History of Present Illness Updated 5/19/02
Note: Results we currently do not understand or want to explore further are highlighted in green.On Thursday, April 4, (when he was 5 months old) the patient's mom reported his crying when being put down and need to be nursed every 2 hours. This was not normal behavior for this child. He still slept through the night.
On Friday, April 5, the patient's left eye began swelling at about 2 p.m. By 6 p.m. the child was noticeably uncomfortable, and by midnight the eye was completely swollen shut. The child was up crying all night. Child's pediatrician was consulted on Saturday morning, April 6 at Mesa Pediatrics and he was given Benadryl, which had no effect on the swelling.
Child was then taken and admitted through the emergency room at Chandler Regional Medical Center on 4/6/02. A CAT scan of the left eye was taken. Blood was taken and tested. He was then given an IV antibiotic (rocephin also known as ceftriaxone).
He was transferred to Desert Samaritan Medical Center at 9 p.m. on 4/6/02 for surgery.
The orbital ophthalmologist (Dr. Edelstein) aspirated the left orbit and placed a drainage tube. Samples were taken from the left eye orbit and left eyelid. The problem was thought to be a periorbital cellulitis possibly caused by an infection. Patient was treated with IV antibiotics.
The response of the eye to antibiotics was slow. Review of the CT scans from Chandler revealed a questionable lesion in the left eye. Cultures and stains revealed no organisms or growths in the eye samples.
A lumbar puncture was done of 4/8/02, which revealed nothing abnormal.
An MRI of the Orbit with and without contrast and an MRI of the brain with and without contrast was performed on 4/10/02. The brain MRI revealed 5 masses in the brain. The MRI of the left eye orbit revealed an approximately 1.5x1.0 cm soft tissue mass in the superolateral aspect of the left orbit that is thought to represent an enlarged lachrymal gland. Some possible bone involvement was noted. In the area of the periorbital enhancement, there is a localized oval shaped lucency surrounded by the area of soft tissue. Impression of the MRI: the findings may represent an inflammatory pseudotumor/ plasma cell granuloma. Histiocytosis is also considered. A malignancy is also considered.
The patient was transferred to Phoenix Children's Hospital on 4/11/02. On admission he was examined and noted to have a number of lesions on his scalp. The lesions were dime-sized and very slightly raised. One was brownish in color. The others were reddish. There is no pus or drainage from the lesions. The parents reported seeing the first lesion sometime in early February 2002. First lesions seen in photographs was on 3/25/02.
A CAT Scan of chest, abdomen, and pelvis was performed on 4/12/02. CT revealed an abnormal appearing Thymus, with a somewhat prominent convex right border. Pulmonary parenchymal nodules were identified in the right upper, middle and lower lobe of the right lung. Subcutaneous nodules were seen in the fat anterior to the right hip and also anterior to the left hip were seen. Impression stated consideration of granulomatous disease, histiocytosis, or lymphoma.
The nodules in his lungs were too small to be biopsied. The decision was made to biopsy one of the scalp lesions and one of the nodules in the groin on 4/16/02. The nodule in the right groin turned out to be a lymph node. The results of pathology on the scalp and lymph node were not conclusive. The scalp biopsy showed an atypical histiocytic proliferation, not further classified. The right groin lymph node biopsy showed a lymph node with reactive change.
During the week of 4/14/02 the parents began noting seizure-like staring episodes. The child would become limp and unresponsive and stare blankly. Parents reported the child had been having these prior to the first hospital admission, but they had been only 5 10 seconds in duration and not alarming. During the week, however, the seizures began increasing in frequency and duration. On 4/16/02 an EEG was performed for approximately 30 minutes, which was normal. The seizures continued, so a 24-hour video EEG was performed on 4/17-4/18/02. The child had several episodes during this time, and they were noted on the video by the neurologist, but the EEG was still normal during these episodes. It was decided no medication could be used given the normal EEG.
By 4/19/02 the seizures had increased to 3 4 minutes in duration. That evening the child began having periods of blindness following the seizures. By the morning of 4/20/02 the child could not see for most times.
