Pediatric Lymphedema: Study of 180 Patients Referred to a Tertiary Lymphedema Clinic.

BACKGROUND: Lymphedema is due to dysfunction of the lymphatic system. It can be primary or secondary. Pediatric lymphedema is more often primary and is a chronic disease with a heavy burden on quality of life. METHODS: Medical records of patients under 18 years of age referred between 1996 and 2021 to the specialized lymphedema clinic at the Sainte-Justine University Hospital Center were reviewed. Demographic data, sex, age at presentation, location of the lymphedema, clinical features, genetic testing, symptoms, complications, investigations, and treatment were collected. RESULTS: Of 180 referred patients, lymphedema was confirmed in 151, and 137 were primary lymphedema. Median age of apparition of primary lymphedema was 7.00 years and was significantly lower in boys than in girls. Primary congenital lymphedema was more frequent in boys (51.0%, 27.3% in girls, P = .007), and onset of primary lymphedema during adolescence was more frequent in girls (53.4%, 25.0% in boys, P = .001). Lower limbs were the most impacted (88.3%). Sixty patients had genetic testing, and 38 (63.3%) of them were discovered to have a pertinent genetic mutation. The most common mutated gene was the FLT4 gene (in 9 patients). Seven patients (5.1%) had associated extensive/central lymphatic malformation and 24 (17.6%) had a polymalformative syndrome/syndromic lymphedema. CONCLUSIONS: Pediatric lymphedema is more frequent in girls, usually involves lower limb, and is most often sporadic, but often associated with a genetic mutation, and genetic testing should be performed.

Non-involuting congenital hemangiomas (NICH) with postnatal atypical growth: A case series.

BACKGROUND: Non-involuting congenital hemangiomas (NICH) are fully formed vascular tumors at birth, with a distinctive clinical, radiologic, and histopathological profile, and classically lack expansion or involution over time. We describe a series of NICH cases with atypical postnatal growth. METHODS: The authors retrospectively analyzed all NICH cases diagnosed from 2007 to 2017. We reviewed charts and photographic databases from our Vascular Anomalies Clinic. We included in the study all NICH with an atypical postnatal growth. Clinical data, imaging, and histopathology were analyzed. RESULTS: Eighty cases of NICH were identified. Nine presented with atypical postnatal growth after a stable period, at ages from 2 to 10 years (mean: 5.3 years). Two patients had associated pain; 5 patients showed new red papules on the surface of the lesion; 2 reported bleeding from the papules; and 1 developed a pyogenic granuloma. All patients had Doppler ultrasound and/or MRI compatible with NICH, and a confirmatory biopsy was performed in 4 cases. In treatment, 2 patients received endovascular embolization, and one required further surgery. CONCLUSIONS: Non-involuting congenital hemangiomas (NICH) may develop significant postnatal growth over time (10% in our series), requiring closer follow-up for longer periods. The development of red papules, pyogenic granulomas, and superficial bleeding may be observed. Since this is a small series, we were not able to establish risk factors for NICH with postnatal growth.

Quality of life in pediatric patients before and after cosmetic camouflage of visible skin conditions.

BACKGROUND: Visible vascular and pigmentary conditions have a negative impact on children's and adolescents' quality of life (QoL). We sought to quantitate the effect of visible skin anomalies and their camouflage on QoL. METHODS: In all, 41 patients, 5 years of age and older, were taught to use cosmetic camouflage. QoL was assessed using the Children's Dermatology Life Quality Index (CDLQI) before and 6 months after the intervention. Satisfaction and use were evaluated after 1 and 6 months. RESULTS: Baseline QoL scores revealed a small impact of vascular anomalies (CDLQI score 4.2) and a small to moderate effect of pigmentary anomalies (CDLQI score 6.1). Six months after the intervention, QoL improved in the study population as a whole (CDLQI score 5.1 vs 2.1, P<.001), with significant improvements documented for facial lesions and vascular malformations. Cosmetic camouflage was well tolerated and patients with pigmentary anomalies were more likely to continue using the products. LIMITATIONS: Limitations include small study population, few male patients, cultural influences not addressed, and limited range of conditions. CONCLUSIONS: Children and teenagers with visible vascular and pigmentary anomalies experience an impairment of QoL that is abrogated by introduction to use of cosmetic camouflage.

Late rebound of infantile hemangioma after cessation of oral propranolol.

Propranolol has become the first line of treatment for infantile hemangiomas (IHs), with a high response rate, but rebound growth after cessation of propranolol has been reported, primarily in the first year of life. We sought to determine the frequency and associated factors leading to late regrowth after successful treatment at an age when the proliferative phase has usually ceased. We retrospectively reviewed the clinical charts, serial photographs, and radiologic images of children with rebound IH occurring after the age of 15 months after a successful course of oral propranolol averaging 2.6 mg/kg/day (range 2-3 mg/kg/day). Thirteen (10 female, 3 male) of 212 patients (6%) treated with oral propranolol since 2008 were evaluated. The mean age at the start of treatment was 5.3 months (range 1.8-13 months), and an average of 10.3 months (range 4.5-16 months) of treatment was given. It took an average of 5.3 months (range 1-13.8 months) for a significant rebound to appear. Late rebound after successful propranolol indicates a prolonged proliferation phase of IH even after 15 months of age. This is compared with previous reports of rebound, which occurred primarily in infants younger than 1 year old. Late proliferation can occur in localized, small, mixed, and deep IH, even after several months of a positive response to propranolol. A second course of propranolol readily controlled the recurrence.

