Taylor Harrington, NICU Nurse.
This case study follows Adrienne, a premature infant whose Neonatal Intensive Care stay was complicated by bowel perforations, significant faecal peritonitis and large volume turbid ascites, secondary to a milk curd obstruction. She required a beside laparotomy in the Neonatal Intensive Care Unit (NICU) and her postoperative journey was very difficult, complicated by hypovolemic shock, an aortic clot, a wound dehiscence with a fistula formation, and a further deterioration with septic shock leading to reduced blood flow to her small bowel.
Adrienne was born prematurely at 23 weeks +5 days gestation weighing 645 grams. Her weight was in the 92nd centile for her gestion and sex. She was intubated at birth for poor respiratory effort and given one dose of surfactant in the delivery room. She was transferred to NICU where she continued to need escalating respiratory support to High Frequency Oscillation Ventilation (HFOV) and received two further doses of surfactant. Maternal placental swabs were positive for Enterococcus Faecalis, and Adrienne received 36 hours of antibiotics after her birth. The antibiotics were discontinued at that time because Adrienne’s blood cultures remained negative.
Although Adrienne’s lungs were still developing and she was requiring significant ventilation support, she was slowly grading up on gastric feeds. She had a brief set back on day 5 of life, when she developed ventilator associated pneumonia, but was treated with another course of antibiotics. She passed her first meconium on day 7 of life and her abdomen was examined to be soft and non-tender.
On day 11 of life, Adrienne deteriorated and had increased abdominal distention. Necrotising enterocolitis is always a concern from premature babies with abdominal distention, so blood cultures were taken, and she was started on antibiotics to cover for gut concerns. Her feeds were stopped, and she was placed nil by mouth (NBM) at this time. Xray films revealed reduced gas in the bowel loops but no free air. She continued to deteriorate over the next few days and on day 14 of life she suffered hypovolaemic/septic shock. She required multiple saline boluses and significant inotropic support of adrenaline, noradrenaline, dopamine and hydrocortisone to keep her blood pressures within normal ranges for a baby of her gestation.
On day 15 of life, Xray films revealed fluid filled bowel loops and abdominal wall oedema, with reduced central gas and looked concerning for a perforation. At this time, Adrienne was prepped for transfer to a tertiary surgical facility where she would undergo a laparotomy to determine what was causing her extreme abdominal distension. She was only 25 weeks +5days corrected gestational age and weighed 790grams.
Once Adrienne arrived in the surgical NICU, it was evident that she was too unstable to transfer down to theatre. She was requiring significant ventilation support, inotropic support and was very unstable during the short transfer from the referring hospital. The paediatric surgeons prepared their team for a bedside laparotomy in NICU.
Surgery commenced within 30 minutes of Adrienne’s arrival. During the laparotomy, the surgical team encountered large volume turbid ascites and significant faecal peritonitis. There were two perforations in the ileum located 5 centimetres apart. There was 50 centimetres of small bowel from the duodenojejunal flexure to the site of the perforations, a 10 centimetre section containing the perforations that was resected and then 25 centimetres of terminal ileum remained intact. The terminal ileum was obstructed with a large milk curd obstruction, which was milked out from the bowel. The bowel appeared otherwise healthy and viable with normal rotation. The surgical team washed out Adrienne’s abdominal cavity with warm saline and formed an ileostomy and a distal mucous fistula. Her abdomen was closed, and she began her long recovery from a milk curd obstruction.
Adrienne remained unwell for a few days postoperatively but was eventually weaned off her inotropes and was making improvements. She still struggled with her underdeveloped lungs but was able to be transitioned onto conventional ventilation and eventually trialled on continuous positive airway pressure (CPAP). Unfortunately, she had two unsuccessful attempts at extubation in the first three weeks post-op. On day 38 of life, postoperative day 23, Adrienne was once again unsuccessfully extubated to Non-Invasive Positive Pressure Ventilation (NIPPV). On day 45 of life (postoperative day 30) the neonatal team decided to start Adrienne on a course of dexamethasone to slow down the progress of her evolving chronic lung disease and wean her oxygen requirement. It was around this time that there was an incidental finding of a large aortic clot that was discovered during a routine echocardiogram looking to see if Adrienne’s Patent Ductus Arteriosus (PDA) was causing her respiratory issues. She required a heparin infusion for this for 4 weeks.
