A case report of a newborn who underwent ultra-high enterostomy and intestinal fluid return after necrosis of intestinal volvulus

Introduction

Background

Congenital intestinal malrotation (CIM) is defined as a congenital malformation caused by abnormal intestinal duct development during the embryonic period. CIM generally develops during weeks 6–10 of embryonic development, at which point the digestive tube grows faster than the abdominal cavity. The abdominal cavity thus cannot accommodate the midgut, resulting in abnormal rotational movement centered on the superior mesenteric artery (1,2). However, some researchers believe that the occurrence of this disease is caused by the halting of intestinal rotation in the embryonic development process, which subsequently causes intestinal malrotation (3-5). Approximately 70–80% of patients show clinical symptoms in the neonatal period, with biliary vomiting as the primary clinical manifestation (6). Midgut volvulus is a common complication of CIM. If a child presents with sudden aggravated vomiting or vomiting with fecal mass, it is more likely to indicate volvulus. However, without timely treatment, patients with long time or severe midgut volvulus can develop secondary necrosis of the entire midgut, resulting in a clinical difference of abdominal distension and hematochezia, which has a mortality rate as high as 38% (7).

Abnormal abdominal ultrasound, abdominal radiographs, and gastrointestinal radiography are important clues for early diagnosis. Additionally, bedside ultrasound examination is particularly important, being both rapid and safe. Based on the abnormal positional relationship between the superior mesenteric artery and vein, clinicians can judge whether there is a volvulus, which is typically manifested as a “vortex sign” (8). Once this condition is diagnosed, surgery should be performed immediately.

Objective

Through this case report, we emphasize the importance of early diagnosis and early treatment of CIM to prevent deterioration of the condition. Additionally, we introduce the surgical procedures and postoperative comprehensive management of children with large intestinal necrosis, so as to provide treatment methods for more children with intestinal necrosis. This manuscript is written in accordance with the CARE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-24-214/rc).

Case presentation

The 61-hour-old male was admitted to the emergency department on September 16, 2022, at 0:10 due to vomiting, abdominal distension for 59 h, and rectal bleeding for 6 h. The patient was born G4P1 at 38 weeks and 6 days of pregnancy by cesarean section due to “head pelvic imbalance”. The pregnancy was monitored at a local county-level hospital without any abnormalities being noted, and there was no asphyxia after birth. The amniotic fluid, placenta, and umbilical cord were normal, and the infant’s birth weight was 3,600 g. The mother’s first three pregnancies were all induced abortions due to fetal arrest (cause unclear). The mother was treated with oral Chinese medicine during the current pregnancy (the specific medication could not be explained clearly by the parents). The patient experienced vomiting 2 hours after birth (59 hours before admission), which manifested as yellow-green gastric contents with abdominal distension. The patient initially visited a local hospital, but the condition did not improve, and rectal bleeding occurred. He was subsequently transferred to our hospital. Upon admission, physical examination revealed the following: temperature 36.0 ℃, respiration 64 times/minute, blood pressure 58/34 mmHg, weight 3.18 kg, difficult breathing, yellowing of the skin, rough breathing sounds in both lungs, a heart rate of 172 beats per minute, total abdominal distension, tenderness, rebound pain, muscle tension, disappearance of bowel sounds, and a heel capillary refill time of 4 s. An emergency blood test analysis upon admission revealed the following: white blood cell count of 13.8×10⁹/L and C-reactive protein (CRP) of 79 mg/L. Orthostatic abdominal radiograph revealed uneven intestinal inflation and intestinal obstruction (Figure 1A), and bedside ultrasound revealed poor intestinal rotation and ascites 1.9 cm (Figure 1B). Coagulation function and blood gas analysis were both normal. After admission, emergency surgery was performed immediately.

Figure 1 Abdominal radiograph and ultrasound tests. (A) Orthostatic abdominal radiograph revealed uneven intestinal inflation and intestinal obstruction; (B) bedside ultrasound revealed poor intestinal rotation (red arrow: swirl sign, the specific manifestation of intestinal malrotation in this child). SMA, superior mesenteric artery; AO, abdominal aorta.

During the operation, 20 mL of bloody ascites was observed in the abdominal cavity. The mesenteric root was twisted 360° clockwise, and the small intestine, from the flexor ligament to the ileocecal region, was completely necrotic, dilated, and had poor elasticity (Figure 2A). After resetting the twisted intestine, the color of the intestine was not improved by the external application of warm saline for 20 minutes. However, the child’s general condition was poor and he could not tolerate more operation time, so intestinal exteriorization surgery was subsequently performed (the intestinal were directly disconnected from the junction of necrosis and normal, and 2 cm of the two broken ends were placed outside the abdominal cavity for 48–72 hours. When the circulation of the children was stable, the abdominal cavity was opened again to observe the recovery of the necrotic intestinal; Figure 2B).

Figure 2 The first operation. (A) The small intestine was necrotic, dilated and poorly elastic from the ligament of Treitz to ileocecal junction; (B) intestinal exteriorization surgery.

