PCF Trepanation with Subtotal Excision of the 4th cerebral sinus blastoma

HOSPITAL DISCHARGE SUMMARY

Case record No 13/10084

Alina Dinarovna Badretdinova (born June 8, 2007), 6 year old, residing at: Kamyshlinskaya st. 51V, Ufa (registered at: Epronovskaya st. 46A, Leninski district, Ufa) was treated at the oncohaematology department of Republic Child Clinical Hospital from 15 till 22 of July, 2013.

Diagnosis: anaplastic ependymoma of the vermis growing into the area of scapus, partial resection, Mo. Complic. - noncommunicating hydrocephalus. Eye movement deviations. Peripheral paresis of the right facial nerve. Secondary atrophy of the optic nerve disks. Tetraparesis. Bulbar syndrome.

Was accommodated for a regular course of Temozolomide (temodal) monochemotherapy. The latest previous time she was treated in hospital at the oncohaematology department of RCCH on 21-27 May, 2013, after the discharge she had a 2-week rehabilitation treatment with Hospital No. 17 neurosurgery department.

At this hospitalization (17.06.2013) there were complaints of the absence of mobility, impairment of consciousness, dysphagy.

Transferred from Hospital No 17, where she was accommodated in neurosurgery department on 8-20 May, 2013. Preceding hospitalizations 13 March – 24 April, 22 April – 8 May 2013.

ANAMNESIS: transferred from Hospital No 17, where the child was in neurosurgery department from 16.10.2012 till 20.11.2012, and the clinical diagnosis was made:

Blastoma of the 4^th cerebral sinus (ganglioblastoma with initial growth from the brain stem), secondary internal noncommunicating hydrocephalus. Post subtotal resection condition.

Complication accompanying acute respiratory viral infection, acute pharyngitis, treatment was conducted. At admission (20.11.12) complaints of lurching walk.

Anamnesis: till September 2012, according to the mother, the child had no health problems. There was an acute beginning of disease – since 18 September 2012 there appeared headache, dizziness, nausea, vomiting, atony, sleepiness complaints. Was hospitalized to the city Child Clinical Hospital No 17 diagnosed with ARVI. Was consulted with a neurologist, the brain was CT’ed, - space occupying lesion revealed in the posterior cranial fossa (PCF).

Transfer to neurosurgery department recommended. At examination (when admitted to ND of CCH17) … a moderate grave condition was observed. Active position. Skin was pink, clean, moderatle moist. Clean vesicular resonance over all lung sections. Auscultatorily vesicular breathing, reaches all sections, no rale. Breathing rate – 20 in-outs per minute. Rhythmic heart tones, heartbeat rate – 94 beats per minute. Arterial tension – 100/70. Soft, painless belly. No peritoneal signs. Undisturbed defecation and urination. Neurological status: clear consciousness. Palpebral fissure D = S. Eye pupils D=S, lively reaction. Convergence paresis on the right. Tongue along the centre line. No bulbar abnormalities. Muscular normotonus. ‘CXP’ D=S, normal. Steady in Romberg pose. No meningeal signs.

Examination in Hospital No 17:

15 October 2012. Electrocardiography: sinus bradicardia, heartbeat rate 65 Electric Cardiac Axis vertical

12 October 2013. CT of brain space occupying lesion in the posterior cranial fossa (mudulloblastoma), after-bypass condition, dislocation syndrome, at the PCF level, diffuse atrophic changes in the cerebral cortex.

8 October 2012. Gastrofibroscopy superficial gastroduodenitis.

17 October 2012. CT of brain space occupying lesion in the PCF, severe secondary internal noncommunicating hydrocephalus with distorted neurolymph outflow, at the level of sylviduct, dislocation syndrome at the PCF level, brain edema.

17 October 2012. Rheoencephalography angiodystonic type with hindered venous return.

22 October 2012 Ultrasound investigation of abdominal cavity organs.

In the left iliac region there is a hyperechoic lesion of linear form, (peritoneal end of the by-pass system free liquid V 3.3 ml

25 October 2012 Chest X-ray lungs and heart without any visible pathology

15 November 2012 Ultrasound investigation of abdominal cavity organsreactive changes in the liver pulp. Biliary dyskenezia of hypotonic type against the abnormal shape. Enlarged spleen.

19 October 2012. Oculist OU initial congested Optic Disk Nerve (ODN).

16 November 2012. Oculistexpressed nystagmus at examination. Paretic changes OD, OU. Pale ODN, obscure on the temporal side, narrowed arteries, curved veins, transparent retina, ocular fundus of intense color.