While Adrienne’s lung function and respiratory complication remained unstable, she was making improvements elsewhere. On day 21 of life (postoperative day 6), the surgical team was happy for Adrienne to start feeds. She was started on 1 ml of expressed breast milk (EBM) every 4 hours. By postoperative day 10 she was tolerating 4 ml of EBM via continuous orogastric feed. At first, she had very minimal output from her active ileostomy, but the team was happy to keep feeding. As she approached full enteral feed on postoperative day 16 (day 31 of life), her stoma outputs remained consistently less than 10 ml/kg/day, but still an improvement. Although Adrienne was tolerating her enteral feeds at this time, her central line remained in situ for her heparin infusion with a small amount of intravenous nutrition running through as well to optimise her growth while she still had line access.
Her stoma output was rather unusual, sometimes going from no output for several hours to having all 10ml/kg/day in one hour. Her chyme was also unusual in its consistency, varying from very thick and formed, to watery looking milk. She had periods of significant abdominal distention and tenderness that were also unexplained. Due to these frequent episodes, Adrienne was having serial x-ray pictures. On postoperative day 9, she had a relatively symmetrical gas pattern. This remained consistent until postoperative day 26, where the gas pattern remained similar, but now there was the development of some air fluid levels. On day 49 of life (postoperative day 34), at a corrected gestation age of 30 +5, Adrienne became acutely unwell with worsening abdominal distention and deterioration requiring an escalation to HFOV in 100% Fi02. She also required significant inotropic blood pressure support once again. X-rays at this stage revealed a more asymmetric gas pattern with interloop fluid and bowel wall thickening. There was no pneumatosis or free air. Adrienne’s stoma stopped working completely and she was once again placed nil by mouth and on antibiotics for gut cover. During this time, she never vomited or had bilious/bloody gastric output.
The team awaited an urgent ultrasound scan which showed generalised bowel wall and mesenteric thickening/oedema. There was good doppler flow in the mesentery, but no significant flow within the bowel wall. Radiologically, this was very worrying for under perfused or frankly ischaemic gut. There were concerns that this global change in the small bowel was a result of Adrienne’s hypotension, gut oedema or the possibility of NEC. Through the multiple tests and investigations after her deterioration, an answer emerged to explain her sudden collapse. Her peripheral blood culture came back positive for Klebsiella and she was in septic shock. She was started on antibiotics to cover this bacteraemia and was able to start the right treatment to get her on the road to recovery. She took a long time to recover from her sepsis and this was a very scary time for her family. There were many days that her family thought that they were never going to take Adrienne home. However, she was a resilient little girl and overcame her sepsis and started to wean off all the extra support.
By day 65 of life, she was started slowly on enteral feeds and by day 78 of life she was back to full continuous enteral feeds of EBM via her orogastric tube. She was feeling much better. She was able to be successfully extubated to CPAP and eventually even progressed to High Flow Oxygen.
On top of everything else, Adrienne suffered from a wound dehiscence in her early days post operatively which healed well but resulted in the formation of an enterocutaneous fistula. The positioning of this fistula was right near the base of the stoma just at skin level and when she would strain or wiggle, the stoma would retract slightly making it very difficult for the stoma appliance to stay in situ longer than 4-12 hours.
At 33+3 weeks corrected gestation (day 75 of life), Adrienne was started on chyme reinfusion therapy using The Insides® Neo. She was postoperative day 70 and weighed 1900g at that time.
Adrienne had already been fitted with the ‘Hollister Pouchkins Bag’ however, due to the nature of her stoma, she was needing multiple daily appliance changes. With her next stoma bag change, the device was fitted and ready for use. The nursing team used a size 8 Fr gastric tube for refeeding which they assembled on the device and inserted into the distal fistula. It was secured at the 5 cm marking on the outside of the bag with the provided clip. The chyme was withdrawn from the bag every 4-6 hours and reinfused using a syringe pump over the next 4-6 hours.
When Adrienne was initiated on chyme reinfusion therapy, she was receiving approximately 75% enteral feeds of expressed breast milk and 25% parenteral nutrition. Her feeds had been pulled back slightly due to static growth and the team decided that while she had central line access, they may as well optimise her growth with parenteral nutrition until the line was no longer needed.
Her outputs remained very small, but all of her chyme was refed into her distal mucus fistula, and she had her first stool in her nappy by day 4! By day 84 of life, corrected gestational age 35+5 weeks corrected, Adreinne was back on full enteral feeds. Her central line remained in situ to finish her course of antibiotics for her Klebsiella infection, so a very small dose of parenteral nutrition was being infused to keep the vein open. However, when her course of antibiotics finished on day 94 of life (37+1 weeks corrected), the line was removed, as the team was happy with her growth.
Chyme reinfusion administered via The Insides® Neo went smoothly for Adrienne. As time moved on her output went from minimal, to over 50 ml/kg/day of very watery/milky chyme and no evidence of reflux from the distal stoma. The neonatal team in conjunction with a neonatal dietician decided to try Adrienne of a special formula called Pepti-Jr that is specifically made for infants with gut malabsorption. That seemed to help, and her high outputs eventually ceased.