Comprehensive postoperative treatments were administered including maintenance of respiration and circulation, analgesia, and fluid infusion.

At 72 hours after the first surgery, another exploratory laparotomy was performed, which revealed necrosis of 51 cm of the jejunum (Figure 3A), with recovery of 73 cm of the ileum (Figure 3B). The jejunum was removed (Figure 3C), and surgery for a duodenal and ileal super-high-position intestinal fistula was performed (Figure 3D). To provide better enteral feeding for the infant, we adopted oral feeding with collection of intestinal fluid at the proximal stoma, intestinal fluid infusion at the distal stoma, and micro pumping of milk at the distal stoma as post-operative management. Two Frey’s urinary tubes were inserted into the proximal and distal stoma and were connected with a syringe (Figure 4A).

Figure 3 The second operation. (A) The jejunum is necrotic; (B) the ileum is recovered; (C) a 51-cm jejunum was excised; (D) a duodenal and ileal super high position intestinal fistula.

Figure 4 Intestinal fluid back transfusion. (A) Two Foley catheters were inserted into the proximal and distal stoma; (B) two Foley catheters were inserted into the proximal and distal stoma connecting with a syringe; (C) secretory fluid was collected every 3 hours from the proximal stoma and pumped through the distal stoma.

Secretory fluid was collected every 3 hours and pumped through the distal stoma (Figure 4B,4C). Oral feeding initially comprised 5–7 mL/3 hours, but the amount of proximal stoma was relatively large, and the dose was 120 gradually adjusted to 2 mL/3 hours. Milk was also pumped through the distal stoma using two pumps and two tubes. All procedures were performed strictly aseptically to prevent infection. The amount of milk at the distal ostomy site was dynamically adjusted according to the patient’s intestinal tolerance. Initially, breastfeeding was chosen, and the proportion of eosinophils (15.3–25.8%) was found to be significantly higher than normal in dynamic monitoring. Later, a combination of breast milk and deep hydrolyzed formula milk was chosen for feeding, and parenteral nutrition was provided according to the existing guidelines (9,10).

Eighty-four days after the second surgery, the third surgery (fistula closure) was performed following evaluation of the patient’s various conditions. The intraoperative findings were as follows: the length from the distal ileum to the ileocecal region of the fistula site was measured as approximately 90 cm, and no stenosis was observed (Figure 5A). Duodenal ileal anastomosis was performed (Figure 5B). Milk feeding was initiated 5 days after the closure of the fistula, and full intestinal feeding was quickly achieved. During hospitalization, the patient had three nosocomial infections, each with fever, and abnormal leukocyte and CRP. The first blood culture was positive for Staphylococcus epidermidis, which was considered peripherally inserted central catheter (PICC)-related bloodstream infection (10.25). the second blood culture was negative, but the stool was positive for occult blood, considered intestinal infection (11.14). The third blood culture was positive for Klebsiella pneumoniae subspecies, which was considered PICC-related bloodstream infection (12.29). Antibiotic use was adjusted based on clinical and laboratory tests (Figure 6 and Table 1), and the PICC was removed accordingly. The patient was hospitalized for 128 days, at which point he had achieved self-feeding of 55 mL every 2 hours and a weight of 5.8 kg, with stable vital signs and normal bowel movements. The patient was discharged after clinical recovery. Upon discharge, regular follow-up visits to the outpatient department were conducted to assess growth and development. At 15 months of age, the patient weighed 9.8 kg (P15–P50), had a body length of 79 cm (P50), and a head circumference of 46.8 cm (P50), whereas his Gesell developmental scale test results were normal (Figure 7).

Figure 5 The third operation. (A) From the distal ileum to the ileocecal region of the fistula site was measured as approximately 90 cm and no stenosis was observed; (B) duodenal ileal anastomosis.

Figure 6 Laboratory tests. (A) WBC test results; (B) CRP test results. The red boxes mean the values of WBC and CRP at the time of three nosocomial infections. WBC, white blood cell; CRP, C-reactive protein.

Figure 7 Follow-up after discharge: developing normally. This image is published with consent from the parents of the patient.

All procedures performed in this study were in accordance with the ethical standards of the Ethics Committee of the Baoding Hospital of Beijing Children’s Hospital, Capital Medical University (No. 202185) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Vomiting is the most common clinical manifestation of neonatal intestinal malrotation. However, patients with midgut volvulus may also present with other clinical symptoms, such as abdominal distension and hematochezia (11,12). In addition, attention should be paid to the difference between intestinal malrotation and duodenal atresia, duodenal stenosis, and annular pancreas. Among these three symptoms, the chronological order of vomiting is duodenal atresia, duodenal stenosis, annular pancreas and intestinal malrotation, with the highest incidence occurring in intestinal malrotation (13). Imaging examination modalities to facilitate diagnosis include abdominal X-ray, abdominal computed tomography (CT), abdominal B ultrasound, and gastrointestinal contrast (14). Plain X-ray films show “double bubble” and “three bubble” signs, predominantly occurring in the third to fourth segment of the duodenum. Abdominal CT and abdominal B-ultrasound examination generally show the superior mesenteric vein moved in front of the superior mesenteric artery (15,16). Yang et al. (17) previously reported that fetal B-ultrasound is associated with specific findings for intestinal malrotation with midgut volvulus, among which the “swirl sign” and “twine sign” are considered direct signs. Because the diagnosis and treatment of this disease are commonly delayed, serious complications, such as intestinal necrosis, shock, and short bowel syndrome after the operation, are common. Some scholars have described this as a “potential danger” in the body (18).