Surgery - - - 19.10.2012 Ventriculoperitoneostomia on the right.

1. After treating the surgical field in the right occipital region an up to 4 cm horseshoe-shaped incision was made around the Dandy point. Hemostasis, the periosteum peeled, trefine opening was made and expanded with cutters to the size of the pump. Coagulation TMO 2x2 mm, lateral ventricle puncture made, pressurized transparent neurolymph obtained. A 6 cm ventricular catheter was set in the ventricular cavity, the pump was laid in the bed. Peritoneal end was laid under the skin through an additional incision 1 cm above the right clavicle towards the anterior abdominal wall.
2. Up to 4 cm incision to the right of the navel, transrectal laparotomy, the distal end of the shunt was put into the abdominal cavity. The shunt is functioning.
Layer-suturing of wounds. Aseptic dressing.

PCF Trepanation with Subtotal Excision of the 4th cerebral sinus blastoma

Under general anesthesia, after treating the surgical field with an alcoholic solution of chlorhexidine there was made a Masrfziger-Town linear incision. The edges of the wound were set apart. The occipital bone scale and posterior atlas half-arcs were skeletized. A burr hole was imposed, the bone splintered, a part of posterior atlas half-arc removed. Pachymeninx was opened in V-shape. Direct sinus bandaged. Vermis located with the yellowish-gray tissue of the tumor upwards, clear boundary located between the cerebellar tonsils. The tumor excision was started through morcellation with coagulation. Hemostasis during the surgery with Tachocomb, spongostan powder, sergicel fibrillar and sergicel-flow. After removing the bulk of the tumor it was revealed to have had fused with the bottom of the 4th ventricle in the upper sections. If you try to remove that part of the tumor a severe stem reaction occurs in the form of blood pressure rise to 150/100 mm, bradycardia up to 52-56 (at three attempts), which gave ground to terminate further surgical procedures in the area of ​​the brain stem. A TachoComb plate was stacked on the sector of the residual tumor.
At the initial admission (20/11/2012) there were complaints of unsteady walk, weakness. Potentially serious condition. Proper constitution, satisfactory nutrition. Skin and visible mucous coat is pale pink, clean. Peripheral lymph nodes not enlarged. Pharynx without hyperemia. Nasal breathing is free. Vesicular breathing, reaches all sections, no rale. Rhythmic heart tones, muffled. The abdomen is soft and painless. The liver and spleen are not enlarged. Undisturbed defecation and urination. Locally: in the occipital area of head there can be palpated the subcutaneous pump of the shunt system. The postoperative scar is formed.
Neurologically: clear consciousness. Position is active. Gets up and walks with support. Symmetrical face. Unsteady in the Romberg pose.
Conclusion by the CPAE pathologist - glioblastoma.

Histology report № 249558-85/12 of 21.11.12 (PAD of RCCH) determined is a tumor formation, represented by complexes of spindle-shaped rounded cells with dark nuclei and condensed chromatin, the nucleolus is not clearly defined. In most cells there can be noted nuclear polymorphism. Among the complexes of tumor cells observed are the foci represented by microcystic fields containing protoplasmic astrocytes, foci of pseudo-palisade structures and rosette-type formations. The tumor tissue shows large foci of necrosis in the form of eosinophilia, erosion of boundaries of the cell nuclei and fibers; vascular proliferation, foci of lime deposits. Conclusion: glioblastoma.

Thus, the diagnosis is: glioblastoma of the vermis, condition after subtotal resection. On 23/11/12 started was the application of adjuvant chemoradiotherapy of HIT-GMB-D/ PIV block : cisplatin 16 mg 1^st -5^th days, etoposide, 1^st – 3^rd days 80 mg, vincristine 1.2 mg 5 day (23-27.11.12), underwent satisfactorily. Accompanying treatment – emictory, fluconazole, antibiotics, antiemetic.

From 21.11.12 radiotherapy (cranial irradiation amounting to 54 g total boost dose with 1,8 g single focal (boost) dose in the tumor bed). Simultaneously, vincristine 1,2 mg weekly (27.11.12, 4.12.12, 11.12.12, 18.12.12). The MRT of spinal cord dated 22.12.12 there are no data for Mts.

The MRT of cerebrum dated 22.11.12 : after subtotal excision of the mass lesion in the PCF there remains the formation of non-homogenous structure with clear and uneven edges 23x24x52 mm. Tricorns not enlarged, the right postcornu has a ventricular peritoneal shunt.

