Acute pancreatitis complicating choledochal cysts in children.

AIM: To analyse the characteristics of patients with choledochal cysts presenting with acute pancreatitis. METHODS: Multicenter retrospective review of all paediatric patients (<18 years) with choledochal cysts managed over a 14-year period (2001-2014) at two tertiary paediatric surgical centres. Patient data were analysed for demographics, presentation, radiological classification of cyst type (Todani), operative interventions, complications and long-term follow-up. RESULTS: A total of 49 patients with choledochal cysts were identified with 15 (31%) being Type I fusiform, 18 (37%) Type I cystic and 16 (32%) Type IV-A. Seventeen (35%) patients presented with acute pancreatitis, one having had an ante-natally diagnosed choledochal cyst. Patients presenting with pancreatitis were older when compared to the non-pancreatitis group (5.1 vs. 1.2 years, P = 0.005). Nine out of 16 (53%) patients with Type IV-A cysts presented with pancreatitis compared to five (33%) of Type I fusiform and three (17%) of Type I cystic. There was however no statistically significant association between Todani types and the development of pancreatitis (Type I fusiform, P = 1.0; Type I cystic, P = 0.063; Type IV-A, P = 0.053). The rate of complications was similar in both groups. CONCLUSION: Pancreatitis was a common presentation in children with a choledochal cyst, however, there was no clear statistically significant association with Todani types and pancreatitis.

Common bile duct stones in infancy: a medical approach.

Symptomatic choledocholithiasis in infancy is not common. It usually presents with jaundice and acholic stools and is diagnosed on abdominal ultrasonography. Favourable outcome of conservative management has been reported, but specific management guidelines are not well defined in the literature. We describe three cases using a combination of ursodeoxycholic acid and antibiotics as a treatment paradigm, which could potentially negate more invasive treatment. All three patients had ultrasonography proven choledocholithiasis with concomitant obstructive liver function test. They were treated with a combination of ursodeoxycolic acid and antibiotics. Patient 1 had an Escherichia coli urinary tract infection and was treated with oral bactrim. Intravenous amoxicillin, gentamicin and metronidazole were used for the other two patients. All three patients responded with a return to normal-coloured stools within 48 h of combination treatment. Repeat ultrasonography done within 11 days after the first study for all three patients confirmed complete resolution of choledocholithiasis. It is postulated that this improvement is as a result of a reduction in inflammation and oedema, associated with a low-grade cholangitis, following antibiotic treatment. This is coupled with improved bile flow with ursodeoxycholic acid therapy. The findings suggest the potential application of this safe, non-invasive therapeutic strategy as initial management in infants with this condition. A follow-up prospective randomised controlled trial may be an answer to prove the validity of this observation but due to the rarity of this problem, it would be a challenge to recruit sufficient number of patients.

Audit of extrapleural local anaesthetic infusion in neonates following repair of tracheo-oesophageal fistulae and oesophageal atresia via thoracotomy.

In order to reduce postoperative opioid requirement, extrapleural local anaesthetic infusion dosing recommendations and guidelines for extrapleural catheter insertion were developed in our institution for 'extubatable' neonates requiring short-gap neonatal tracheo-oesophageal fistula/oesophageal atresia repair (via thoracotomy) and audited prospectively. Data audited included patient characteristics, analgesia details and ventilation duration. We divided patients into two groups: group 1 - term patients (=36 weeks gestational age) with birth-weights =2.5 kg; group 2 - pre-term patients (<36 weeks gestational age), with birth weights <2.5 kg and those with co-morbidities. There were 26 neonates in group 1 and 11 in group 2. All received extrapleural infusions of bupivacaine or levobupivacaine: the majority (90%) =300 µg.kg(-1).hour(-1) (median duration 43 hours, range 1.5 to 72 hours); 36% required morphine infusion and 39% were ventilated (median duration 34 hours, range 3 to 140 hours). In group 1, 24% required morphine infusion compared with 64% in group 2. Most group 1 patients (77%) were extubated immediately postoperatively; 20% had short duration ventilation (median 15 hours, range 11 to 37 hours); one required longer-term ventilation (231 hours). 82% of group 2 were ventilated for a median of 72 hours (range 3 to 140 hours). Review of patients' co-morbidities facilitated guideline revision. These now specify use in neonates requiring short-gap tracheo-oesophageal fistula/oesophageal atresia repair who are term at =36 weeks gestational age and =2.5 kg birth-weight, anticipated as ready for extubation either immediately or shortly after surgery.