In this study, the necrotic area of the intestinal canal was large and the general condition of the newborn was poor at admission. In order to shorten the operation time and improve the condition of the child quickly, it was also a chance to reverse the necrotic intestinal canal. The first operation was performed with intestinal exteriorization surgery, and various treatments were given after operation to ensure the stability of multiple organ functions so as to restore the necrotic intestinal canal as far as possible. A second operation was performed 72 hours later, with mixed results. Although the jejunum was necrotic, the ileum remained intact. Direct intestinal anastomosis is impractical and inevitably leads to complications; as such, the jejunum could only be excised while duodenal and ileal double-lumen ostomies were performed. However, the next difficulty we encountered was whether to perform ultra-high position enterostomy, followed by how to feed the patient and care for the ostomy. We reviewed the relevant literature and found only a few reports of this kind of ultra-high position enterostomy. Zhou et al. (19) reported 14 cases of intestinal fluid infusion treatment following neonatal colostomy, including one case of intestinal malrotation accompanied by midgut torsion (the other cases were with intestinal atresia, meconium peritonitis, meconium intestinal obstruction, and necrotizing enterocolitis). The fistula site was located 15 cm away from the Treitz ligament, which was still further away from the fistula site in this child (close to the Treitz ligament). Intestinal fluid reinfusion was initially described by Puppala in 1985, and has since been shown to be safe and effective (20-23); however, relevant studies are scarce. With reference to this method, we collected the secretion from the proximal stoma and pumped it into the distal intestinal canal through the Foley catheter. The reasons for this choice were as follows: the texture of the Foley catheter is soft, the model can be selected, the water sac plays a fixed role, and the ostomy secretions can be prevented from corroding the surrounding skin. Aseptic operation should be strictly controlled in the process of collection and pumping to prevent infection. In addition to surgery, comprehensive management, such as circulation maintenance, nutrition assurance, and anti-infection treatment, is also an important guarantee to ensure the successful treatment of this child in this case, the neonate was maintained in the neonatal intensive care unit (NICU) from March 16 to July 13, with daily rounds of surgeons’ discussion on treatment options. In addition, as the infant was the first child born to the parents, who had lost three pregnancies by abortion due to fetus suspension, the parents showed great determination and strong cooperation. A few days before the patient was discharged, he was transferred to the surgical ward to allow his parents to adopt more quickly to his care. Yang et al. (24) previously reported 48 cases of neonatal intestinal malrotation admitted to the Department of Neonatology, Kaifeng Children’s Hospital, Henan Province, from 2012 to 2016. Among these 48 cases, four were intestinal necrosis, surgical reduction, and intestinal refolding of the abdominal cavity, of which two patients underwent a second laparotomy 48 hours later. In that study, most of the intestinal blood circulation recovered in two patients, with intestinal resection and intestinal anastomosis eventually being cured, while the other two cases of intestinal necrosis died of shock and multiple organ failure.

Martinez et al. (25) previously reported that in a registry of 119 pediatric participants at an authorized facility in the United Kingdom, 57.1% were diagnosed with intestinal malrotation in the neonatal period. In this cohort, 70% of the children had volvulus at the time of diagnosis, of whom one-third underwent bowel resection during the initial surgery, resulting in 23.4% being diagnosed with short bowel syndrome; more than 60% of the patients had gastrointestinal symptoms that persisted for a full year after the index surgery, of whom more than 40% had seen a dietitian and 17% had seen four or more gastroenterologists. However, our patient has been developing and eating well since discharge, with no requirement for repeated clinic visits or re-hospitalization.

Conclusions

The surgical treatment of neonatal intestinal malrotation with necrosis of the midgut volvulus is critical to prevent poor prognosis. As such, prenatal screening is recommended. Once diagnosed, emergency surgery should be performed as rapidly as possible. Selection of an appropriate surgical method and position of the stoma, correct application of intestinal fluid reinfusion, comprehensive perioperative management, a collaboration of physicians, surgeons, and nursing teams, as well as the cooperation of parents, are all keys to the successful treatment of this disease.

Acknowledgments

We would like to express our gratitude to the patient and his family, as well as for the help of all the physicians in the course of the medical care.

Funding: This work was supported by a grant from Baoding Science and Technology Project of Hebei Province (No. 2041ZF132).

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-24-214/rc

Peer Review File: Available at https://tp.amegroups.com/article/view/10.21037/tp-24-214/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-24-214/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the Ethics Committee of the Baoding Hospital of Beijing Children’s Hospital, Capital Medical University (No. 202185) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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