A 4.5-year-old boy initially presented on April 9, 2024, with abdominal pain. Ultrasonography revealed gas-filled, dilated bowel loops. His clinical condition improved after symptomatic management. On April 29, abdominal pain recurred and was accompanied by vomiting, and conservative management proved ineffective. The patient was transferred to the Children’s Hospital on May 2 with diagnoses of sepsis and bowel obstruction and was admitted to the intensive care unit (ICU). Exploratory laparotomy on May 3 revealed a massive mesenteric lymphangioma (approximately 10.3 × 6.5 × 12.3 cm) that had caused small bowel torsion with resultant ischemic necrosis. Consequently, the majority of the small intestine along with the ileocecal region was resected, and an end-jejunostomy was created from the approximately 20 cm of remaining viable jejunum. Postoperatively, antimicrobial therapy and nutritional support were administered. However, the patient’s nutritional status progressively deteriorated, developing jaundice and liver dysfunction. On June 10, a wound infection complicated the clinical course, necessitating incision and drainage. A cloudy, pale-yellow exudate was noted around the stoma site.

The patient had mild jaundice. Subcutaneous fat was decreased in the limbs and abdominal wall. The abdomen was scaphoid and soft. A stoma was visible in the left abdomen with ulcerated peristomal skin. A pinpoint fistulous tract was noted near the umbilicus. An old surgical incision approximately 7 cm long was visible below the umbilicus, with a subcutaneous drain in its center, draining turbid yellow fluid.

The patient’s clinical course was complex and critical, from initial onset through emergency surgery and small bowel transplantation to six months postoperatively. To illustrate the disease progression, key interventions, and major clinical events in chronological order, a detailed timeline is summarized in Table 1.

Short bowel syndrome; cholestatic hepatitis; severe malnutrition with cachexia; status post jejunostomy; abdominal wall infectious sinus tract; status post small bowel resection.

• Phase One: Control of Intra-Abdominal Infection

A comprehensive CT scan was performed after admission to exclude lymphangiomas at other sites.

Surgical record: The original surgical incision was debrided, and purulent fluid was evacuated. A purulent sinus tract, indicative of an intra-abdominal abscess with an abdominal wall fistula, was identified. The incision was extended to access the abdomen, followed by debridement of the abscess and copious irrigation. A single abdominal drain was placed and secured. After achieving hemostasis and closing the abdomen, the abdominal wall wound was irrigated, debrided, and sutured. A 16-gauge neurosurgical drain was also secured at the stoma site.

• Phase Two: Transitional Management for Short Bowel Syndrome

The patient was transferred to the general ward postoperatively with continuous monitoring of vital signs. On postoperative day 15, he developed an unexplained high-grade fever (peak 40 °C) without abdominal distension, pain, or other discomfort. The stoma was well-perfused, with yellow, loose stools in the stoma bag. Physical examination and abdominal ultrasound were unremarkable. The patient had recurrent fever episodes accompanied by profuse sweating, requiring increased fluid resuscitation. Elevated inflammatory markers led to empirical cefoperazone administration, which reduced the fever. Clinical evaluation identified no pulmonary or abdominal source of infection. Potential sources included prolonged use of a peripherally inserted central catheter (PICC), extended high-concentration parenteral nutrition, and a history of repeated thrombolysis. Following PICC removal, tip culture revealed Gram-negative bacilli, prompting initiation of meropenem (20 mg/kg every 8 h) and subsequent clinical improvement. A nutritionist was consulted for nutritional optimization.

• Phase Three: Small Bowel Transplantation

(1) Indications for Transplant Assessment: During the transitional period, the patient developed severe hypokalemia (serum potassium 2.38 mmol/L) with convulsions, and hypoglycemia (fasting blood glucose 1.9 mmol/L). These life-threatening complications stemmed from inadequate nutrient absorption due to the ultra-short remnant bowel, which failed to meet the patient’s metabolic and developmental demands. Consequently, small bowel transplantation was deemed imperative. Following a multidisciplinary team assessment, the patient was listed for transplantation.

(2) Surgical Record: The graft, a 3.8-meter small bowel segment from a brain-dead donor, was allocated through the China Organ Transplant Resource Sharing Computerized System (COTRS) and procured under standard protocols with ethical committee approval. At laparotomy, severe intra‑abdominal adhesions were encountered. The native small intestine remnant measured approximately 20 cm distal to the Treitz ligament. After extensive adhesiolysis, the left upper quadrant stoma was taken down. The donor’s vascular pedicles were trimmed, with bleeding branches ligated, and the graft was flushed and stored in ice-cold preservation solution (cold ischemia time: 90 min). The recipient’s infrarenal aorta and inferior vena cava were exposed. Vascular anastomoses were performed in an end-to-side fashion: first, the donor superior mesenteric artery (SMA) to the recipient aorta, followed by the donor superior mesenteric vein (SMV) to the recipient inferior vena cava (IVC), using a two-stitch technique for each anastomosis (warm ischemia time: 10 min). Upon reperfusion, the graft regained a healthy color and peristalsis after rehydration with warm saline. Bowel continuity was restored with a hand-sewn overlapping anastomosis between the native distal bowel and the donor proximal jejunum, and a second similar anastomosis to the transverse colon stump, both reinforced with seromuscular sutures. A diverting T-loop stoma was created in the right lower quadrant. A three-lumen gastrostomy tube was placed in the left upper abdomen. The abdomen was closed in layers over three drainage tubes.

The patient was transferred to the ICU for close monitoring and specialist care.

(1) Supportive and symptomatic management was provided, with careful control of vital signs, including blood pressure, temperature, and heart rate.

(2) Antimicrobial therapy comprised cefoperazone, ganciclovir, and voriconazole, supplemented with vancomycin.

(3) An intensive immunosuppression regimen was implemented, including azathioprine and tacrolimus (with concentration monitoring), supplemented with high-dose methylprednisolone pulse therapy.

(4) Enhanced supportive care and nutritional management were provided, including parenteral nutrition support, monitoring of electrolyte balance, and prompt correction of electrolyte imbalances.

(5) The color, output volume, and consistency of the transplanted bowel stoma were closely monitored. Surveillance colonoscopies showed healthy graft mucosa.

(6) Hepatic and renal function were monitored; hepatoprotective and choleretic agents such as glutathione and deoxycholic acid were administered.

Enteral nutrition was initiated on postoperative day (POD) 15 and was well tolerated. Sutures were removed on POD 18. Due to high stoma output, a nutrition consultation on POD 20 resulted in a switch to formula pump feeding, which improved absorption and allowed gradual advancement of enteral feeds. Transient fluid imbalance with hyperkalemia on POD 20 raised concern for renal impairment, but the patient’s condition stabilized by POD 26. With enteral nutrition successfully advanced without any signs of intolerance, the patient was transferred from the ICU to the surgical ward and was discharged in stable condition on POD 48.