Conservative Airway Management Successful in Majority of Infants With Pierre-Robin Sequence at Queensland Children's Hospital: A Retrospective Review.

PURPOSE: Pierre-Robin Sequence (PRS) is a congenital abnormality characterized by micrognathia, glossoptosis, and variable severity upper airway obstruction. Clear management algorithms are lacking, particularly the indications for surgical versus nonsurgical intervention. The authors reviewed the management of these children in Queensland. METHODS: All children diagnosed with PRS at Queensland Children's Hospital from April 2014 to October 2019 were identified (n=45), and their charts were retrospectively reviewed. Three management patterns emerged: prone/lateral positioning, nasopharyngeal airway (NPA) use, and surgery (tracheostomy or mandibular distraction). RESULTS: Most children (n=30; 67%) were managed successfully nonsurgically with an NPA (median age of insertion 0.25 mo, median duration 5.0 mo). Of these, 12 patients (40%) also required supplemental oxygen. The median age of NPA cessation was 5.5 months, with oxygen therapy ceasing at a median 8.25 months, upon which no further support was required. The remaining majority (n=13; 29%) of children were managed without an NPA, using positioning alone (10/13; 77%) or positioning combined with supplemental oxygen (1/13), CPAP (1/13), or both adjunct measures (1/13). Only 2 patients underwent surgical intervention. Feeding supplementation using nasogastric tube was necessary in 78% of patients for a median duration of 4 months. Cleft palate co-existed in all but one patient. CONCLUSION: Management of upper airway obstruction in PRS children is variable between units. Over a 5-year period, 96% of children with PRS were successfully managed without surgical intervention at the Queensland Children's Hospital. These findings contrast with some other literature and may suggest that more careful consideration of surgical intervention in PRS patients is prudent.

Nasal Septal Flaps for Repair of Large or Recurrent Palatal Fistula: Report of Technique and 4-Year Experience.

SUMMARY: Large or multiply recurrent oronasal fistulas following cleft palate repair present a challenging problem. Nasal septal mucoperichondrial flaps have been widely used for repair of skull base defects; however, their use in the repair of oronasal cleft palate fistulas has not previously been described. In this pilot study, the authors describe anterior palatal fistula repair using a nasal septal flap and review their experience with this technique over 4 years. Fourteen patients with anterior palatal fistulas not amenable to repair using local palatal flaps were included for analysis. The mean size of the fistula was 12 mm in maximum dimension. Flap healing with complete or near-complete closure of fistula was achieved in 13 patients (93%). Five of these patients had a small, slit-like residual fistula that was asymptomatic. Nasal septal flaps are a new technique for repair of large or recurrent palatal fistulas. The procedure is well-tolerated with minimal side effects, high success rate, and low incidence of recurrence. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Microsurgical hepatic artery reconstruction in paediatric liver transplantation.

BACKGROUND/AIMS: Hepatic artery thrombosis is a serious complication of paediatric liver transplantation often leading to retransplantation. It is also associated with decreased patient survival rates. In 1999 microsurgical hepatic artery reconstruction by Plastic and Reconstructive Surgeons was introduced to the Queensland Liver Transplant Service at the Royal Children's Hospital, Brisbane, in an attempt to decrease hepatic artery complications. METHODOLOGY: A review of the computerised database of the Queensland Liver Transplant Service was undertaken. Between March 1985 and December 2005, 252 transplants were performed without microsurgery. Since December 1999, 23 transplants were performed with microsurgical hepatic artery reconstruction by Plastic and Reconstructive Surgeons using the operating microscope. RESULTS: There were a total of 28 cases of hepatic artery thrombosis in 275 transplants. Survival for patients with hepatic artery thrombosis was significantly worse than for patients without (one year survival rate 61.5% versus 83.6%, p = 0.0065). The microsurgery group had a lower incidence of hepatic artery thrombosis (4.3% versus 10.7%, p = 0.29), a lower retransplantation rate (4.3% versus 9.1%, p = 0.38) and increased one year patient survival (91.3% versus 79.7%, p = 0.31). CONCLUSIONS: Microsurgical hepatic artery reconstruction in paediatric liver transplantation may decrease hepatic artery thrombosis rates, decrease retransplantation rates and improve survival.

Method of quadriceps attachment following upper tibial resection.

Limb salvage procedures for osteosarcomas of the upper tibia present combined problems of knee-joint and tibial reconstruction. Many methods of overcoming this have been described. We describe a new technique of prosthetic knee-joint and upper-tibial reconstruction, combined with a vascularized fibular bone flap for reattachment of the quadriceps muscle and tendon unit, resulting in superior long-term function.

The role of cranial expansion for craniocephalic disproportion.

The appropriate treatment for craniocephalic disproportion, such as caused by slit ventricle syndrome, is uncertain. We have reviewed the treatment and outcomes of 4 children who underwent cranial expansion over a period of 5 years. The ages at cranial expansion were 16 months, 3 years 6 months and 2 at 6 years. Two children had slit ventricle syndrome and were treated with bilateral parietal expansions. Two children had non-syndromic craniosynostosis, one of these having vitamin D-resistant rickets. The latter 2 were treated with posterior cranial vault expansion, and one also had a craniocervical junction decompression performed. Post-operatively, symptoms of raised intracranial pressure resolved in all cases, and there was radiological evidence of re-establishment of normal CSF pathways. Although a rare condition, cranial expansion operations can be successful in appropriately selected cases of craniocephalic disproportion.

Craniopagus: second Brisbane case. Case report.

Craniopagus is a rare and intriguing condition with an incidence of one in 2.5 million births. The chance of a neurosurgeon seeing a case in a working lifetime is unlikely. The chances of two cases from the same community within 12 months are remote in the extreme. The authors present a second case of craniopagus born and separated in Brisbane, Australia, in 2001 and discuss the intricacies of surgical separation and the lessons learned.

Separation of craniopagus joined at the occiput. Case report.

Siamese or conjoined twins have intrigued both the physician and layperson for centuries. The craniopagus type (joined at the head) is exceedingly rare, with an incidence of one in 2.5 million births. Most clinicians never see a case of craniopagus, and those who do rarely see more than one. The authors present a case of the craniopagus type of conjoined twins born and recently separated in Brisbane, Australia. The prenatal diagnosis, subsequent investigations, separation, and outcome are presented.