Optimizing Surgical Treatment of Internationally Adopted Children With Cleft Lip and/or Palate: Understanding the Family Experience.

OBJECTIVE: To understand the experience of families with children undergoing cleft surgery following adoption from a country outside the United States. To identify factors, including the timing of surgery, that influence family function throughout the surgical experience. DESIGN: Semistructured qualitative interviews were conducted with parents of internationally adopted children postrepair of cleft lip and/or cleft palate and coded by a multidisciplinary study team. Mixed methods were used to contextualize themes derived from the parent interviews. RESULTS: Twenty parent interviews were conducted, and four core themes were identified: (1) parental anxieties prior to surgery, (2) considerations for the timing of surgery, (3) impact of the surgical experience on the child and family, and (4) modifiable sociocontextual factors. Parents considered a strong child bond with at least one parent and the ability of the child to communicate basic needs to be important before undergoing surgery. In retrospect, parents generally felt that the surgical experience did not have a negative impact on their child or their families and that the surgical experience may have even facilitated bonding and attachment with their child. Acceleration of family bonding was expressed more often by parents of children who were adopted at older than 2 years. CONCLUSIONS: In our study, parents reported that cleft surgery soon after international adoption did not appear to impair child bonding or adjustment. Specific family and provider factors that could optimize the experience for families were identified.

Causal attributions of cleft lip and palate across cultures.

Objective : To describe and compare the causal beliefs associated with cleft lips and/or palates across several different countries. Design : Cross-sectional survey. Setting : Operation Smile surgery screenings in six developing countries. Participants : Two hundred seventy-nine adult patients and parents of children with cleft lips and/or palates in Kenya, Russia, Cambodia, India, Egypt, and Peru. Interventions : In person interviews were conducted with interpreters. Main Outcome Measure : As part of a larger study, a semistructured questionnaire was created to explore cleft perceptions, belief systems that affect these perceptions, and social reactions to individuals with clefts. Results : Causal attributions were grouped by category (environment, self-blame, supernatural, chance, unknown, or other) and type of locus of control (external, internal, or unknown). Results indicate significant difference by country for both causal attribution category (P < .001) and type (P < .001). This difference was maintained in multivariate analyses, which controlled for differences by demographic variables between countries. Conclusions : This study provides evidence that causal attributions for clefts are influenced by culture. As harmful beliefs about cause may continue to impact affected individuals and their families even after a repair, it is insufficient to provide surgical care alone. Care of the entire person must include attempts to change misinformed cultural beliefs through educating the broader community.

Breast reduction in the irradiated breast: evidence for the role of breast reduction at the time of lumpectomy.

Given the high incidence of breast cancer in our society, it is common to encounter patients with macromastia who desire breast reduction after breast-conserving therapy by lumpectomy and radiation. We hypothesize that radiation leads to a significant increase in postoperative complications after breast reduction. All patients with a history of unilateral breast lumpectomy and radiation who subsequently underwent bilateral breast reduction by a single surgeon from 2004 to 2008 were retrospectively reviewed. Outcomes including cellulitis, wound breakdown, seroma, and need for repeat operations were compared between the radiated and nonradiated breast. The Fisher's exact test was used for statistical analysis. Twelve patients (mean age, 57 years) underwent bilateral breast reduction a mean of 86 months after unilateral lumpectomy and radiation. The nonradiated breasts had no complications postoperatively. The radiated breasts had a significant increase in complications with a total of five breasts (42%, p<0.04) having postoperative complications including cellulitis in two breasts, seroma requiring drainage in five breasts, two cases of fat necrosis, and one case of wound dehiscence. This resulted in two admissions for intravenous antibiotics and two repeat operative procedures. Additionally, three patients had significant breast asymmetry or contour deformities after reduction requiring operative revisions. Breast reduction after radiation leads to a significant increase in complications. Given this data, patients with macromastia undergoing breast conservation therapy for cancer should be considered for reduction at the time of lumpectomy and prior to radiation.

