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.

Consensus statement for the treatment of infantile haemangiomas with propranolol.

Although most infantile haemangiomas do not require treatment due to a natural history of spontaneous involution, some require early intervention. The Australasian Vascular Anomalies Network and the Australasian Paediatric Dermatology Network have developed a consensus statement for the treatment of infantile haemangiomas with oral propranolol. Infants with haemangiomas that are life threatening, at risk of ulceration, or at risk of causing a significant functional impairment, psychological impact or physical deformity should be treated early with oral propranolol. Oral propranolol is safe and effective and in most healthy infants oral propranolol can be started in an outpatient setting.

Extending the indications for primary nerve surgery in obstetrical brachial plexus palsy.

PURPOSE: This study identifies a small subset of patients with obstetrical brachial plexus palsy who, while they do not meet common surgical indications, may still benefit from primary nerve surgery. METHODS: Between April 2004 and April 2009, 17 patients were offered primary nerve surgery despite not meeting the standard surgical indications of the authors. The authors performed a retrospective analysis of these 17 patients using prospectively collected data. RESULTS: This group of 17 patients were identified as having poor shoulder function at about 9 months of age despite passing the Cookie Test. Fourteen patients underwent surgical intervention and three families declined surgery. All patients in the operative group regained some active external rotation after surgery. Five patients in this group have required further interventions. Two of the three patients for whom surgery was declined have had no subsequent spontaneous improvement in active external rotation. DISCUSSION: The commonly used indications for primary nerve surgery in obstetrical brachial plexus palsy may not adequately identify all patients who may benefit from surgical intervention. Patients who pass the Cookie Test but have poor spontaneous recovery of active shoulder movements, particularly external rotation, may still benefit from primary nerve surgery.

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.