Richard G Azizkhan

Richard G Azizkhan
Cincinnati Children's Hospital Medical Center | CCHMC · Surgical Services

MD, PhD (hon)

About

156
Publications
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Publications

Publications (156)
Chapter
Malignant tumors diagnosed during the neonatal period are rarely encountered but extremely difficult to manage. Because factors such as drug absorption, metabolism, distribution, and elimination are affected by age and physiologic maturity, complications associated with the immature physiology of the neonate are common. Age-dependent maturation of...
Article
Full-text available
Background and objectives: Complicated vascular anomalies have limited therapeutic options and cause significant morbidity and mortality. This Phase II trial enrolled patients with complicated vascular anomalies to determine the efficacy and safety of treatment with sirolimus for 12 courses; each course was defined as 28 days. Methods: Treatment...
Article
The most common hepatic vascular tumor in the pediatric population is the infantile hepatic hemangioma. Although these lesions have a spectrum of presentations, there are three main subtypes that have been described – focal, multifocal and diffuse. An algorithm on the work-up, treatment and follow-up of these lesions can be based on this categoriza...
Article
A multicenter study of pectus excavatum was described previously. This report presents our final results. Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and p...
Article
Full-text available
The classification system for vascular anomalies now used by experts worldwide comprises two distinct disease entities that differ in their biologic and pathologic features: vascular tumors and vascular malformations. Vascular tumors include infantile and congenital hemangiomas, tufted angiomas, and kaposiform hemangioendotheliomas. Infantile heman...
Article
Junctional epidermolysis bullosa (JEB) is a particularly devastating type of epidermolysis bullosa, especially in the newborn period. Data about the number of new cases of JEB in the United States were collected from the records of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator. Seventy-one children with...
Article
Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas...
Article
To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB). We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010. Twenty-five patients with JEB were identified, and 12 patien...
Chapter
This chapter focuses on surgical interventions for treating chyle reflux. As with many areas of vascular medicine, multidisciplinary management is the norm, with surgeons, medical specialists, and interventional radiologists all playing important roles. An understanding of chyle transportation from the bowel lacteals to the ­thoracic duct and venou...
Article
Epidermolysis bullosa (EB) has become recognized as a multisystem disorder that poses a number of pre-, intra-, and postoperative challenges. While anesthesiologists have long appreciated the potential difficult intubation in patients with EB, other systems can be affected by this disorder. Hematologic, cardiac, skeletal, gastrointestinal, nutritio...
Article
Dilated cardiomyopathy (DC) has been reported in severe epidermolysis bullosa (EB) subtypes. Poor nutritional status, low carnitine levels, selenium deficiency, chronic iron overload, drugs and viral etiology have been proposed as potential contributors. This was a retrospective, descriptive, multicenter study describing EB patients that developed...
Article
Epidermolysis bullosa (EB) is a spectrum of rare, inherited, blistering skin disorders, primarily affecting the skin and pharyngoesophageal mucosa. EB affects approximately 2 to 4 per 100,000 children each year. Blistering and scarring occur in response to even the most minor trauma. In this article, the authors outline the potential management opt...
Article
The availability of pediatric subspecialty care is critically important to the health and well-being of infants, children, and adolescents. Moreover, timely collaboration with pediatric subspecialists is an essential element of the standard of care for children: the community-based medical home. In this model, primary care practice teams coordinate...
Article
To determine the prevalence of adrenal insufficiency in infants with hemangiomas following treatment with systemic glucocorticoids (GCs). Prospective study for 18 months. Hemangioma and vascular malformation center at a tertiary care children's hospital. Sixteen infants with hemangiomas had an adrenal axis evaluation as soon as possible following t...
Article
Esophageal atresia/tracheoesophageal fistula (EA/TEF) repair using an open muscle-sparing thoracotomy has been the standard approach used in our institution. Whereas perioperative mortality is now very uncommon, short- and long-term morbidity is very common in these patients. However, the complexity of the esophageal anatomy and significant comorbi...
Article
1 +Reyes MLCattani AGajardo HGarcia CMcGrath JAPalisson F Bone metabolism in children with epidermolysis bullosa. J Pediatr 2002;140 (4) 467- 469PubMed Link to Article[[XSLOpenURL/10.1067/mpd.2002.123287]]2 +Fewtrell MSAllgrove JGordon I et al. Bone mineralization in children with epidermolysis bullosa. Br J Dermatol 2006;154 (5) 959- 962PubMed Lin...
Article
Infants with multiple cutaneous hemangiomas often present with hepatic hemangiomas. They can follow a benign clinical course or require complex management. We reviewed our experience in the management of hepatic hemangiomas. We performed a retrospective review of patients (1996-2007) with hepatic hemangiomas treated in our institution. Twenty-six p...
Article
Full-text available
