All patients in this series had normal antenatal karyotyping, microarray, and normal Apgar score at birth, and they all underwent a full neonatal physical assessment, echocardiography, renal and cranial ultrasound (US). Anomalies other than EM are described on a case-by-case basis below.

A male of 39 + 2 weeks gestational age with a confirmed antenatal diagnosis of EM at 23 weeks gestation was born via elective caesarean section. His mother was diagnosed with maternity-related hypothyroidism and was on levothyroxine. His birth weight was 3.22 kg. He was transferred to our tertiary paediatric referral centre at 5 h of life.

In the immediate post-natal period, he was kept nil per orum and commenced intravenous fluids. On arrival, he was self-ventilating, maintaining his oxygen saturation above 96% on room air. Both cardiac and respiratory examinations were unremarkable, and no dysmorphism was noted. He had normal male external genitalia, both testes palpable within the scrotum and a normally sited anus. A routine echocardiogram showed a small apical ventricular septal defect (VSD). His cranial and renal US were normal.

The EM contained bowel loops and the liver. The sac was intact. The diameter of his abdominal wall defect was measured at 5 cm, and the liver shadow was visible at the superior aspect of the sac. Gradual compression dressing was applied on day 1 of life. The patient was commenced on small-volume oral feeds on day 1 of life and gradually progressed to full feeds by day 3 of life. After 5 days, the sac was noticed to be dry and covered by eschar, so the umbilical suspension was stopped, and the gauze bandage was applied to the sac and lumbar region. He underwent surgical closure of EM at the end of the second week of life.

Intraoperatively, the EM sac was noted to be dry, intact, and containing liver only. The upper surface of the sac was adherent to the antero-superior surface of the liver (Fig. 7). The sac was excised except for the part that was adherent to the liver. The abdominal wall defect was incised cranially, and caudally to allow reduction of the liver into the peritoneal cavity. The rectus sheath was defined with minimal dissection and closed with a 2–0 Ethibond continuous suture. An umbilicoplasty was performed using 5–0 Caprosyn sutures.

The cranial surface of the sac was adherent to the antero-superior surface of the liver

Postoperatively, the patient was kept fasting with an NGT in place for decompression. He was commenced on intravenous antibiotics and nurse-controlled analgesia (NCA). On the first postoperative day, he developed tachycardia, increased work of breathing and abdominal distension. He was transferred to the high dependency unit (HDU) and commenced on supplemental oxygen via nasal prongs. Pain management was optimized, and his septic work-up and chest x-ray were unremarkable.

On the third postoperative day, oral feeding was restarted, and he was discharged from the HDU to the general ward. He tolerated full oral feeds and was discharged home on his eighth postoperative day. Three weeks following discharge, he presented to the emergency department with a reducible left inguinal hernia, and he underwent a left inguinal herniotomy the following day. He was discharged home less than 24 h postoperatively. On routine follow-up 3 months later, the EM repair was intact. (Fig. 8).

Follow-up in 3 months shows an int act EM repair

Our second case is a female baby with a gestational age of 38 + 5. She had an antenatal diagnosis of EM confirmed at 35 weeks, with a small inlet VSD. She was born by elective caesarean section to a healthy mother. Her birth weight was 3.49 kg. She was transferred from the maternity hospital to our tertiary paediatric referral centre at 3 h of life.

On arrival, she was self-ventilating. Her EM defect was measured at 6 cm and contained liver and bowel. Additionally, the patient was noted to have dysplastic left middle and index fingers. She had a patent and normally sited anus, and she passed meconium on day 1 of life. An echocardiogram showed a 4 mm atrial septal defect (ASD) and a small VSD with left to right shunt. US of the urinary tract and cranial contents were normal.

Gradual compression dressing was applied on the first day of life. Simultaneous parenteral nutrition and trophic oral feeding were commenced on day 1 of life and gradually increased to full oral feeding on day 8 of life with cessation of parenteral nutrition. Given the size of the sac and its contents, the patient was discharged home with planned delayed repair. The dressing was changed every 3 days at the surgical day unit. At 4 months of age, she underwent surgical repair.

