Specialist & Consultant Pediatric Surgery oral assessment prep

Knowledge: With shock, acidosis, a raised lactate and blood per rectum, my working diagnosis is midgut volvulus on malrotation with ischaemic bowel. The narrow-based mesentery lets the midgut twist clockwise around the superior mesenteric artery, and the whole midgut can necrose within hours. Atresia, necrotising enterocolitis and sepsis with ileus are differentials but do not change the priority. A normal plain film never excludes volvulus. In a stable baby an upper gastrointestinal contrast study is the test of choice, showing a malpositioned duodenojejunal flexure or a corkscrew duodenum, but peritonism and acidosis mean imaging must not delay laparotomy.

Management: I would pass a large-bore nasogastric tube on free drainage, secure access, send gas, blood count, coagulation, cross-match and cultures, give 10 to 20 mL/kg 0.9% saline boluses with reassessment, and start broad-spectrum antibiotics. I would aim to operate within the hour, resuscitating in parallel. At laparotomy I would detort anticlockwise, wrap the bowel in warm packs and assess viability, then complete a Ladd procedure: divide Ladd bands, widen the mesentery, place small bowel right and colon left, and remove the appendix. If viability is doubtful I would not resect widely but plan a second look at 24 to 48 hours. Frankly necrotic bowel is resected, with stomas if he is unstable. Afterwards he needs intensive care, parenteral nutrition and follow-up for short bowel if resection was large.

Ethics and communication: Consent comes from his guardian, but I would not delay surgery to find a particular relative, because UAE law permits emergency treatment without consent. I would tell the parents honestly that some bowel may already be dead and that a second operation, stomas or lifelong intestinal failure are possible. If transfer was delayed, I would record the facts neutrally and use the incident system, not the corridor.

Reference: Holcomb GW, Murphy JP, St Peter SD, Holcomb and Ashcraft's Pediatric Surgery (7th ed); Langer JC, Intestinal Rotation Abnormalities and Midgut Volvulus, Surgical Clinics of North America, 2017

Knowledge: This is an isolated blunt splenic injury in a child who has responded to resuscitation, and non-operative management succeeds in the large majority, including high-grade injuries. The decision rests on haemodynamic response, not CT grade. Children compensate well, so persistent tachycardia is a more reliable warning than hypotension, which is late. Preserving the spleen avoids overwhelming post-splenectomy infection. A handlebar mechanism also raises pancreatic, duodenal and small bowel injury, which early CT can miss. The APSA evidence review supports physiology-based length of stay and activity restriction of about injury grade plus two weeks.

Management: I would take responsibility as the paediatric surgeon, admit her to a monitored bed, keep her nil by mouth initially, cross-match and repeat haemoglobin at about six hours. Further instability is treated with blood, 10 to 15 mL/kg packed red cells, not more crystalloid. Tranexamic acid within three hours of injury is reasonable at 15 mg/kg, maximum 1 g. I would define failure in advance: instability despite transfusion, or ongoing transfusion need, means theatre, attempting splenic salvage only if she is stable enough. Angioembolisation is for ongoing bleeding in a stable child where paediatric interventional radiology exists. I would re-examine her repeatedly, check lipase and avoid routine repeat CT. After any splenectomy she needs pneumococcal, meningococcal and Haemophilus vaccines and antibiotic prophylaxis.

Ethics and communication: Disagreement with a colleague is settled by discussing the evidence away from the family, and the agreed plan with its triggers is documented. I would explain to the parents that surgery may still be needed and take consent for possible splenectomy early. I would check that the story fits the injury as routine safeguarding. If the hospital cannot provide paediatric surgical and transfusion cover overnight, safe care means transfer.