This study evaluated changes in both physical and psychosocial quality of life reported by the parent and child after surgical repair of pectus excavatum. As part of a multicenter study of pectus excavatum, a previously validated tool called the Pectus Excavatum Evaluation Questionnaire was administered by the research coordinator, via telephone, t...
Article
Full-text available
The utility of laparoscopic appendectomy (LA) in children remains controversial. The determination of the efficacy of LA in children is complicated by variable postoperative management, duration of antibiotics,and criteria for discharge. The aim of this study was to examine the results of a commitment to LA and the concurrent implementation of an e...
Article
Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung le...
Article
Xanthogranulomas larger than 2 cm are rarely reported variants of this otherwise common, benign lesion usually measuring 1 mm to 1 cm in diameter. The natural history of such macronodular xanthogranulomas is uncertain, but appears to parallel the generally predictable course toward involution seen in micronodular lesions. Two patients with macronod...
Article
Indications for treatment of lymphatic malformations include disfigurement, symptoms, and infection. Patients with lymphatic malformations often undergo resection or sclerotherapy to prevent possible complications, as it has been thought that lymphatic lesions do not regress. A retrospective analysis of all head and neck lymphatic malformations for...
Article
Full-text available
To provide clinicians with an overview of the complications and functional and clinical outcomes of children affected by massive lymphatic malformations affecting multiple sites in the head, neck, and/or chest. We reviewed the medical records of all patients who received treatment for massive cervicofacial lymphatic malformations at our tertiary ca...
Article
Recessive dystrophic epidermolysis bullosa (RDEB) is associated with high nutritional demands, esophageal strictures and dysphagia. About one quarter of the patients require gastrostomy tube placement to maintain adequate nutritional status. To minimize trauma to the skin and pharyngoesophageal mucosa caused by commonly used gastrostomy tube insert...
Article
Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management. This observational study followed pectus exca...
Article
In light of the paucity of literature on lymphatic malformations of the tongue base, our aim was to present our experience and long-term outcomes of patients with this rare and challenging pathologic entity. Medical records of 25 patients treated by the 3 senior authors (RGA, MTC, and RTC) between 1974 and 2003 were retrospectively reviewed, and co...
Article
Vascular malformations are the second major category of vascular anomalies. In contrast to vascular tumors, they are present at birth and grow commensurately with the child. Although the molecular mechanisms underlying the formation of these lesions remain unclear, lesions are known to result from abnormal development and morphogenesis. Histologic...
Article
During repair of an esophageal atresia with tracheoesophageal fistula (EA/TEF), a right-sided aortic arch (RAA) presents a technical challenge. We reviewed our experience with EA/TEF focusing on the impact of RAA. A retrospective review of patients with EA/TEF at our institution from 1990 to 2004 was performed. Of 61 patients, 53 had a left aortic...
Article
Although surgery has been the mainstay of treatment of chondrogladiolar pectus carinatum (PC), several authors have advocated the benefits of nonoperative approaches to induce chest wall remodeling. Based on our initial success with compression bracing, we have integrated this modality into our treatment algorithm. We reviewed the charts of all pat...
Article
Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder that is associated with significant esophageal strictures, resulting in dysphagia and nutritional failure. Although endoscopically guided balloon dilatation is a widely used treatment, the use of an endoscope carries the risk of oropharyngeal trauma. To minim...
Article
In this paper, we describe the development of a new approach to mounting silicon pressure microsensors inside a 5 French (1.67 mm outer diameter) neonatal catheter tube for intravascular blood pressure measurements. Kapton film was used as a low-cost, flexible interface between the flip-chip bonded sensors at one end and wires to external electroni...
Conference Paper
A pressure microsensing catheter for neonatal care has been developed to measure intravascular blood pressure. The design uses polymer flip-chip bonding on a flexible Kapton film for mounting silicon pressure microsensors into the 5 French dual lumen neonatal catheters (1.67 mm o.d.). While other groups have mounted MEMS sensors on guide wires, our...
Article
Doxorubicin (Dox) is one of the most useful chemotherapeutic agents for patients with advanced neuroblastoma (NB). A series of Dox analogs with bulky substitutions at the C-4' at amino-sugar have been designed to impair interactions between the drug and P-glycoprotein (P-gp), a multidrug drug resistance (MDR) transporter. Two analogs, WP744 and WP7...
Article
Our aim was to present our experience with complications caused by placement of metallic stents in the pediatric airway. We conducted a retrospective study of the medical records of patients with complications resulting from metallic stent placement, managed by the senior authors between 1993 and 2002. Nine children had complications associated wit...
Article
Objectives: To describe complications encountered in association with massive cervical lymphangiomas and treatment outcome as a function of lesion size, location and involvement.Methods: A retrospective chart review of all patients diagnosed with lymphangioma of the head and neck from January 1985 to December 2002 at a tertiary care pediatric cente...
Article