An elliptical 6 cm incision was made around the neo-epithelialized sac, taking care to remain extraperitoneal. On opening the peritoneum, blunt finger dissection was used to detach the colon from the sac. The fascial edges were defined with minimal dissection from the skin edges to preserve blood supply. The viscera were inspected to rule out malrotation or obstruction, and no bands were identified. The fascial defect was closed with 2–0 Ethibond. The subcutaneous layer was closed using 3–0 undyed Polysorb and the skin with 5–0 Caprosyn.

Oral intake was re-introduced postoperatively on the day. The patient was discharged home on day 2 postoperatively. A 3-cm diameter weakness was felt along the suture line on her annual review. This was treated expectantly; on review 2 years postoperatively, it completely resolved, with no weakness or hernia noted.

A female baby of 38 + 2 gestational age was transferred from a maternity hospital at 3 h of life with EM. She was born to a healthy mother. Antenatal ultrasound confirmed EM at 24 weeks. The patient was born via elective lower segment caesarean section. Her birth weight was 3 kg.

The exomphalos sac was intact. The defect size measured 7 cm, and the sac contained the liver only. She had a patent normal-site anus and normal external genitalia. A routine echocardiogram showed a structurally normal heart; cranial and renal US were normal.

The patient was commenced on trophic feeds with maternal expressed breast milk (EBM) on day 1 of life and gradually increased in volume; full volume was reached on day 8. Gradual compression dressing was commenced on day 1 of life. The patient had a transient episode of desaturation on day 5, but it resolved spontaneously. The parents were trained in managing and changing the compression dressing with the help of a multidisciplinary team, including occupational therapists, physiotherapists, and clinical nurse specialists.

The patient was discharged home after 4 weeks in the hospital. Surgery was performed 3 months later. The defect was 6 × 6 cm in size and contained liver alone. A midline incision was made, the redundant skin was excised, the bowel was inspected, and the sac contents were reduced. The fascial edges were dissected, and the defect was closed using a continuous 2–0 Ethibond suture with mild tension. She was kept in a general ward postoperatively and commenced on feeds day 2 postoperatively. She was discharged home on day 3 postoperatively.

The patient was reviewed in the clinic 4 weeks later. She recovered well following her surgery and had an intact repair on examination.

A female baby, with gestational age 38 + 2 weeks, had a confirmed antenatal diagnosis of EM. Born via emergency LSCS to a primigravida mother. Her birth weight was 2.56 kg. The patient was transferred to the tertiary paediatric centre at 24 h of life.

On assessment, she was on room air. Her cardiac and respiratory exams were unremarkable, and no dysmorphic features were noted. The EM defect was measured at 6 cm; the sac was intact and contained bowel and liver. The echocardiogram showed a patent ductus arteriosus and a small VSD. Both cranial and renal US were normal.

Gradual compression dressing was commenced on day 2 of life. EBM feeding was commenced via NGT and advanced gradually to reach full volume feeds on day 9 of life. The parents were trained on how to apply the compression dressing, and the patient was discharged home at 6 weeks of age. Surgical repair was planned at 11 weeks of age; however, it was delayed because she developed a lower respiratory tract infection at the time.

In an outpatient clinic follow-up at 4 months of age, she was found to have bilateral inguinal hernias, which were repaired a week later. Delayed EM closure was planned at 6 months of age. Unfortunately, due to the COVID-19 pandemic, this was further delayed until eventually performed when she was 1 year old.

Intraoperatively, an elliptical incision was made around the redundant abdominal skin. She was noted to have malrotation, so Ladd’s procedure was performed. Skin flaps were raised, and the fascial defect was closed with Ethicon 2–0; an umbilicoplasty was undertaken.

Postoperatively, she was commenced on oral feeds on the second postoperative day and was discharged home on day 3. On routine follow-up 1 year later, the repair was intact, and there was no evidence of a hernia. The patient was referred to plastic surgery for further umbilicoplasty.