Double duodenal atresia with perforation: a case report.

Duodenal atresia is a common cause of neonatal bowel obstruction. Double duodenal atresia has been reported as a rare variation of duodenal atresia. This report discusses the importance of an appreciation of unusual anatomy in neonatal duodenal obstruction and highlights the importance of assessing bowel continuity intraoperatively.

Ursodeoxycholic acid's role in avoiding diversionary surgery in idiopathic fibrosing pancreatitis.

Obstructive jaundice in childhood is rare but a cause of great concern and warrants aggressive investigation into its aetiology. Idiopathic fibrosing pancreatitis (IFP) is a rare cause of this phenomenon and is presently a diagnosis of exclusion with lymphoma a main differential diagnosis. IFP appears to be a self limiting occurrence and as a result the previous gold standard of therapy of surgical diversion may ultimately be unnecessary. We describe ursodeoxycholic acid as a useful adjunct to treatment allowing more time for clinicians to evaluate the nature of the underlying disease process without hastily moving towards invasive surgery until clearly indicated.

Spontaneous chylothorax in a 2-year-old child.

A previously well 2-year-old girl presented with acute respiratory distress. After multiple investigations she was diagnosed with spontaneous chylothorax, attributed to strenuous vomiting. To our knowledge, this is the second reported case of spontaneous chylothorax occurring after the neonatal period.

Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long-term follow-up study.

BACKGROUND & AIMS: Esophageal atresia (EA) is the most common congenital anomaly of the esophagus. There are few long-term follow-up data on adults who had surgery for EA as infants. The primary aims were to evaluate the prevalence of esophageal symptoms and pathology and second to develop recommendations for follow-up. METHODS: This is a descriptive study of individuals attending a clinic in an adult tertiary referral hospital, established to provide care for adults who had surgery for EA as infants. Individuals aged 20 years or older were identified from an existing database and invited by telephone to attend the clinic. One hundred thirty-two patients attended the clinic from 2000-2003. Individuals were assessed by using a structured questionnaire. Endoscopy was performed in 62 patients because of symptoms. RESULTS: Reflux symptoms were reported by 83 (63%), including 25 (19%) with severe symptoms. Dysphagia was reported by 68 patients (52%). Of those who underwent endoscopy, reflux esophagitis was present in 36 patients (58%), Barrett's esophagus in 7 (11%), and strictures in 26 (42%) patients. One patient was diagnosed with esophageal squamous cell carcinoma. Men who were 35 years or older and individuals with severe reflux symptoms were at high risk of having severe esophagitis or Barrett's metaplasia. CONCLUSIONS: Reflux symptoms, esophagitis, and Barrett's esophagus are common in these individuals. We recommend clinical assessment as adults and upper endoscopy for reflux symptoms or dysphagia. Transition of young adults from pediatric care to an adult gastroenterology clinic with expertise in EA appears to be highly beneficial.

Triad of tracheoesophageal fistula-esophageal atresia, pulmonary hypoplasia, and duodenal atresia.

We describe 3 cases of tracheoesophageal fistula with esophageal atresia who also had right pulmonary hypoplasia-aplasia and duodenal atresia. We review the current literature and propose that this may be a distinct association.

Spontaneous liver hemorrhage during laparotomy for necrotizing enterocolitis: a potential role for recombinant factor VIIa.

Necrotizing enterocolitis remains a serious condition in very low birth weight infants, particularly in those infants who require surgery. Perioperative hemorrhage is a potentially fatal complication in this population. We describe our experience in 4 premature infants with necrotizing enterocolitis who received recombinant factor VIIa to manage life-threatening intraoperative hemorrhage.