering it daily for 15 days; the dosage is regulated by the weight and age of the patient.
The electrostimulation is performed by a device that emits electrical energy sinuosity of low intensity.
The electrodes are applied in symmetrical points of the organism; The most selected in Retinitis Pigmentosa are those of the parietal and temporal, periorbital and cervical regions. They can also be applied in the plantar region of the lower limbs and on the palms of the hands.
The action of this therapy is to produce a micromassage at the level of the blood capillaries and the lymphatic circulation. Through this, the electroionic balance of the person under study can be assessed. If the answer is not similar in symmetrical points, then it is in the presence of a person with electroionic imbalance and its prognosis is more reserved, as long as this imbalance is maintained.
The number of applications is variable, its average ranges between 10 and 15 sessions, with a time of 5 min. of duration for each selected region.
Contraindications of the application of electrostimulation.
In patients who have been implanted with metal prostheses in knees, skull and other organs; example, pacemaker.
According to the characteristics of each patient, it is valued to complement the treatment for Retinitis Pigmentosa with other medications already exhaustively reviewed in this chapter.
When marked retinal and choroidal vascular damage is observed, drugs with hemorrhagic action are used. The cellular antischemics are used when there is a great commitment of visual acuity, choroidal damage and macular damage. Oxidoreductores are indicated to protect from oxidative damage to ocular structures, caused by free radicals, we recommend the ingestion of Lutein for the protection of macular damage, very frequent in these patients.
In all cases in which some type of drug therapy is indicated, the patient's health status, possible allergy to the drug, its toxic action, contraindications and possible side effects must be previously assessed.
OTHER RECOMMENDATIONS MADE TO PATIENTS ARE:
1. Protection of the eyes from ultraviolet radiation by means of glasses.
2. Do not smoke, because of the harmful effects of smoking.
3. Do not ingest retinotoxic medications, for accelerating photoreceptor damage.
4. Perform systematic exercises.
5. Diet rich in fruits, vegetables, legumes and low fat.
Cuban Treatment for Retinitis Pigmentosa