Cochlear Implantation in children with CHARGE syndrome.

UNLABELLED: CHARGE syndrome is a rare congenital condition that manifests with anomalies of coloboma, heart defects, choanal atresia, mental retardation, genitourinary and ear anomalies that can affect almost any part of the auditory pathway. In those patients with a significant sensorineural hearing loss, cochlear implantation has become a potential therapeutic option. METHODS: Chart review of three cases from the Southern Cochlear Implant Programme. OUTCOMES: All patients met clinical diagnostic criteria of CHARGE syndrome, and had abnormal inner ear anatomy with profound sensorineural hearing loss. One child had previously undergone cochlear implantation which was unsuccessful due to increasing non-auditory stimulation. All patients had successful cochlear implantation with full insertion of a Nucleus Freedom Implant with contour Advance. All patients showed improvement in their audiological function; one child has high functioning verbal communication, one child uses both sign and verbal communication with improved speech quality and ability to speech read, and one child responds reliably to sound, understands short phrases and attempts to vocalize, but this is limited by tracheostomy. CONCLUSION: Cochlear implantation faces numerous challenges in children with CHARGE syndrome, but with appropriate patient selection can result in successful audiologic and quality of life outcomes.

The use of Monogen in the conservative management of chylous fistula.

BACKGROUND: Chylous fistula is an uncommon complication of lower neck dissection. If untreated, it can lead to severe electrolyte disturbance, fluid, and protein loss and ultimately threaten skin flaps and vital structures. Conservative management aims to decrease chyle volume by replacing long-chain triglycerides (LCTs) with medium-chain triglycerides in the diet or by total parenteral nutrition. METHODS: In 2001, Greenlane Head and Neck unit conducted a prospective 2-year study of all patients with chylous fistula. RESULTS: Eleven patients developed chylous fistula in the period between 2001 and 2003, out of 210 neck dissections. All cases were managed conservatively. The mean time to diagnosis was 1.5 days (1-4), time to closure was 8.1 days (4-26), and mean duration of treatment was 11.5 days (4-35). CONCLUSION: We would recommend conservative management of chylous fistulae with a low LCT diet using Monogen alone to be continued only for the duration of the fistula.

Effect of immunocompromise on metastatic cutaneous squamous cell carcinoma in the parotid and neck.

BACKGROUND: Our aim was to examine the effect of a compromised immune state on the outcomes in patients treated for metastatic cutaneous squamous cell carcinoma (SCC). METHODS: A retrospective analysis of patients with metastatic cutaneous SCC to the parotid and neck treated at Greenlane Hospital between 1992 and 2002 was conducted. Outcomes were compared between immune-competent and immunocompromised patients. A logistic regression analysis of likely risk factors for poor outcome was done. RESULTS: Forty-nine patients were identified, nine of whom were immunocompromised. All patients were treated by parotidectomy and/or neck dissection. The facial nerve was sacrificed in 42% of the patients. Thirty-seven patients underwent postoperative radiotherapy (76%). Recurrence was significantly more common in the immunocompromised group (56% vs 28%), with higher rates of local and distant recurrence. Survival at 1 and 2 years was reduced. CONCLUSION: Immunocompromise has a significant impact on the outcome of metastatic cutaneous SCC to the parotid and neck, affecting recurrence and survival.