The biggest difficultly for Adrienne was the number of times per day her bag was leaking. The little fistula caused many problems for the nursing team and became a frequent source of frustration. As a result, the decision was made to perform a reanastomosis so Adrienne could continue to grow and develop without the constant interruption in her sleep cycles to change her ostomy appliance. After all, preterm babies do their best growing and brain development when sleeping.
Despite the frequent bag changes, Adrienne was thriving with chyme reinfusion therapy. For the first time in her life, her growth chart was starting to normalise.
On day 104 of life, 37 +6 week corrected, weighing 2850g grams, Adrienne was ready for her reanastomosis. This time she was stable enough to make the journey down to theatre rather than a bedside operation. She spent a total of 29 days using The Insides® Neo and had gained a total of 950 grams! This was an average of 33 grams per day.
Adrienne’s parents were relieved when her surgery finally went ahead. It had been delayed multiple times due to more urgent cases coming in, and poor Adrienne had fasted three times for her reanastomosis. Anyone who knew Adrienne knew that she was NOT happy when she was not fed! Her surgery finally went ahead as planned and her parents could start planning for the journey back to her local hospital and ultimately, home. The time she spent in the tertiary surgical NICU had been difficult for Adrienne’s Family. They lived far away from the hospital and had a very young child at home that was still breastfeeding. They were looking forward to a much easier life outside of hospital.
Adrienne’s surgery went to plan, and the surgeons were able to resect the small portion of bowel that had formed the fistula. Postoperatively, she was transferred back to NICU, ventilated and sedated with instructions to be extubated back to high flow once awake. She remained NBM until the return of gut function. She started feeds on postoperative day 2 and was titrated up to full enteral feeds by postoperative day 8. She quickly recovered from her reanastomosis and she had her first bowel motion on postoperative day 4.
On day 114 of life, only 10 days after her reanastomosis surgery, Adrienne was discharged from the surgical team and was transported back to her local hospital. Her parents were ecstatic they were finally able to see some light at the end of the tunnel.
Adrienne’s growth chart is difficult to interpret due to her multiple periods of being ill and oedema.
Around 29-31 weeks gestation, you can clearly see fluctuations in her weight bouncing between the 25th and 50th centile as she had periods of being very oedematous and then losing weight secondary to fluid shifts and furosemide infusions.
Around 31-32 weeks when she was feeling better and started back on enteral feeds, you can start to see her growth pattern normalise again and track along the 50th centile. At 33 weeks she had a drop to just below the 25th centile when she was transitioned to more enteral feeds. At this time the team pulled back her feeds once again and made used of the central line she required for antibiotics and started parenteral nutrition. Chyme reinfusion was started around 33 weeks to optimise her growth.
With the use of chyme reinfusion, using The Insides® Neo, you can see that she does not drop under the 25th centile, but rather cross it and tracking along slightly above the 25th centile. She continues this trend, flirting closer to the 50th centile at times before her reanastomosis at nearly 38 weeks.
After her reanastomosis her growth skyrockets and she finally crosses that 50th centile line again at 40 weeks. Presumably this could be due to the fact that her ostomy appliance was leaking so frequently due to her fistula. This growth pattern clearly shows that she was losing a lot of chyme, and therefore nutrition, secondary to the ostomy appliance leaks. Her early reanastomosis was the right call.
After transfer to her home hospital, Adrienne had a slight set back when she contracted Rhino Virus and needed a few days of CPAP. She also had some worsening abdominal distention at that time and x-rays were suspicious of a possible obstruction at the level of her anastomosis. However, she recovered after 24 hours on CPAP and her abdominal distention resolved. She remained on high flow therapy for a few extra days but once fully recovered from her cold she was self-ventilating for the first time in her life!
She continued to make progress on her breathing and oral feeding and finally on day 150 of life, 46 +2 weeks corrected, weighing 4505 grams, she was discharged home with her family!
Adrienne was born extremely premature and became unwell with a milk curd obstruction and two subsequent bowel perforations requiring a laparotomy and stoma formation within the first three weeks of life. She had multiple postoperative complications and was very unwell for the main duration of her intensive care stay. She spent a total of 29 days using The Insides® Neo. During this time, she had adequate weight gain and a successful removal of her central line. She spent a total of 150 days in intensive care before being discharged home with her family.
Case Abstract detailing the use of The Insides® System with an Enterocutaneous Fistula patient, with the main article published in Journal Colorectal Disease.
The following two case studies describe how critical thinking and stomal therapy skills were challenged during the management of two patients with complex enterocutaneous fistula (ECF).
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