Because the initial biopsies had failed to yield a diagnosis, more tissue was needed. A chest CT was performed on 4/19/02 to see if the lung nodules were at all attainable. The nodules had not changed since the prior CT and were thus too small to biopsy. A brain MRI was performed on 4/20/02, which showed that the brain lesions had increased in size since the first MRI of 10 days earlier. The largest tumor in the center of the brain between the ventricles was the least risky to biopsy. A CT of the orbit was NOT performed.
An ultrasound guided endoscopy brain surgery was performed on 4/20/02 to obtain tissue from the center tumor. Ten samples were taken of this tumor as well as a sample of another scalp lesion. Surgery was successful with no complications. CT scans were taken of the head on 4/21 and 4/22 to view swelling in the brain. Recovery from surgery was good. Neurosurgeon commented that the orbit lesion was still present and appeared somewhat like a derma-cyst.
After brain surgery, child's seizures decreased in length but were still several minutes long. Neurology was consulted again and the child was put on Phenobarbital on 4/22/02 to treat the seizures. By 4/24/02 the seizures and blindness were gone.
Initially, the pathologists at Good Sam though the brain lesions showed some kind of histiocytosis. The results were reviewed in their group meeting. The clinical diagnosis was Langerhans Cell Histiocytosis; however, the samples were sent to a reference pathologist for confirmation.
While awaiting the reference pathologist's diagnosis, further test and baselines were done and the child was prepared for treatment.
A complete skeletal survey was performed on 4/23/02. Two bone lesions were identified, one in the orbit of the left eye and one in the skull where brain endoscopic surgery had been performed (and was therefore not truly a 'lesion'). The rest of the skeleton was normal.
Specific Gravity of Urine test was done 4/23/02 to test for diabetes insipidis, which was normal (negative for DI). On 4/24/02, a porta catheter was installed in the child's chest, a bone marrow aspiration and biopsy were taken, and a lumbar puncture was performed. The lumbar puncture showed no abnormal or malignant histiocytoid cells were found (note: white blood cell count was 9 compared with 3 in the prior LP). The bone marrow was normal and a CD1a was negative.
An eye exam was performed on 4/24/02 which revealed no problems in the functioning of his eyes.
An ABR hearing evaluation was performed on 4/26/02 which revealed normal auditory thresholds and normal synchrony of the auditory system through the brainstem was measured for both ears.
On 4/27/02 patient had completed all assessment and preparation for treatment and was stable. Treatment plan was pending final diagnosis. Patient was discharged from PCH on 4/27/02 with no treatment plan.
On 5/1/02 Dr. McClain at Texas Children's Hospital saw the child. Treatment would await final pathology.
On 5/3/02 reference pathologist report was received and a diagnosis of Juvenile Xanthogranuloma (JXG) was given. The pathology was forwarded to a second reference pathologist. No results are back yet from the second reference pathologist.
On 5/9/02, a follow-up MRI was performed to assess the risk of hydrocephalus due to the center brain lesions getting too big. It was felt that there was still some time to begin chemotherapy treatment before the lesion would present a problem. The lesion could be removed surgically, but we would try to see if it would respond to chemotherapy first.
Doctors at PCH and Texas Children's consulted on treatment plan. Child would not be allowed on the international LCH III study because of the lack of definitive diagnosis, but would follow the study protocol. The more aggressive, three drug protocol would be used: Prednisone, Vinblastine, and Methotrexate.
Child began the initial 6 week protocol of Prednisone, Vinblastine, Methetrexate at PCH on 5/11/02. He is following the high risk arm with Methotrexate. The medication & chemotherapy the child has taken can be found here.