Reference: Gates RL et al, Non-operative management of solid organ injuries in children: an American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee systematic review, Journal of Pediatric Surgery, 2019; American College of Surgeons, ATLS Advanced Trauma Life Support Student Course Manual (10th ed, 2018)

Knowledge: Conjugated hyperbilirubinaemia in an infant is always pathological, and pale stools, dark urine, a raised GGT and a triangular cord sign make biliary atresia the working diagnosis. Differentials include choledochal cyst, Alagille syndrome, alpha-1 antitrypsin deficiency, progressive familial intrahepatic cholestasis, cytomegalovirus, galactosaemia, hypothyroidism and hypopituitarism. Results of Kasai portoenterostomy are better the earlier it is done, ideally before about 60 days of age, so she is already near the limit. Most cases obliterate the extrahepatic ducts up to the porta hepatis, so drainage depends on the portoenterostomy. The raised INR reflects vitamin K malabsorption, and the first thing that can harm her is an intracranial haemorrhage.

Management: Today I would give intravenous vitamin K and recheck the INR. In parallel I would send alpha-1 antitrypsin phenotype, cytomegalovirus testing, thyroid function, cortisol and a galactosaemia screen, without letting the work-up delay surgery, and refer her the same week to a centre experienced in Kasai surgery and liver transplantation. At operation, an intraoperative cholangiogram or direct inspection confirms the diagnosis, followed by a Kasai portoenterostomy. Afterwards she needs cholangitis prophylaxis, ursodeoxycholic acid, fat-soluble vitamins, a medium-chain triglyceride formula and bilirubin monitoring. Adjuvant steroids did not improve drainage in the START trial. Fever after a Kasai is cholangitis until proven otherwise. Failure to clear jaundice within the first few months means early transplant assessment.

Ethics and communication: I would explain to her guardian, who gives consent, that the Kasai is not a cure, that many children eventually need a liver transplant, and that time matters. If the family prefer surgery abroad, I would help them arrange it quickly rather than resist. I would document the delay since the pale stools began and use it to strengthen stool colour education in local primary care.

Reference: Hartley JL, Davenport M, Kelly DA, Biliary atresia, Lancet, 2009; Fawaz R et al, Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of NASPGHAN and ESPGHAN, Journal of Pediatric Gastroenterology and Nutrition, 2017

Knowledge: The likely diagnosis is Wilms tumour, the commonest renal tumour of childhood, peaking at three to four years. The main differential is neuroblastoma, which calcifies, encases vessels and displaces rather than arises from the kidney; others are clear cell sarcoma and rhabdoid tumour. I would look for aniridia, genitourinary anomalies and overgrowth syndromes. The lung nodules suggest stage IV disease. SIOP practice, followed by many centres including in the Gulf, gives preoperative chemotherapy usually without biopsy, while the Children's Oncology Group favours upfront nephrectomy. Hypertension is often renin mediated, and acquired von Willebrand disease is recognised.

Management: I would refer immediately to the paediatric oncology team and not operate before that discussion. Work-up includes urinary catecholamines, blood count, renal function, coagulation with a von Willebrand screen, baseline echocardiography before anthracycline, and a central line. Hypertension is treated, often with a calcium channel blocker. Under SIOP practice he would receive about six weeks of vincristine, actinomycin D and doxorubicin for metastatic disease, then restaging. Biopsy is kept for atypical features, such as an unusual age or imaging. At surgery I would perform a transperitoneal radical nephroureterectomy, avoid tumour rupture or spill because it upstages the child and adds treatment, and sample regional lymph nodes as the protocol requires. Postoperative chemotherapy and possible lung radiotherapy depend on histological risk group and response.

Ethics and communication: I would give the diagnosis honestly with the oncologist present, explaining that most children with Wilms tumour, including many with lung spread, are cured, without making promises. Consent comes from the guardian after full discussion of treatment and late effects. If the family seek treatment abroad, I would support a timely transfer with full records, and offer psychosocial support.