Male genital lymphedema: clinical features and management in 25 pediatric patients.

PURPOSE: Genital lymphedema in the pediatric population is poorly understood. The purpose of this study was to determine the epidemiology, morbidity, and treatment outcomes for males with genital lymphedema. MATERIALS AND METHODS: Male patients with genital lymphedema evaluated at our vascular anomalies center between 1995 and 2011 were reviewed. Etiology, age-of-onset, location, morbidity, and treatment were analyzed. RESULTS: Of the 3889 patients with vascular anomalies, 25 (0.6%) had genital lymphedema: 92% (23/25) with primary and 24.0% (6/25) with familial/syndromic lymphedema. For primary disease, the mean age-of-onset was 4.5 ± 6.3 years with 60.9% (14/23) presenting in infancy, 13.0% (3/23) in childhood, and 26.1% (6/23) in adolescence. Combined penoscrotal lymphedema was identified in 72.0% (18/25) of patients; 19 children (76.0%) had concomitant lower extremity involvement. The most common complication was cellulitis (24.0%). Surgical contouring was performed in 44.0% (11/25) of patients. Patients with operative intervention and follow-up (n=6) had sustained improvement after a median of 4.2 years (range: 0.3-11.0). CONCLUSIONS: Lymphedema of the male genitalia is typically idiopathic. Most patients develop swelling in infancy but can present in adolescence and occasionally childhood. The penis and scrotum are usually both involved and concurrent lower-extremity swelling is common. Surgical debulking can improve symptoms and appearance.

Changes in frontal morphology after single-stage open posterior-middle vault expansion for sagittal craniosynostosis.

BACKGROUND: There is controversy regarding whether the frontal bossing associated with sagittal synostosis requires direct surgical correction or spontaneously remodels after isolated posterior cranial expansion. The authors retrospectively measured changes in frontal bone morphology in patients with isolated sagittal synostosis 2 years after open posterior and midvault cranial expansion and compared these changes with those occurring in age-comparable healthy control groups. METHODS: Forty-three patients age 1 year or younger (mean, 6 months) with sagittal synostosis underwent computed tomography scan digital analysis immediately after and 2 years after posterior-middle cranial vault expansion. Quantitative angular and linear measures were taken along the midsagittal and axial planes to capture both aspects of frontal bossing. The change in values over the 2 years were compared with healthy controls with normal computed tomography scans taken to rule out head trauma. RESULTS: All measures indicative of frontal bossing decreased significantly from the time of posterior-middle vault expansion to 2 years postoperatively. Whereas the majority of patients at time of the operation had frontal bossing measures greater than two standard deviations outside the age-comparable control mean, almost all patients were within two standard deviations of the norm 2 years later. Lateral forehead bossing and anterior cranial growth was greater the older the patient was at the time of the operation, suggesting that the more time that passed before the operation, the more compensatory anterior fossa growth occurred. Central forehead position relative to the anterior cranial base was greatest in the younger patients at the time of operation, suggesting that a central forehead bulge was an early compensatory response to premature sagittal fusion. CONCLUSIONS: As a group, patients with sagittal synostosis start to normalize their forehead morphology within 2 years if an isolated posterior operation is performed at 1 year of age or younger, and this occurs by a combination of restriction of growth and reduction relative to patients without synostosis. This protocol decreases the risks of intraoperative positioning, forehead contour deformities, and two-stage operations. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Primary lymphedema: clinical features and management in 138 pediatric patients.

