Mai Thy Truong

Propranolol for the treatment of a life-threatening subglottic and mediastinal infantile hemangioma.

An infant with a subglottic hemangioma remained in respiratory distress after multiple treatments failed and was found to have an enlarging mediastinal infantile hemangioma compressing the trachea. Treatment with oral propranolol resulted in resolution of symptoms within 2 days and a 50% reduction in lesion size within 1 week.

Pediatric vocal fold paralysis after cardiac surgery : Rate of recovery and sequelae

Objective To determine the rate of recovery of pediatric vocal fold paralysis (VFP) after cardiac surgery. Study Design and Setting Retrospective case series from January 2000 to 2005 at 4 tertiary care pediatric hospitals. Results A total of 109 children with VFP were identified. Of 80 patients with follow-up >3 months, 28 (35%) recovered vocal fold function with a median time to diagnosis of recovery of 6.6 months. Fifty-two (65%) patients had persistent vocal fold paralysis with a median follow-up time of 16.4 months. Twenty-five (45%) of 55 patients demonstrated aspiration or laryngeal penetration with modified barium swallow. Twenty-nine (27%) of the 109 patients underwent surgical intervention for their airway, feeding, or voice. Conclusions Pediatric VFP is not an uncommon complication after cardiac surgery and can result in serious sequelae. This study demonstrates a 35% rate of recovery, 45% rate of aspiration, and 27% rate of complications that require surgical intervention.

Sleep endoscopy as a diagnostic tool in pediatric obstructive sleep apnea

OBJECTIVES Ten to twenty percent of children have persistent obstructive sleep apnea (OSA) after adenotonsillectomy (T&A). We hypothesize that sleep endoscopy, a flexible fiberoptic examination of the pharynx under anesthesia, is an effective tool for identifying sites of persistent obstruction. METHODS In this retrospective cohort study, we reviewed records of children who had symptoms consistent with OSA and a positive polysomnogram (PSG) who underwent sleep endoscopy followed by sleep endoscopy directed surgery. Data collection included age, BMI and co-morbidities. Apnea-hypopnea index (AHI) was compared to pre and post surgery for each child using a paired t-test. RESULTS Of the 80 children who underwent sleep endoscopy followed by directed surgery, 65% were male, mean age was 6 years (SD 3.75 years), average BMI was 19 (SD 0.43 years) and 28% had co-morbidities. For the 51% of patients who had persistent OSA after T&A, the mean AHI after sleep endoscopy directed surgery was significantly lower then before surgery (7.9 vs. 15.7, p<.01). For the 49% of patients who had never undergone surgery for OSA, or who were surgically naïve, and underwent sleep endoscopy directed surgery, the mean AHI was significantly lower then before surgery (8.0 vs. 13.8, p<.01). CONCLUSIONS Sleep endoscopy is a consistently reliable tool for identifying the sites of obstruction in both surgically naive children and those with persistent OSA after T&A.

Propranolol for the treatment of airway hemangiomas: a case series and treatment algorithm.

OBJECTIVES (1) To present six patients with symptomatic airway hemangiomas treated with oral propranolol. (2) To review the diagnostic and treatment options for airway hemangiomas and propose a new management protocol. STUDY DESIGN Retrospective review. SETTING Tertiary care childrens hospital. SUBJECTS AND METHODS Pediatric patients diagnosed with obstructive airway hemangiomas treated with oral propranolol. Patients were followed for symptomatic improvement and relief of airway obstruction on imaging or laryngoscopy. RESULTS Seven patients presenting with airway obstruction were treated with propranolol. One patient had a focal hemangioma confined to the subglottis. Four patients had airway hemangiomas that extended beyond the confines of the larynx and trachea. A sixth patient had a bulky supraglottic hemangioma. A seventh patient with an extensive maxillofacial lesion failed propranolol therapy and was found to have a pyogenic granuloma on final pathology after excision. Six patients had failed standard medical therapy and/or surgical interventions and were treated successfully with oral propranolol with improvements in airway symptoms and oral intake, requiring no further surgical intervention. Treatment was initiated as early as 1.5 months of age, and as late as 22 months. No adverse side effects of propranolol were noted. CONCLUSIONS Oral propranolol was successfully used to treat airway hemangiomas, resulting in rapid airway stabilization, obviating the need for operative intervention, and reducing the duration of systemic corticosteroid therapy while causing no obvious adverse effects. These outstanding results enable the possibility of use of a standardized diagnostic and treatment algorithm for airway hemangiomas that incorporates systemic propranolol.

