Adverse Events in Young and Preterm Infants Receiving Topical Timolol for Infantile Hemangioma.

BACKGROUND: The success of oral propranolol for treatment of infantile hemangiomas (IHs) has led practitioners to use topical ß-blockers. In preterm infants, clinicians frequently turn to topical timolol, with the presumption that topical application will result in less systemic absorption. We used Holter monitoring to assess for drug-induced bradycardia in high-risk infants. METHODS: We retrospectively reviewed the charts of 22 at-risk infants who received a Holter monitor to assess for association between timolol administration and development of significant bradycardia. RESULTS: Four infants had episodic bradycardia detected by Holter monitoring. Two of these infants were full term; weighed more than 3,000 g; and had rare, brief, asymptomatic episodes unrelated to the timing of the timolol application. The other two infants had symptomatic bradycardia while on timolol and were the only two babies that weighed less than 2,500 g at initiation of therapy. Both were young (postmenstrual age [PMA] 34 and 37 wks) at initiation and had a timolol dose above the average exposure for the cohort. CONCLUSION: In this cohort of at-risk infants, topical timolol appeared to provide safe treatment for IHs in full-term infants receiving a dose of less than 0.2 mg/kg/day, but infants with a PMA of less than 44 weeks and weight at treatment initiation of less than 2,500 g may be at risk of adverse events, including bradycardia, hypotension, apnea, and hypothermia. We recommend close monitoring of temperature, blood pressure, and heart rate in premature and low-birthweight infants with IHs at initiation of and during therapy with topical timolol.

Gastrointestinal bleeding in infantile hemangioma: a complication of segmental, rather than multifocal, infantile hemangiomas.

OBJECTIVE: To highlight an association of facial segmental hemangiomas with gastrointestinal bleeding in infants with infantile hemangiomas. STUDY DESIGN: We conducted a multicenter retrospective case series study. RESULTS: Ten female patients met study inclusion criteria; 8 were Caucasian, 9 had a facial segmental hemangioma, and 9 cases met the diagnostic criteria for definitive posterior fossa malformations, hemangioma, arterial lesions, cardiac anomalies/coarctation of the aorta and eye abnormalities syndrome with abnormalities of the aorta and cerebral arteriopathy. Severe gastrointestinal bleeding requiring blood transfusion occurred in 9 cases, with age at presentation of gastrointestinal bleeding ranging from 8 days to 6 months. When detected, the location of the hemangioma in the small intestine was in the distribution of the superior mesenteric artery. More than one agent was required to control the gastrointestinal bleeding, including oral or intravenous steroids, vincristine, oral propranolol, interferon, and resection of the small intestine. All cases needed ongoing support care with red blood cell transfusions. CONCLUSIONS: Gastrointestinal bleeding is a rare complication of true infantile hemangioma. The segmental pattern of the cutaneous hemangioma associated with gastrointestinal bleeding should suggest a segmental infantile hemangioma of the lower gastrointestinal tract.

Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin.

OBJECTIVE: To prospectively evaluate a cohort of patients with infantile hemangioma in the midline lumbosacral region for spinal anomalies to determine the positive predictive value of infantile hemangioma for occult spinal anomalies and to make evidence-based recommendations for screening. STUDY DESIGN: A multicenter prospective cohort study was performed at 9 Hemangioma Investigator Group sites. RESULTS: Intraspinal abnormalities were detected in 21 of 41 study participants with a lumbosacral infantile hemangioma who underwent a magnetic resonance imaging evaluation. The relative risk for all patients with lumbosacral infantile hemangiomas for spinal anomalies was 640 (95% confidence interval [CI], 404-954), and the positive predictive value of infantile hemangioma for spinal dysraphism was 51.2%. Ulceration of the hemangioma was associated with a higher risk of having spinal anomalies. The presence of additional cutaneous anomalies also was associated with a higher likelihood of finding spinal anomalies; however, 35% of the infants with isolated lumbosacral infantile hemangiomas had spinal anomalies, with a relative risk of 438 (95% CI, 188-846). The sensitivity for ultrasound scanning to detect spinal anomalies in this high-risk group was poor at 50% (95% CI, 18.7%-81.3%), with a specificity rate of 77.8% (95% CI, 40%-97.2%). CONCLUSIONS: Infants and children with midline lumbosacral infantile hemangiomas are at increased risk for spinal anomalies. Screening magnetic resonance imaging is recommended for children with these lesions.

