21 year woman facing post ureterosigmoidostomy complications

History taken in April, 2021 when she got admitted with us

A 21 year old woman, hailing from Nalgonda, Telangana presented with the cheif complaints of 

Vomiting since 4 days

Weakness in all her four limbs since 4pm since day  before yesterday 

Patient studied till 10th grade at  Nalgonda government school. She was alright until 1 year back, after which she started finding it hard to control her urine, she started wetting her clothes frequently and her mother would always find her bed wet. She was brought to our hospital where in her mother was told that her right kidney was entirely damaged so they had to place a pipe into her left kidney. She had a gross right hydroureteronephrosis and a moderate left hydroureteronephrosis. A left nephrostomy was done for her along with bladder biopsy, she was on the drain for 6 months after which it was removed. Her bladder biopsy revealed granulomatous picture suspicious of TB. 

The patient was diagnosed with Genitourinary TB and Thimble bladder. She was started on ATT and  ureterosigmoidostomy was performed on  On 10/11/2020. She completed her ATT for 6 months.

She was discharged on Tab Nodosis 1000mg per day initially. It was increased to 2500mg Nodosis per day.

Over the past one year, she says she has lost a significant amount of weight and she has been having loss of appetite since an year. She also tells that post surgery, she has experienced on and off low grade fever. 

5 months post surgery, 5 days back, she started having Vomiting which were green in color, watery in consistency after consuming meals, 3-4 episodes per day for 3 days. 

She was given antiemetics for her vomiting. 3 days later she started experiencing pain in both her lower limbs on walking even to her washroom. At this point she experienced no weakness. 

At around 4pm that day, she suddenly found it difficult to get up from her bed. She found it difficult to lift her left lower limbs after around 20 minutes She developed weakness of her left upper limb. After 20 mins she developed weakness of right lower limb and right upper limb. She started finding it hard to hold her neck.

She however had no complaints of numbness or tingling in her limbs, no band like sensation, she was able to feel her clothes

She was able to urinate and pass bowel with no complaints. She had no complains of sweating, palpitations.

On presentation to us: 

She was a thin built woman

PR - 72bpm

BP - 

100/70mmhg in supine posture

90/70mmhg in standing posture 

 She had hypotonia in all her limbs.

Power in her all her limbs were 3/5

Reflexes were absent in her Triceps, biceps and  supinators.

Her knee reflexes were 3+ with absent ankle reflex. Her plantars were mute.

Her touch, pain, temperature, joint position senses were intact but her vibration was 6 secs upto the ankle level. 

10 secs in all the above joints.

 Mri spine turned out to be normal 

But however they mentioned about a nodule in her left lung measuring 23mm

 Abg shows 

PH was 7.217

Pco2 -26.6

Hco3 - 10.3

Po2 - 93.8

Showing metabolic acidosis with respiratory alkalosis

Anion gap of 23

Delta ratio of 0.8

And her potassium was 3.1

After potassium supplementation 

Her power has improved to -4/5 in her lower limbs and +4/5 in upper limbs sir. 

Her biceps, Triceps, supinators were 2+ today 

Knees were 3+

Ankle reflex was absent

Plantars were flexion on the right side and mute on the left

Mri spine turned out to be normal 

But however they mentioned about a nodule in her left lung measuring 23mm

She presented yesterday 20/9/2021, night with fever since 3 days
Generalised weakness since 3 days
Vomiting since 3 days
Cough since 3 days
Since 3 days she has been experiencing high grade fever associated with chills, no diurnal variation, gets relieved on 
taking paracetamol along with vomiting, 4-5 episodes per day, non projectile, non bilious, non blood tinged yellow in color and non productive cough 
She has generalized weakness and is walking to the washroom with the help of her mother
On presentation to ICU her vitals were 

PR - 120bpm
Bp:120/70 mm hg
RR - 18cpm
Grbs:124
Spo2 - 98% on Room air 
Lungs - Inspiratory crepts in bilateral IAA, ISA
CNS -
HMF intact
Power        Right     Left
UL              3/5       3/5      
LL              3/5       3/5
Reflexes 
B                 2+            2+
T                 2+            2+
S                 2+            2+
K                 2+            2+
A                 +               +
Plantars   Flexion 

Couldn't elicit her power properly sir as she wasn't allowing us to examine her 

Cvs - S1,S2 + 

Per Abdomen- soft
Non tender
Bowel sounds +

Discussion:

Ureterosigmoidostomy is a urological intervention performed to treat various conditions such as invasive bladder cancer, bladder exstrophy, vesicovaginal fistula, or urethral trauma. However, this intervention may lead to several metabolic complications. Here, we report an interesting case with quadriparesis and intestinal paralysis resulting from severe hypokalemia and hyperchloremic metabolic acidosis 

In epithelial cells of the distal colon, the epithelial sodium channel (ENaC) and the potassium (K) channel are expressed and play roles in the reabsorption of sodium (Na) and the secretion of K, which is similar to the kidneys [8]. These actions are regulated by the renin-angiotensin system via the mineralocorticoid receptor. In our case, both the renin activity and the aldosterone concentration on admission were extremely elevated likely due to dehydration, which was caused by fever and anorexia associated with the urinary tract infection. Therefore, this increased activity of the renin-angiotensin system was supposed to induce the secretion of K and the reabsorption of Na in both the kidneys and the distal colon. As a result, the patient developed severe hypokalemia followed by quadriparesis and intestinal paralysis. In accordance with this possibility, both the renin activity and the aldosterone concentration were normalized on day 16 after hospitalization when his hypokalemia had already improved 

In epithelial cells of the sigmoid colon, the chloride ion/bicarbonate ion (Cl−/HCO3 −) exchanger is expressed and is quite activated. As the diverted urinary stream bathes the intestinal lining, Cl− is reabsorbed in exchange for secreted HCO3 −. Thus, the extensive contact time with the colon increases the magnitude of the Cl−/HCO3 − exchange. The loss of alkali coupled with chloride accumulation results in hyperchloremic metabolic acidosis. In our case, prolonged contact time of the diverted urinary stream with the colon due to the hypokalemia-associated intestinal paralysis was likely to cause severe metabolic acidosis 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411658/

A hyperchloremic metabolic acidosis is observed with ileal or colonic segments when they are interposed into the urinary tract. In the bowel, sodium and bicarbonate are secreted in exchange for hydrogen and chloride ions.

When urine is in contact with the bowel wall, ammonia, hydrogen and chloride are also reabsorbed. This results in a chronic acid load. A large bowel surface area, prolonged contact time, patient co-morbidities and pre-existing renal failure contribute to the development of metabolic complications. Patients with reservoirs are at increased risk than those with simple conduits due to the prolonged contact time with urine and the larger surface area.[15] Patients may present with weakness, anorexia and vomiting.

A hypochloremia, hypokalemia metabolic acidosis is seen when the stomach is used. This is a significant problem in patients who are dehydrated or who have renal failure as the bicarbonate excretion is impaired. Patients may present with lethargy, seizures, respiratory complications and ventricular arrhythmias.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3822347/#:~:text=A%20hyperchloremic%20metabolic%20acidosis%20is,and%20chloride%20are%20also%20reabsorbed.