Reference: SIOP Renal Tumour Study Group, UMBRELLA SIOP-RTSG 2016 protocol (Wilms tumour treatment guidelines); Holcomb GW, Murphy JP, St Peter SD, Holcomb and Ashcraft's Pediatric Surgery (7th ed)

Knowledge: This is testicular torsion until proven otherwise: sudden onset, vomiting, a high horizontal testis and an absent cremasteric reflex. Torsion of an appendage, epididymo-orchitis and an incarcerated hernia are differentials, but none justifies waiting. Most testes are saved if detorsion occurs within about six hours, and salvage falls steeply after that. Doppler ultrasound should not delay exploration when the clinical picture is clear. Under UAE law consent for a person under 18 is given by the guardian, and informed consent is required before treatment except in an emergency. Waiting eight hours would very likely cost him the testis, so this meets the emergency threshold.

Management: I would give analgesia, keep him nil by mouth and alert theatre. I would ask the family to keep calling the father, while I involve the senior administrator on call as facility policy requires and ask a second consultant to confirm the emergency in writing. I would explain everything to the boy and seek his assent. If the father remains unreachable, I would proceed under the emergency exception, recording the time of each call. Through a scrotal incision I would detort the testis, assess viability with warm packs, fix it with non-absorbable sutures, and fix the other side, because the anatomical predisposition is usually bilateral. A clearly necrotic testis is removed. I would update the father as soon as he is reachable.

Ethics and communication: The uncle deserves courtesy, but he is not the guardian unless he holds legal authority, and his wish to wait cannot override the boy's interest in emergency care. The mother's agreement, the boy's assent, the second opinion and the reason it could not wait are all documented. I would avoid confrontation, and if the testis is lost I would disclose this openly.

Reference: Federal Decree-Law No. 4 of 2016 on Medical Liability and Cabinet Resolution No. 40 of 2019 (UAE); EAU Guidelines on Paediatric Urology, European Association of Urology and European Society for Paediatric Urology, 2024

Knowledge: This is oesophageal atresia with a distal tracheo-oesophageal fistula, the commonest type, confirmed by gas in the stomach. The absent thumb and single umbilical artery point to the VACTERL association, so I must look for vertebral, anorectal, cardiac, renal and limb anomalies. The early dangers are aspiration from the upper pouch, acid reflux through the fistula, and, if ventilated, gas escaping into the stomach, causing distension and even perforation. Absence of gastric gas would instead suggest pure atresia with a long gap. Echocardiography matters twice: major cardiac disease and low birth weight drive prognosis, and a right-sided aortic arch may change the side of the thoracotomy.

Management: I would place a Replogle tube on continuous low suction, nurse him head up, keep him nil by mouth on intravenous 10% dextrose, and avoid bag-mask ventilation. If intubation becomes necessary, I would use gentle pressures and be ready to ligate the fistula urgently if the stomach distends. Before surgery I need echocardiography, renal ultrasound, spine radiographs and an anal examination. Repair is planned within the first day or two through a right extrapleural thoracotomy, or thoracoscopically in experienced hands: fistula division and ligation and a primary end-to-end anastomosis. If the gap is too long, a staged approach with gastrostomy is used. After surgery I would watch for anastomotic leak, stricture needing dilatation, recurrent fistula, tracheomalacia and gastro-oesophageal reflux, which is common and usually treated with acid suppression.

Ethics and communication: I would explain to the parents why he cannot feed yet and that further procedures such as dilatations are common; consent comes from the guardian. If tests show a life-limiting condition such as trisomy 18, the plan should be revisited in an honest, unhurried goals-of-care discussion rather than proceeding by default. Where parents are related, I would offer genetic counselling without blame.

Reference: Krishnan U et al, ESPGHAN-NASPGHAN Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Esophageal Atresia-Tracheoesophageal Fistula, Journal of Pediatric Gastroenterology and Nutrition, 2016; Holcomb GW, Murphy JP, St Peter SD, Holcomb and Ashcraft's Pediatric Surgery (7th ed)