Nevus simplex: a reconsideration of nomenclature, sites of involvement, and disease associations.

BACKGROUND: Nevus simplex (NS) is a common birthmark on the forehead, glabella, upper eyelids, and nape. More widespread involvement can be confused with port-wine stains (nevus flammeus) and other vascular birthmarks. OBJECTIVES: To further categorize the anatomic locations in infants with extensive NS and evaluate for any possible disease associations. METHODS: We conducted a retrospective review of patients with extensive NS seen at two tertiary care centers. RESULTS: Twenty-seven patients with extensive NS were identified. All had at least one typical site of involvement: glabella (77.8%), nape (59.3%), and eyelids (55.6%). Additional sites were the scalp, including the vertex, occiput, parietal (66.7%); nose (66.7%); lip (59.2%); lumbosacral skin (55.6%); and upper and mid back (14.8 %). LIMITATIONS: Retrospective nature of the study and relatively small sample size. CONCLUSIONS: We propose the term "nevus simplex complex" for NS with more widespread involvement beyond the typical sites. Consistent use of the term "nevus simplex" will aid in correct diagnosis and appropriate management of these birthmarks.

Aicardi-Goutières syndrome: cutaneous, laboratory, and radiologic findings: a case report.

Aicardi-Goutières syndrome is a primarily autosomal recessive disorder characterized by congenital encephalopathy, basal ganglia calcifications, elevated interferon-alpha in blood and cerebral spinal fluid, and negative studies for intrauterine infections that can mimic the syndrome. Cutaneous manifestations include pernio, photosensitivity, and cutaneous vasculitis. We present a case of Aicardi-Goutières syndrome to increase awareness of the disorder and its possible relation to systemic lupus erythematosus.

More evidence confirming the safety of general anesthesia in pediatric dermatologic surgery.

Outpatient surgical procedures performed in medicine have been increasing, as has the use of general anesthesia. Children are more likely to require general anesthesia for procedures because of developmental constraints limiting their cooperation. Dermatology procedures are typically painful and can require time to perform and thus necessitating the use of general anesthesia. Studies evaluating safety and complication rates associated with the use of general anesthesia in children are needed to determine the safety of this practice. The purpose of this investigation was to assess whether the use of general anesthesia to perform pediatric dermatology surgical procedures was associated with any significant adverse events. A retrospective chart review was conducted for eligible patients with a procedure code in the range of 10,040-19,499 for a period of 2 years. Records were reviewed for documentation of any unplanned visits, events or complications. A total of 681 procedures were reported for 226 unique patients with 235 diagnoses. No anesthesia-related adverse events were identified in any of these patients. Eleven patients had documentation of minor complications of the wound. General anesthesia can be used safely in pediatric dermatology surgery patients without associated complications in the appropriate setting, indication, and patient population, using state-of-art technology and equipment, and experienced pediatric-trained anesthesiologists.

Immunosuppressive effects in infants treated with corticosteroids for infantile hemangiomas.

BACKGROUND: Infantile hemangiomas are the most common benign tumors of infancy. Up to 38% of hemangiomas require treatment with systemic medications because of complications. Corticosteroids have been the mainstay for treating such hemangiomas. However, prospective studies evaluating their immunosuppressive effects in infants with hemangiomas are lacking. OBSERVATIONS: Sixteen patients who presented to the Birthmark and Vascular Anomalies Center at the Children's Hospital of Wisconsin from November 1, 2006, through February 28, 2008, were enrolled in the study. A significant reduction in the numbers of all B- and T-lymphocyte subpopulations was observed after corticosteroid administration. CD19(+) B lymphocytes and CD4(+) T cells were significantly reduced by 8 weeks of corticosteroid therapy, whereas CD8(+) T cells were reduced at week 16 compared with baseline. Immune function was also affected because 13 and 5 patients had protective diphtheria titers and tetanus titers, respectively, 3 months after discontinuation of corticosteroid therapy compared with baseline. CONCLUSIONS: These results demonstrate that corticosteroids measurably affect both lymphocyte cell numbers and function in this patient population. Prophylaxis with the combination of trimethoprim and sulfamethoxazole should be considered in infants treated with corticosteroids for infantile hemangiomas. We also recommend that tetanus and diphtheria antibodies be checked in patients who receive oral corticosteroids during the immunization period and that additional immunization be administered if the titers are not protective after corticosteroid therapy.