Blood group A(2) rhesus neg.

Underwent hospital treatment in oncohaematology dept. of the RCCH. Treatment was continued – radiation therapy of cerebrum till the total boost dose of 30.2 g, endured satisfactorily.

Vincristine 1.2 mg on 25.11.12, diacarb half a tablet with asparcam, ambrobene, erespal, biceptol.

29.12.12. the first stage of radiotherapy completed, discharged from the hospital for 2 weeks.

Was hospitalized in oncohaematology dept. of the RCCH on 08-25 January 2013. Cerebrum radiotherapy continued (TBD=54.2 g )

Vincristine 1.2 mg 10+17+24.01.13 From 24.01.13 arbidol, nimesulid. General Blood Test of 24.01.13 L5.8 thous., Hb 95g/l, Tr 286 thous., blood sedimentation rate 5mm/h, biochemistry and urine analysis no pathology

1 block of supporting therapy (21-23.02.13) Vincristine 1.275 mg 1 day, CF650 mg – 1-3 days

The latest admission – from 13.03.13. Complaints of sleepiness, tissiculation, dry cough.

Treatment 2 block of supporting therapy from 14.03.13 till 16.03.13. Etopozid 132 mg 1-3 days, carboplatin 176 mg 1-3 days, supporting treatment – dexamethasone, asparcam, diacarb, antiemetic, infusive therapy, antibiotics, antimicotics.

Gradual negative dynamics of neurological condition – the girl gradually quits walking and talking –increasing tetraparesis, eye-movement distortions, bulbar syndrome. Neurological status – Consciousness – profound somnolentia. Weak reaction to examination. Pupils symmetrical, lowered reaction to light. Convergent squint. Disphonia, disphagy. The shunt functionizes. The 1.04.13 MT shows swelling of upper sections of the 4^th ventricule, the tumor being tightly glomerated with the brain stem, cerebral edema. =Surgical interference not indicated due to the patient’s severe condition and the impossibility of radical excision”

Intensive antiedemic dehydrating therapy is applied (dexamethasone 10 mg per day from 14.03.13 till 02.04.13, the dose increased due to growing edema of stem, Dexamethasone 6g 4 times per day intravenously, from 02.04.13 till 11.04.13, and from 12.04 till now (22/07/13 – TN) Dexamethasone 4g 4 times per day intravenously). Supporting treatment – infusive therapy (glucose-potassium-magnesium mix, albumin, salt solutions), biseptol, asparcam, diacarb, Quamatel, tube feeding.

From 10.04.13, after a repeated discussion with neurosurgeons, - additional administration of cavinton intravenously, neurox intravenously, cereton intravenously, B1 and B6 vitamins, piracetam.

MT of 11.04.13. A series of MRT-tomograms of supra- and subtentorial structures of cerebrum, without contract enhancement, shows the condition after removal of the PCF space-occupying lesion. Post-surgery changes are detected in the PCF: in the 4^th ventricule view has an oval-shaped space-occupying lesion 19x22x38 mm with the signs of perifocal edema. The tricorns and the 3^rd ventricule are enlarged. Subarachnoid cavities are randomly enlarged convexitally in the frontal area and parietal lobe, not sharply in the temporal regions, with deepened sulci. No convincing evidence of the shunt dysfunction revealed. Hypophysis of usual size and position. Stem structures without pathology signs. Cerebellar tonsils on the McRae line. = conclusion: the MR-signs of a PCF space-occupying lesion. Post chemo- and radiation therapy condition. A series of MRT-tomograms of supra- and subtentorial structures of cerebrum, with contract enhancement, show a more intensive MR-signal from the PCF space-occupying lesion in the 4^th ventricule projection, of roundish-oval shape, sized 19x22x38 mm. = conclusion: the post-chemo- and radiation therapy condition.

The clinical case has been consulted y prof. O.G. Zhukova (head of neurooncology dept at Federal Research and Clinics Centre), recommendation – palliative cyclic polychemotherapy Temozolomide 100 mg 1-5 days once in 4 weeks.