Chemotherapy for the initial 6 week protocol is as follows:
5/11/02 6/14/02: Prednisone daily
5/11/02: Vinblastine, Methotrexate
5/17/02: Vinblastine
5/24/02: Vinblastine, Methotrexate
5/31/02: Vinblastine
6/7/02: Vinblastine, Methotrexate
6/14/02: Vinblastine
6/14/02 - 6/21/02 Prednisone taperDuring the various chemotherapies, the child developed a bad diaper rashes. Nystatin was given. Diaper rashes cleared up but sometimes took a while. Child did not experience any vomiting, but may have been a bit nauseous during treatment. In addition the child's voice became hoarse after treatment. No other side effects were observed.
Child was exposed to Rotavirus (via his roomate) while in the hospital (PCH) on 5/11/02. Mother was sick with Rotavirus on 5/15. Sister (2 years old) on 5/18, Grandmother on 5/21.
Child started vomiting of his medications (mainly prednisone) and loss of appetite starting around 5/16/02. On 5/20, Child began waking numerous times in the night crying and having diahrea. On 5/23 child had more severe symtoms: trouble nursing, not sleeping. crying in pain, arching back and diahrea, vomiting and crying with no tears. Augmentin was stopped. Child became serverly dehydrated on 5/24 and was given IV fluids. On 5/24/02 child also tested positive at PCH for Rotavirus. Child also had some blood in his stools.
On 5/15/02, 4 days after the start of chemotherapy, the child's left eye began swelling again. Reason is unknown?? Child had a mild fever (100.8) and discomfort that was eased by Tylenol. Eye surgeon was consulted again and surgical biopsy options were discussed. No biopsy would be taken of the orbit lesion unless brain lesions forced brain surgery. Child was started on antibiotics (Augmentin) as a precaution on 5/17/02. Eye swelling and fever were gone by 5/19/02.
Blood counts were taken weekly during the initial 6 week protocol. The blood count history can be found here .
An MRI of child's head was done on 5/22/02. Results showed the large lesion in the center had not grown. Growth of others has appeared to slow down but there may be some new lesions not apparent on previous MRI's.
Child is still getting new skin lesions now on his face (temple & eyebrow) and on his left arm. One lesion on the scalp is becoming raised and yellow. The scalp lesions seem to flare up during chemotherapy, looking more marked and red. On 5/25, several of the older lesions began developing the yellow bumps in them. Count of skin lesions was 26.
On 6/2/02 child developed a dry cough and runny nose. Child is alert and playful. Some gagging and vomiting with the cough.
6/10/02 child developed a rash on his stomach, chest, elbows, knees and left arm.
On 6/12/02 child developed two white blisters under his tongue. He was given oral nystatin and the blisters went away. No new skin lesions have appeared.
On 6/16/02 child became fussy and showed a fever which quickly rose to 102.2. He was given Tylenol, which reduced his fever and eased his disomfort. He was taken to the emergency room where his blood counts were taken. Resuls of cultures were negative.
On 6/19/02 an MRI of the brain, a skeletal x-ray and a body CT scan were performed. Review of the scans showed the following:
- Brain scan showed the brain lesions have possibly not grown
- Skeletal X-ray showed no new lesions
- Body CT scan showed a possible spot in the kidney and some thickening of the bowel.
Recommendation from treating physician was to begin another 6 week course (on 6/21) of the same protocol treatment but re-do the scans in 3 weeks and reassess then.On 6/21/02 child became fussy with a rash on his skin. He also had been having some light fevers, a little diarrhea, has lost about a pound and a half, and is neutropenic (low neutrophil counts - see Ben's blood counts). So it was decided to delay start of protocol a few days to start on 6/24 instead.
Chemotherapy for the 2nd 6 week protocol is as follows:
6/24/02 6/??/02: Prednisone three days per week
6/24/02: Vinblastine, Methotrexate
7/1/02: Vinblastine
7/8/02: Vinblastine, MethotrexateOn 6/24/02 patient had a slight ear infection. He was given IV Rosephlyn during chemotherapy course.
As of 6/27/02 no new skin lesions have appeared since 5/25.
TO UPDATE
Other Chemo
Blood Counts
Weight Gain or LossSCANS from 6/21
SCANS from 7/21
Growth
2CdA
