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DEPARTMENT OF PUBLIC HEALTH OF THE CITY OF MOSCOW CLINICAL RESEARCH CENTER OF MEDICAL HELP TO CHILDREN WITH DEVELOPMENT DISORDERS OF CRANIOFACIAL AREA AND INNATE DISEASES OF THE NERVOUS SYSTEM Aviatorov, St, 38, Moscow, 119620 Russia
CLINICAL RESEARCH CENTER OF MEDICAL HELP TO CHILDREN WITH DEVELOPMENT
DISORDERS OF CRANIOFACIAL AREA AND INNATE DISEASES OF THE NERVOUS SYSTEM
CASE RECORD # 4376/08
Angelina Khramtsova, 1 year old (D.O.B. November 18, 2007) was a patient at the neurosurgery unit of the
Clinical Research Center from December 22 until December 31, 2008 (from December 23 till December 30, 2008 she
was in the Intensive Therapy and ER unit) with the following diagnosis
: unverified tumor of the chiasm-cellar area of
the brain, condition after polychemotherapy of two lines, continued growth of the tumor, occlusive uncoupled
hydrocephalus, condition after ventriculoperitoneal ostomy with the junction of the frontal horns of the lateral
ventricles (interventriculostomy and ventriculoperitoneal ostomy on the right), relative compensation, hyperthermal
syndrome of unidentified (central?) genesis; generalized spasmodic attacks (febrile?), 3rd degree hypotrophy.
CASE HISTORY: The case history is described in detail in the previous case records (attached). The
patient’s condition remained to be stable until December 21, when at the background of a fever (38.8°C) tonic generalized convulsions have developed lasting for about one hour and stopping on their own after the body temperature went down. The patient was admitted to the Clinical Research Center on a scheduled date
for a complete check-up (of the main disease) and for making up a decision on the future treatment tactics.
AT THE TIME OF ADMISSION: the patient’s condition is severe, without, however, significant negative
dynamics as compared to the previous hospitalization. She continues to have a fever up to febrile numbers, generalized tonic tension (horneotony?) remains. The fever is resistant to the administered anti-febrile therapy. The weight is 5,850 g. The patient feeds partially on her own, and partially through an enteric feeding tube, 110-120 ml every 3 hours, the amount of food is digested. The skin appears to be clear and a little pale. The visible mucous are pink and clear. The turgor of tissues is somewhat down due to hypotrophy. The fauces is not hyperemic and clear. The patient is breathing by herself, breath rhythm is correct. The frequency of breathing motions is 28 per minute, auscultative puerile and passes equally into all the areas, there are no rales. Heart tones are clear, rhythmic, no noise is heard. Blood pressure is 95/55 mm. of mercury column. Heart rate is 138 per minute, the pulse on the periphery arteries is of satisfactional quality. The abdomen is of normal shape, no abdominal distension, soft, available for the deep palpation in all areas. Urination is normal, urine output is adequate. Stool is spontaneous and regular. Neurological status: the head has hydrocephalic shape. Head circumference is 45.0 cm. The bigger fontanel is soft, sized 2.0 x 2.0 cm. The girl is conscious, although weak and adynamic. No meningitis symptoms are observed. Reaction to the examination is by opening eyes, double-sided semi-ptosis, pupil reaction to light is weak. The look is not fixed, the pupils are D=S. Internal strabismus. Spastic tetraparesis with the symptoms of apparent hypertonus D>S. Bilateral Babinsky effect. Also noted are recurrent tonic generalized convulsions. Reservoir of the shunting system valve easily empties and quickly fills when run through. MRI of the brain dated December 23, 2008: in comparison with the MRI dated October 14, 2008 and CT scanning dated December 12, 2008, no significant growth of the size of the gigantic tumor of the midline brain structures is found. However, an apparent increase of the volume of its central necrosis is observed. Also in the dynamics an increase of the sizes of lateral ventricles is found with signs of periventricular edema, convexital subarachnoid spaces are not traced. One side of the interventricular anastomosis is in the cavity of the frontal horn of the right lateral ventricle, the other one is in the cavity of the frontal horn of the left lateral ventricle. The end of the ventricular catheter of the ventriculoperitoneal shunt is in the cavity of the right lateral ventricle; the position of the catheters is satisfactory. X-ray thoracic and abdominal examination (dated December 4, 2008, radiation dose 20 mkSv): no lungs pathology is found, the ventriculoperitoneal shunt is intact, the peritoneal catheter is in the abdominal cavity at enough length. On December 23, 2008 a diagnostic ventricular puncture is performed: transparent colorless liquor is obtained in rare drops
. Clinical analyses of the ventricular solution dated December 23, 2008: albumen – 0,5 gr/l, cytosis – 34/3 (l – 24, n – 10, m – 0), erythrocytes – 0–1, glucose is absent. Clynical analyses of blood: Hb – 101 gr/l, Ht – 32,6%, Er 4,24 x 1012/l, Tr - 250x109/l, L - 12,0 x 109/l (granular leukocyte – 61,1%, bond neutrophil – 4, microxyphil <!-- google_ad_section_end --> – 65, erythrocytes <!-- google_ad_section_end --> – 2, monocyte – 7, ɥ -22, ERS – 34 mm/h). General analyses of urine: alkaluria, albumen and glucose are absent, leucocytes 0-1 in sight.