BACKGROUND: Lymphedema results from maldevelopment of the lymphatic system (primary) or injury to lymphatic vasculature (secondary). Primary lymphedema is far less common than the secondary condition. The purpose of this study was to determine the clinical features of primary lymphedema in the pediatric age group. METHODS: The authors' Vascular Anomalies Center database was reviewed for patients evaluated between 1999 and 2010 with onset of lymphedema before 21 years of age. Cause, sex, age of onset, location, and familial/syndromic association were determined. Morbidity, progression, and treatment were analyzed. RESULTS: Lymphedema was confirmed in 142 children: 138 cases (97.2 percent) were primary and four (2.8 percent) were secondary. Analysis of the primary cohort showed that 58.7 percent of the patients were female. Age of onset was infancy, 49.2 percent; childhood, 9.5 percent; or adolescence, 41.3 percent. Boys most commonly presented in infancy (68.0 percent), whereas girls usually developed swelling in adolescence (55.3 percent). Lymphedema involved an extremity (81.9 percent), genitalia (4.3 percent), or both (13.8 percent). The lower limb was most commonly affected (91.7 percent), and 52.9 percent had bilateral lower extremity disease. Eleven percent of patients had familial or syndromic lymphedema. Cellulitis occurred in 18.8 percent of children; 13.0 percent required hospitalization. The majority of patients (57.9 percent) had progression of their disease. Treatment was compression garments alone (75.4 percent) or in combination with pneumatic compression (19.6 percent); 13.0 percent had operative intervention. CONCLUSIONS: Pediatric primary lymphedema usually involves the lower extremities. Boys typically are affected at birth, and girls most often present during adolescence. Most patients do not have major morbidity, are successfully managed by compression, and do not require surgical treatment.

Vascular anomalies of the male genitalia.

PURPOSE: Vascular tumors and malformations of the male genitalia can affect urinary, sexual, reproductive, and emotional function. METHODS: Male patients with a genital lesion evaluated or treated at our center from 1995 to 2010 were reviewed to analyze presentation, diagnosis, treatment modalities, and outcome. RESULTS: Of the 3889 male patients, 117 had a vascular anomaly of the genitalia: 12 tumors and 105 malformations. The referring diagnosis was accurate in 72.7% of patients with a tumor, whereas 46.3% of malformations were misdiagnosed. Tumors included infantile hemangioma (n = 10) and kaposiform lymphatic anomaly (n = 2). Common vascular malformations were lymphatic (n = 46), venous (n = 33), and capillary-lymphatic-venous (n = 16). Presenting signs for tumors included ulceration (33.0%) and ambiguous genitalia (25.0%). Malformations manifested with swelling (40.0%), fluid leakage (16.2%), and pain (16.2%). Treatment was necessary for 69.9% (79/113) of patients. The remaining lesions (34/113) were observed. Tumor management included observation, pharmacotherapy, and excision. Malformations were largely treated with sclerotherapy and/or surgical procedures. CONCLUSIONS: Vascular anomalies of the male genitalia are uncommon and frequently misdiagnosed. Accurate diagnosis can be made and appropriate treatment can be instituted based on presentation, natural history, and radiographic imaging. Observation and pharmacotherapy are the mainstays of tumor management. Malformations require sclerotherapy and/or resection. Interdisciplinary care optimizes outcomes for males with these often-disfiguring vascular lesions.

Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema.

BACKGROUND: There are many causes for a large lower limb in the pediatric age group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to determine the differential diagnosis in pediatric patients referred for lower extremity "lymphedema" and to clarify management. METHODS: The authors' Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of the lower extremity. Records were studied to determine the correct cause for the enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies were also analyzed. RESULTS: A referral diagnosis of lower extremity lymphedema was given to 170 children; however, the condition was confirmed in only 72.9 percent of patients. Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic lymphatic malformation (19.6 percent), noneponymous combined vascular malformation (13.0 percent), capillary malformation (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema (6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3 percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children with lymphedema was older than in patients with another diagnosis (p = 0.027). CONCLUSIONS: "Lymphedema" is not a generic term. Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema; the most commonly confused causes are other types of vascular anomalies. History, physical examination, and often radiographic studies are required to differentiate lymphedema from other conditions to ensure the child is managed appropriately.