CASE 1
Mattia is a 6-year-old boy with no previous medical history. He came for
intermittent cramp-like abdominal pain in the last 48 hours, no fever,
vomiting or diarrhea. Since some signs of inflammation in the appendix
were found at an initial abdominal ultrasound scan, Mattia was sent to
Pediatric Emergency Room (PER). Physical examination showed signs of
acute abdomen. Therefore, he underwent a video-assisted trans-umbilical
appendectomy with histological confirmation of acute appendicitis. After
an initial clinical improvement, that allowed him to start walking
independently on postoperative day four, we observed a progressive
exacerbation of abdominal pain associated with difficulty in maintaining
the sitting position.
The next day we also noticed an intermittent tenderness in the lower
limbs and inability to keep the upright position and nocturnal enuresis.
During a neuropsychiatric evaluation, the patient reported that he did
not feel the urge to urinate and refused to leave autonomously the
wheelchair. The neurological evaluation highlighted the presence of
hypotonia and hyposthenia of the lower limbs, abolished patellar
osteotendinous reflexes, abnormal plantar reflex in bilateral toe
extension (Babinski sign). An electroneurography, a lumbar puncture and
a magnetic resonance imaging (MRI) have been recommended to complete the
diagnosis. The neurographic study showed only an asymmetry between left
and right. Lumbar puncture showed elevated protein level
(>700 mg/dl) in the cerebrospinal fluid, which supported
the suspect of a Guillain-Barre syndrome (GBS). Therefore, a treatment
with intravenous immunoglobulin at a dose of 0.5 g/kg/day was started.
The following day the clinical picture of the patient appeared
unchanged. An encephalic and spinal MRI was executed, showing an
epidural intracanal tissue located from D7 to D11, associated with
blurred signs of medullary suffering (Figure 1A and 1B).
An urgent surgical decompressive laminotomy on 5 levels was performed,
with removal of most of the pathological tissue (Figure 1C and 1D). The
histological examination reported a pre-B lymphoblastic lymphoma. Mattia
has been subsequently transferred to the Onco-hematology department to
undertake a specific chemotherapy treatment for the underlying disease.
High-dose steroid therapy with methylprednisolone succinate was
prescribed.
After surgery Mattia showed a persistent severe hypotonia in the lower
limbs, absence of spontaneous movements with weak patellar and Achilles’
osteotendinous reflexes and recovery of tactile sensitivity.
During the hospitalization, the initial paraparesis gradually improved
thanks to a targeted rehabilitation program. Distal motor deficiency was
associated with intestinal dysmotility, anal sphincter disfunction and
spastic neurogenic bladder. The initial difficulty in perceiving the
stimulus to defecate and the anal sphincter atony has progressively
improved, until resolution. Neurogenic bladder, characterized by an
increase in daily urinations with a small amount of urine and mintional
urge, was treated by oxybutynin. Intermittent catheterizations were
performed six times a day as prophylaxis of urinary tract infections.
The patient was found to have 3 asymptomatic bacteriuria, from catheter
urinary sample cultures, treated according to the antibiogram.
Currently, the number of daily urinations has partially reduced and
mintional urge has improved.