On December 23, 2008 a medical consolation was summoned. Chair: Clinical Research Center
Director Professor Prityko A.G., Academician of Russian Academy of Natural Sciences, Emeritus doctor of
Russian Federation. CONCLUSION:
based on the conducted MRI of the brain, no reliable growth of the tumor is
found, however its apparent central autolysis is noted, which can implicitly serve as evidence for the disease
progression. At the same time also noted is increase of the brain lateral ventricles’ size in dynamics with the growth of
periventricular edema and pressure on the convexital subarachnoid spaces at the background of taking high dosage of
dexamethasone. Also noted is that the position of the ends of catheters I-II of interventricular anastomosis and the
ventricular catheter of the shunting system is in satisfactory. Reservoir of the shunting system valve easily empties and quickly fills, the bigger fontanel is not swollen, as well as there are no signs of ascites. The ventricular punction did not provide data in favor of intracranial hypertension. There are no inflammatory changes in the clinical analyses of the liquor. The apparent pathological symptoms, hypothermia up to febrile numbers resulting in febrile convulsions are most likely to be of general nature at the background of the main disease and the tumor autolysis. Nevertheless, taking into consideration the increase of the brain lateral ventricles’ size in dynamics with the growth of periventricular edema and pressure on the convexital subarachnoid spaces it seems plausible that the shunting system does not function properly. With the above mentioned in mind, the decision was to perform the following surgery to the child: ventriculoperitoneostomy on the opposite side (on the left) implanting a valve to low pressure. Also to decrease the risk of infecting the shunting system ventricular and peritoneal catheters impregnated in antibiotics – Codman Bactesial - will be implanted. The surgery was performed on an urgent
basis on November 24, 2008, without complications.
On December 23, 2008 at 9:40pm, at the background of no stool for 15 hours, acute abdominal distension
was noted with moderate diffuse tension of frontal abdominal hernia. Also reported is vomiting (three times) with fecal smell. Auscultation: acute suppression of intestinal peristalsis. This condition was accompanied by continuous fever up to 40.2°C and generalized tonic muscle tension. Prescribed medication: relanium, perfalgan (paracetamol) intravenously. The child was moved to the intensive therapy and ER unit of the Clinical Research Center where she received symptomatic and pathogenetic therapies. By December 24, 2008 symptoms of the dynamic intestinal obstruction at the background of electrolyte derangements were cut off. On December 24, 2008 the following surgery was performed: ventriculoperitoneostomy on the left. The child received antibacterial (tienam: Imipenem + Cilastatin) and antifungal (Diflukan: Fluconazole) therapies; correction of electrolyte derangements was done. The child’s condition remained to be highly severe. Consciousness is suppressed to somnolence; however, reaction to examination appeared. Also observed are spasmodic tonic attacks at the background of febrile fever which are not arrested by antipyretic therapy.
Based on clinical diagnosis, treatment anamnesis, severity and dynamics of the child’s condition, the board of
doctors finds the patient’s neuro-oncological illness incurable. Only mitigating and symptomatic treatment is
On December 30, 2008 the girl was transferred to the neurosurgery area. During the examination it is determined that
the condition of the child remains extremely severe. Sleepy level of consciousness. During examination she moved and
cried periodically. The skin and visible mucous appear to be clear. Febrile fever is up to 38.2°C. Heart tones are
muffled, frequency is 200 beats per minute. BP is 85/60 mm of Mercury. Breathing is pure, heard in all parts of lungs,
no rattling. The abdomen is soft, painless. No stool during the last 24 hours. See neurological status below. No
electrolyte imbalance. On December 30, 2008 MRI of brain was performed: the left lateral ventricle is depressed, the
right one remains considerably enlarged, the signs of periventricular tumor remain in place, however arachnoidal rimas
(grooves) are noticeable (subarachnoidal distance on the left is wider, on the right it appeared to narrow). In the
chambers of left and right side ventricles the shades of ventricular bypass catheters are noticeable. Antibacterial and
antifungal therapy was continued in the area with baclofen. Due to incurability of the main diagnosis the mother of the
child declined inpatient continuation of the treatment, she is taking the child home, she has been informed of the
possibility of critical worsening of the child’s condition. On December 31, 2008 the stitches were removed.
At the time of release there were no abnormalities in blood test according to the age (Hb- 134 g/l, L – 7.9 x
10 9/L, Tr – 260 X 10 9/L), the condition is extremely severe, stable. The child is sent home and will be attended by the pediatrician, oncologist and neurologist at residence. The child was not exposed to infections while in the hospital. The following is recommended:
Iin case of tonic seizures – relanium IV 0.5% - 0.5 ml
In case of fever above 38.5 C – Aminazin IV 2.5% - 0.2 ml.
Dexametazon IV 2.0 mg – 3 times a day.
December 31, 2008 Attending Physician Kuptsova E.V. The head of the department Kholodov B.V.
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