Nepal: Kanti Childrens Hospital

Jebina Diagnosed

Peutz-Jeghers Syndrome (PJS)

Jebina, a 6-year-old girl from Baneshwor, was admitted to the Pediatric Surgery Department of Kanti Children’s Hospital on November 13, 2017 with a provisional diagnosis of intussusception, an intestinal condition. According to the patient’s mother, Jebina was brought to the Emergency Unit with complaints of abdominal pain and nonbilious vomiting that lasted 2 days. She had history of recurrent colicky pain since the age of 6 months. She had no other history of previous diseases, but a significant family history of disease.

On admission, Jebina’s vitals were within the normal ranges. Baseline laboratory tests and an abdominal ultrasonography (USG) were done. Her complete blood count indicated leukocytosis with a high white blood cell count of 13,200/cmm. The abdominal USG showed signs of intussusception in the upper left abdomen. Her other blood biochemistry results and urinary analysis were normal.

As a result of her physical examination results, Jebina underwent an investigative surgical procedure called an explorative laparotomy, and an intussusception was found. However, the intussusception could not be reduced manually and a polypoid mass in the small intestine was the lead point (the cause of the intussusception). During the examination, three polyps were found at 5cm, 8cm, and 10cm. The large bowel was normal. The intussuscepting segment of the bowel with the three polyps was excised. Surgeons removed 10-15cm of the bowel, performed end-to-end anastomosis to repair it. The specimen polyps were sent for examination.

Jebina was kept in the postoperative ward for 3 days. During her stay, she was re-examined and found to have a mucocutaneus hyperpigmentation, dark spots on her mucus membranes. She began feeding 2 days after admission and was transferred to the General Surgical Ward. Jebina was discharged 5 days after admission with postoperative care instructions and a follow-up appointment scheduled in the Surgical Outpatient Department (SOPD). She was seen in the SOPD with no complaints.

The examination of the polyps revealed they they were Peutz Jegher type polyps, a type of gray-brown noncancerous growth, with the largest one measuring 4x2x1.5cm. Jebina’s Peutz Jegher’s Syndrome diagnosis was confirmed on the basis of 3 hamartomas (noncancerous growths), mucocutaneous hyperpigmentation, and the polyp examination. The patient was counseled about the disease and advised to come in for regular follow-ups.

Arnav Shrestha

Treated for Infantile Hypertrophic Pyloric Stenosis (IHPS)

Arnav Shrestha, a 2-month-old boy from Banepa, was admitted to the Pediatric Surgery Intensive Care Unit at Kanti Children’s Hospital on November 2, 2017 with a provisional diagnosis of Infantile Hypertrophic Pyloric Stenosis (IHPS), an intestinal muscular condition which causes the obstruction of the gastric outlet. According to the patient’s mother, the baby was delivered via caesarean section in the hospital after a full-term pregnancy, and the delivery was uneventful. Arnav’s birth weight was 3.6kg, and he was feeding well. As the breast milk was not sufficient, formula was added to his diet when he was 7 days old.

At the age of 5 weeks, Arnav started vomiting persistently and severely after each feeding. The baby was taken to a physician for treatment. An antiemetic drug was prescribed to address his vomiting, and his mother was advised to burp him after every feeding. The patient’s mother also reported that she visited three private hospitals within 15 days, as the baby’s symptoms had not subsided. Finally, Arnav’s parents decided to take him to Kanti Children’s Hospital.

The patient was brought to the Emergency Unit and had a consultation with the on-duty RMF surgical doctor. He weighed 2.7kg and was severely dehydrated but conscious and showed no abdominal distension. Apart from scanty urinary output, the baby presented no other symptoms, and there were no other systemic abnormalities. There was no visible intestinal peristalsis (involuntary movements of the muscles in the digestive tract) but the doctor was able to feel an abdominal mass and thus began to suspect IHPS. The patient underwent all baseline laboratory tests as well as an abdominal ultrasound scan.

Arnav was admitted to the SICU and withheld from consuming food or fluids. His hematocrit, random blood glucose, urea, and creatinine levels were within the normal range except for serum electrolytes; his sodium level was low at 119 mEq/L and his potassium level was also low at 2.7mmol/L. Arnav’s dehydration as well as his sodium and potassium deficit were treated with an intravenous solution, and his fluid input and output continued to be closely monitored. An abdominal USG revealed a lesion in the upper central abdomen with diameter near 12mm and a wall thickness of 6mm, and his pyloric canal, which connects the stomach to the intestines, was abnormally long at nearly 17mm, which is a key diagnostic for IHPS.

The patient was prepared for his operation on the 3^rd day of admission. Perioperative findings confirmed IHPS. A surgical procedure called a Ramstedt Pyloromyotomy was performed, and the surgery went as planned. Arnav was kept in the SICU for 2 days on IV fluids, antibiotics, and analgesics. He started feeding 2 days after the surgery and was transferred to the general Surgical Ward. The patient was discharged 4 days after surgery with postoperative care instructions and a follow-up scheduled in the SOPD. The patient was seen in the SOPD with no complaints.

Rishal Poudel

Infantile hypertrophic Pyloric Stenosis (IHPS)

Rishal Poudel, a 13-month-old boy from Pokhara, was brought to Kanti Children’s Hospital on December 7, 2017. He was referred from Ishan Hospital to Kanti Children’s hospital for PICU management. Rishal was admitted to the Emergency Unit with vomiting and loose stool. According to the parents, he was feeling well until 2 days before admission when he began vomiting after each feeding and having diarrhea. They also reported one instance of abnormal body movement.

When Rishal was admitted to the Emergency Unit, he was unresponsive. All the necessary laboratory tests were done, and he was started on antibiotics. After a consultation in the PICU, Rishal was transferred to the PICU where further testing revealed a low GCS (which indicates high coma severity) and acute kidney injury (AKI), and the patient was intubated.

3 days after admission, Rishal was still unresponsive, so a head CT scan was done revealing cerebral edema, or swelling of the brain. All the basic investigations were done as well as a lumbar puncture (LB) to test the spinal fluid. Still, the child was not showing any signs of recovery and not responding to verbal stimulation and light. An eye consultation and an ENT consultation were also scheduled. A brain MRI revealed cytotoxic edema, an excess of water which causes the brain cells to swell. In response to the MRI results, the patient’s antibiotics and other medications were changed or adjusted.

On the 13^th day of admission, Rishal was extubated and shifted to a paying ward. During his stay there, he was fed through an NG tube and underwent physiotherapy. He soon showed active movement of his arms and legs but still could not feed orally. After 9 days in the paying ward, the hospital suggested the parents take the child home but continue feeding through an NG tube. The child was discharged under oral antibiotics and the parents were asked to follow up in the Outpatient Department (OPD) and advised to continue encouraging oral feeding. After 1 week, Rishal was brought to the OPD for a follow-up which revealed that the child was feeding orally again and continuing physiotherapy.