Treatment: 1 block Temozolomide 100 g 22-26.04.13, supporting therapy - antiemetic, famotidine, B1 and B6 vitamins, vitamin C, asparcam, diacarb, tube feeding, Biseptol 480 x 2 times per day according to the scheme +++---, cereton, neurox, dexamethasone (the dose lowered from 16 mg to 6 mg per day), infusive therapy with glucose-potassium-magnesium mix with aminophylline; from 30.05.13 changed to oral administration of gliathylene 400 mg x 2 times, pentoxiphylline 0.1 x 3 times, famotidine 20 mg x 2 t. By the release from hospital the dexamethasone dose 6 mg per day (2mg x 3t. intravenously). Transfusion of er.mass A(2) rhesus neg. dated 08.05.13 (No.1)

7 May 2013. A meeting of physicians prof. Z.M Yenikeyeva, deputy Chief Doctor R.V. Basharova, dept. head T.N. Krasavtseva, chief child oncologist R.R. Bairamgulov, doctor in charge V.B. Makhonin concluded: child A.D. Badretdinova, 5-year-old; diagnosis – anaplastic ependymoma of the vermis growing into the area of stem, partial resection, Mo. Complic. - noncommunicating hydrocephalus. Eye movement deviations. Peripheral paresis of the right facial nerve. Secondary atrophy of the optic nerve disks. Tetraparesis. Bulbar syndrome. Vegetative state.

The child was administered operative treatment in the form of partial resection of tumor and VPS, chemoradiotherapy under E-HIT protocol, 2 blocks of supportive therapy, showing negative dynamics – progressing stem symptoms, the condition stabilized against the post-syndrome treatment. However, a gross neurological defect remains (deep somnolentia, eye-movement symptoms, deep tetraparesis, bulbar syndrome, facial nerve paresis, persistent vegetative state). For anti-tumor treatment administered is the therapy with Temozolomide (5 days 100 mg orally or once in 4 weeks intyravenously), next course is planned for 20.05.13. currently the child is administered the post-syndrome treatment (general nursing, tube feeding, dehydration, vasoactive, nootropics). Discharge allowable for the outpatient therapy.

Note for residence pediatrician: application should be submitted to obtain Temozolomide 100 mg in capsules No.5 – 12 packs for supportive therapy outpatiently during 1 year (under the DLO program, as for a disabled child).

On 8-20.04.13 the child was in the neurosurgery dept of CCCH No 17 undergoing rehabilitation treatment (massage, nootropics, vasoactive). Subjectively, the mother notes improvement in the form of some motion activity.

Description as at 20 May 2013. Serious condition. Temperature 37.3*C. The severity is caused by the brain stem edema (tetraparesis, bulbar syndrome). Consciousness – somnolentia. Reaction to examination significantly reduced. Normal body temperature. Clean and pink skin. Satisfactory skin care. No stain on the mucosa. Harsh breathing, into all sections, no rhonchus. Breathing rate 24 in-outs per minute, bradypnea in sleep. Rhythmic heart tones, muffled, haemodynamics without cardiotonic support. Soft and painless abdomen. Defecation once in 1-2 days, stool not thinned. Urination not controlled. Sufficient diuresis.

Neurologically: consciousness deeply stunned. Does not walk, does not sit, holds the head unsteadily. Cannot fix the stare for long. Convergent strabismus. Right facial nerve paresis. Symmetrical pupils, react to the light. Tongue – leftward deviation. Expressed bulbar abnormalities (dysphagia, dysphonia) Tetraparesis. Pathological foot signs.

At the current admission a Temozolomide 100 mg monochemical therapy was administered orally.

21-25.05.13, accompanying treatment – tube feeding, neuromedin 5.0 i.m., zofran, vinpocetine half a tablet, twice, orally, transfusion of Er.mass 21.05.13.

A series of control MRT-tomograms without contract enhancement shows the condition after subtotal removal of space-occupying lesion in the PCF. There remains the space-occupying lesion, with sharp and jagged contours, of irregular structure, sized up 18x20x55 mm in the area of cerebellar vermis and 4^th ventricle. The lateral ventricles are enlarged, there is a pronounced interstitial edema. The right postcornu has a ventricular peritoneal shunt. Subarachnoid cavities are randomly enlarged convexitally in the frontal and parietal areas, not sharply in the temporal regions, with deepened sulci. The stem is compressed anteriorly, revealed is an interstitial edema. Cerebellar tonsils on the McRae line. The sella is not enlarged, hypophysis’ structure not changed. Conclusion: Condition after subtotal removal of posterior cranial fossa tumors, chemotherapy and radiation therapy. A series of MRT-tomograms of the cerebrum, after a contract enhancement, reveal intense enhancement of contrast agent in the space-occupying lesion in the vermis and the 4^th ventricule area, sized 18x20x55 mm. = conclusion: the post-chemo- and radiation therapy condition. Conclusion: Condition after subtotal removal of posterior cranial fossa tumor, chemotherapy and radiation therapy.