Supraglottoplasty for Occult Laryngomalacia to Improve Obstructive Sleep Apnea Syndrome

OBJECTIVE To evaluate the polysomnographic outcomes after supraglottoplasty (SGP) performed for obstructive sleep apnea syndrome (OSAS) associated with occult laryngomalacia. DESIGN Retrospective case series with medical chart review. SETTING Tertiary pediatric medical center. PATIENTS Twenty-two patients aged 2 to 17 years met the inclusion criteria of polysomnography-proven OSAS and occult laryngomalacia seen on flexible fiber-optic sleep endoscopy. Infants with congenital laryngomalacia were excluded. INTERVENTION Carbon dioxide laser SGP was performed either alone or in conjunction with other operations for OSAS. MAIN OUTCOME MEASURE Preoperative and postoperative nocturnal polysomnographic data were paired and analyzed statistically. RESULTS Supraglottoplasty for occult laryngomalacia resulted in statistically significant reduction in the apnea-hypopnea index (AHI) (from 15.4 to 5.4) (P <.001). Subgroup analysis of children who underwent either SGP alone or in combination with other interventions showed comparable reductions in AHI. Medical comorbidities were associated with worsened postoperative outcomes, although still significantly improved compared with baseline. Overall, 91% of children had an improvement in AHI, and 64% had only mild or no residual OSAS after SGP. CONCLUSION Supraglottoplasty is an effective technique for the treatment of OSAS associated with occult laryngomalacia.

Primary Hyperparathyroidism in Pregnancy: A Case Series and Review†

Objectives/Hypothesis: To review the clinical significance of primary hyperparathyroidism during pregnancy including the maternal, fetal, and neonatal sequelae. Additionally, to discuss treatment options and describe three cases where surgical parathyroidectomy was successful for treatment of hyperparathyroidism refractory to medical management during pregnancy.

A child with silent sinus syndrome and spontaneous improvement after sinus surgery

Silent sinus syndrome is characterized by an asymptomatic hypoplastic maxillary sinus with progressive enophthalmos and hypoglobus. This is a disease rarely affecting children with the majority of reported cases involving adult patients. Treatment is primarily surgical with endoscopic sinus surgery to restore aeration of the sinus along with orbital reconstruction to restore facial symmetry. In this report, we describe a 7 year old child with facial asymmetry and no sinonasal symptoms. CT showed an opacified hypoplastic right maxillary sinus. One year after endoscopic sinus surgery, there was spontaneous improvement of facial asymmetry and relative maxillary sinus size.

The Superior Labial Frenulum in Newborns: What Is Normal?:

Introduction and Objectives: There has been an emergence of procedures to release the superior labial frenula in infants, yet little is known about the normal appearance or incidence of severe attachment, or “lip-tie.” The objective of this article was to develop a classification system for superior labial frenula and to estimate the incidence of different degrees of attachment. Methods: A prospective cross-sectional study. Newborns were examined and had photographs taken of their upper frenula. Relevant medical professionals rated the appearance of the labial frenula using a previously described Kotlow classification system. The raters assessed each photograph twice and were blinded to their previous rating and to other raters’ scores. Results: All newborns have a labial frenula, with most attached at the gingival margins (83%). Raters had poor intra- and interrater reliability (64% to 74% and 8%, respectively), using the Kotlow classification system, which improved when the classification system was simplified. Conclusions: The Kotlow classification of lip-tie fails to be reproducible by relevant experts. The majority of infants had a significant level of attachment of the labial frenulum. As more procedures are done to release the upper lip frenulum, it is important to understand what degree of attachment is normal, or more common.

Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia

OBJECTIVE Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fishers exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.