Therapy of ulcerated hemangiomas.

BACKGROUND: Cutaneous ulceration is the most common complication of infantile hemangiomas (IHs) seen in a pediatric dermatology practice. OBJECTIVE: The most effective treatments in our experience are compared to those in the current literature. METHODS: The study was a retrospective chart review of therapy of 169 ulcerated IHs at a tertiary care pediatric hospital and a literature review. RESULTS: Combination therapy was the rule. Local wound care was required in all, pain management in 72%, pulsed dye laser in 42%, infection control in 38%, diminution of the hemangioma through systemic therapy in 36%, and suppression of bleeding in 2%. LIMITATIONS: A retrospective review compared to a case-control study has inherent bias. In addition, our cases were all at a tertiary referral center. CONCLUSION: All ulcerated IHs benefit from local barrier creams or dressings. Pulsed dye laser, antibiotics, topical morphine 0.1% in hydrogel, topical becaplermin, and, most importantly, systemic therapy (especially propranolol) to reduce the hemangioma may be useful.

Propranolol in the treatment of problematic infantile hemangioma: review of 35 consecutive patients from a vascular anomalies clinic.

BACKGROUND: Propranolol, a nonselective ß-blocker, has been reported as efficient for controlling the growth of complicated infantile hemangiomas (IHs). No uniformly accepted protocol exists regarding the administration of oral propranolol for IH. OBJECTIVE: We sought to share our experience using propranolol for problematic IH and to evaluate the efficacy of this treatment modality. METHODS: A retrospective chart review analysis was performed for 35 consecutive children treated with propranolol as an oral solution on an outpatient basis in our dermatology/vascular anomalies clinic. A protocol was established with the help of our pediatric cardiologists, including pretreatment electrocardiography and echocardiography. Medical photographs taken after 2 months of treatment were rated by two independent evaluators. RESULTS: We treated 31 girls and 4 boys with a median age of 3.5 months. Rapid improvement was reported in the first days of treatment in 34 patients. Mean improvement after 2 months was 61.5%. No serious adverse effects were reported. CONCLUSION: Propranolol was effective in controlling the proliferative phase of problematic IH. It was well tolerated in our study. Outpatient treatment is possible if parents follow strict guidelines. Propranolol should be a first-line treatment for problematic IH in carefully selected patients.

Propranolol in the treatment of problematic infantile hemangioma: review of 35 consecutive patients from a vascular anomalies clinic.

BACKGROUND: Propranolol, a nonselective ß-blocker, has been reported as efficient for controlling the growth of complicated infantile hemangiomas (IHs). No uniformly accepted protocol exists regarding the administration of oral propranolol for IH. OBJECTIVE: We sought to share our experience using propranolol for problematic IH and to evaluate the efficacy of this treatment modality. METHODS: A retrospective chart review analysis was performed for 35 consecutive children treated with propranolol as an oral solution on an outpatient basis in our dermatology/vascular anomalies clinic. A protocol was established with the help of our pediatric cardiologists, including pretreatment electrocardiography and echocardiography. Medical photographs taken after 2 months of treatment were rated by two independent evaluators. RESULTS: We treated 31 girls and 4 boys with a median age of 3.5 months. Rapid improvement was reported in the first days of treatment in 34 patients. Mean improvement after 2 months was 61.5%. No serious adverse effects were reported. CONCLUSION: Propranolol was effective in controlling the proliferative phase of problematic IH. It was well tolerated in our study. Outpatient treatment is possible if parents follow strict guidelines. Propranolol should be a first-line treatment for problematic IH in carefully selected patients.

Propranolol versus prednisone in the treatment of infantile hemangiomas: a retrospective comparative study.

The goal of the current study was to compare the clinical effectiveness of oral propranolol with that of oral prednisone in the treatment of infantile hemangiomas (IH). Patients treated for IH with oral propranolol were retrospectively matched with patients treated with oral prednisone according to type, location, and size of the IH and age at start of treatment. Response to treatment was evaluated by rating serial medical photographs taken 1, 2, and 6 months after initiation of treatment. Degree of clinical improvement in overall appearance (including color and size) was rated as follows: worse or stable (0), slight (<25%), moderate (25-50%), good (50-75%), or excellent (>75%). A second assessment was done using a 100-mm visual analog scale to rate improvement at 6 months. Pre and post-treatment imaging was available for several patients. Twelve pairs of infants with IH were analyzed. At 1 month, clinical improvement in the propranolol group was moderate to good in all patients. In the prednisone group, only one patient had moderate improvement, with others showing slight (7/12) or no improvement or stabilization (3/12) from baseline and one case worsening. At 6 months, the propranolol group showed good to excellent response in all cases, whereas nine in the prednisone group showed slight to moderate response. Doppler ultrasound and magnetic resonance imaging correlated with the clinical improvement in the cases in which it was performed. No major side effects were observed in either group. Propranolol appears superior to oral prednisone in inducing more-rapid and greater clinical improvement in this study. A larger prospective study comparing these two treatment modalities is warranted.