The clinical picture as of today (18/06/13). Serious condition. The severity is caused by neurological defect (tetraparesis, bulbar syndrome). S = 0,84 m² Consciousness – somnolentia. Reaction to examination significantly reduced. Normal body temperature. Clean and pink skin. Satisfactory skin care. No stain on the mucosa. Harsh breathing, conducted into all sections, no rhonchus. Breathing rate 24 per minute, heart rate 80 per min. T = 36,8 ° C. Rhythmic heart tones, muffled, haemodynamics without cardiotonic support. Soft and painless abdomen. Defecation once in 1-2 days, stool not thinned. Urination not controlled. Sufficient diuresis.

Neurologically: consciousness deeply stunned. Does not walk, does not sit, holds the head unsteadily. Fixes the stare for not long. Convergent strabismus. Right facial nerve paresis. Symmetrical pupils, react to the light. Tongue – leftward deviation. Expressed bulbar abnormalities (dysphagia, dysphonia) Tetraparesis. Pathological foot signs.

Was treated in the oncohaematology dept. of RCCH on 17-24.06.13. Admitted for continued administration of Temozolomide monochemical therapy. The dynamics show a little improvement of mental status and motion activity. Administered were Temozolomide 126 mg 18-22.06.13, accompanying treatment (infusion therapy, antiemetics, exercise, Normase). Albumin 10% 100 x 3 times. Takes chemotherapy satisfactorily.

tube feeding, neuromedin 5.0 i.m., zofran, vinpocetine half a tablet, twice, orally, transfusion of Er.mass 21.05.13.

Examination: neurologist consultation of 18.06.13. Diagnosis: Vegetative state. Severe degree tetraparesis. Secondary occlusive hydrocephalus, descending atrophy of the optic nerve disks. Paresis of the right facial nerve. Pseudobulbar syndrome. Treatment of the underlying disease recommended.

Ophthalmologic examination of 18.06.13 Visus OD finger count near face (detects correctly!), OS 0,02-0,03.

Objectively: OD eyelids calm, incomplete closing. Eyeball allocated inwards, arbitrary driven outwards, essentially restricted, max up to 10 ° inwards according to Grishberg. No abnormalities in the front section, expanded pupil, sluggish photoreaction. Optical media are transparent. Examination of the fundus is significantly impeded, in the area of ​​the posterior pole there can be seen monotonous pale optic discs of small size with clear boundaries, vascular bundle is characterized with significant venous congestion given the narrow blood vessels of the arterial bed. Retin-MZ and peripherals have no abnormalities, ophthamloscopes poorly.

OS eyelids calm, complete closing. The eyeball is in the orthophoria position, the eyeball movement is not restricted in any direction. No abnormalities in the front section, expanded pupil, sluggish photoreaction. Optical media are transparent. Examination of the fundus determines monotonous optic disk, clear boundaries, moderate venous congestion with relative pallor of the arterial bed, retin-MZ and peripherals have no abnormalities.

Conclusion: OD - esotropia resulting from the paresis of the facial nerve. Downward atrophy of the optic disc. Signs of a hypertensive syndrome. Recommended further examination upon stabilization. Keratoprotektive treatment of the OD to be continued.

Was treated at the department of oncohematology, RCCH on 15-22.07.13, admitted for the next set of Temozolomide. The clinical picture of a moderate positive dynamic (motion emerged, clear consciousness, feeding without tube).

Treatment administered: Temozolomide 130 mg 16-20.07.13, accompanying treatment – ondancetron, Normase, biseptol, diakarb). Took it satisfactorily.

General Blood Test of 16.07.13. L3.0 thous., Hb 119g/l, Er. 3,87 mln, Tr 206 thous., blood sedimentation rate 4mm/h, P2E1L35M7S55

Urine Analysis on 18.07.13, no pathology.

General Blood Test of 22.07.13. L3.1 thous., Hb 118g/l, Tr 134 thous.

Discharged for a break in treatment.

Recommended to carry out the next block of chemotherapy on 13/08/13. (6 caps. 100 mg)
Treatment received within the Russian Federation VMP (hitech medical aid)

Signature V.B. Makhonin, Doctor in charge

Signature T.N. Krasavtseva, Department Head

22/07/13

Date: 2015-12-24; view: 908