Recent research pertaining to laser applications for the treatment of capillary malformations (port wine stains) and cutaneous and subglottic hemangiomas is reviewed within the context of advances in laser technology and current therapeutic practices and controversies. Although laser advances have resulted in the concomitant growth of new clinical...
Article
Although rare, complete tracheal rings are the most common cause of congenital tracheal stenosis. The last 2 decades have seen an evolution in management, with increasing awareness of the potential advantages of slide tracheoplasty. Between March 2001 and August 2002, 11 children had complete tracheal rings corrected by slide tracheoplasty. Ages ra...
Article
In 1978, Drs. Fischer and Martin were among the first to preserve anorectal continence and create a pelvic reservoir in adult patients, in what has become the ileal pouch-anal anastomosis (IPAA). Here we review our institutions' experience with 379 of these procedures from 1978 to present. To assess the specific health concerns of patients with ulc...
Article
For children with perforated appendicitis, the use of a prolonged course of intravenous (i.v.) antibiotics is equivalent to a short course of i.v. antibiotics followed by sequential conversion to oral (PO) antibiotics. Prospective, randomized, clinical trial. Multicenter study in tertiary children's hospitals. Children (aged 5-18 years) with perfor...
Article
Young patients with differentiated thyroid cancer typically present with regional lymph node involvement (60% to 80%), and 10% to 20% have distant metastases. This study characterizes the clinical presentation, treatment, and outcome in patients with differentiated thyroid cancer who were less than 21 years of age at diagnosis and who presented wit...
Article
Juvenile papillomatosis of the breast ("Swiss cheese disease") is a benign localized proliferative condition of the breast which occurs almost exclusively in young adult women. Patients with this lesion often have a family history of breast carcinoma, and rarely carcinoma may coexist with the lesion at the time of diagnosis. We present two cases of...
Article
Gastrointestinal bleeding in infants and children can be quite alarming and anxiety-provoking for parents and caregivers alike. In most cases the cause is benign, and the course self-limited. However, in the patient with significant bleeding, an aggressive diagnostic approach is warranted. The differential diagnosis can be extensive and varies depe...
Article
Intestinal vascular malformations in infants and children are rare but must be considered in the differential diagnosis of gastrointestinal bleeding. Many vascular malformation syndromes have associated intestinal lesions. Localization of vascular lesions is essential for successful management. A variety of treatment options including laser photoab...
Article
Appendicitis remains a difficult diagnosis in children. Ultrasonography is increasingly used for the diagnosis of appendicitis, although the proper clinical role for this test remains unclear. To evaluate the clinical utility of ultrasonography in appendicitis, the authors analyzed prospectively all children evaluated for possible appendicitis from...
Article
We undertook a retrospective study of pediatric blunt splenic injuries treated nonoperatively at our institution from 1990 to 1995 (n = 72) to assess the impact of serial computed tomography (CT) scans on the outcome. Median number of studies per child was three. The result showed that the appearance of the splenic injury remained unchanged or impr...
Article
Commonly encountered head and neck lesions in children are described with an emphasis on evaluation, diagnosis, and treatment. Congenital lesions typically require excision, although hemangiomas usually resolve spontaneously. Acute suppurative lymphadenitis is common and readily diagnosed. Chronic lymphardenitis remains a diagnostic challenge and m...
Article
Cervical extracorporeal membrane oxygenation (ECMO) cannula position is often difficult to confirm by chest x-ray alone. Malposition requires a second surgery to rectify the problem. Reoperation places the patient at risk for infection, bleeding, or death. This study analyzes indications for cannula repositioning and suggests an alternative standar...
Article
Currarino's triad is a congenital malformation involving the combination of anorectal stenosis, a presacral mass (most often a teratoma or ventral menigocele) and an anterior sacral bony defect (scimitar sacrum). Current theories regarding its embryogenesis are difficult to reconcile with our current understanding of caudal neuraxial and hindgut de...
Article
This study was done to define the extent of disease and evaluate the effect of staging and treatment variables on progression-free survival in patients with differentiated thyroid carcinoma who were less than 21 years of age at diagnosis. Differentiated thyroid cancer in young patients is associated with early regional lymph node involvement and di...
Article
Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the effect of therapy on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT). The authors conducted a retrospective review of children with SCT treated at 15 Childrens Cancer G...
Article
Purpose: The regional pediatric trauma center in Buffalo, NY, has been active in pediatric injury prevention programs, including community education and distribution of bicycle helmets, since 1990. Since June 1, 1994, the use of bicycle safety helmets for children under 14 years of age has been mandated by a state law in New York. The authors unde...
Article
The authors investigated whether amelioration of intestinal mucosal injury, due to ischemia-reperfusion (I/R), with oxygenated perfluorocarbon (PFC) would reduce an oxidant-generated lung injury. The small intestine is increasingly recognized as a primary effector of distant organ injury. Clinical and experimental studies suggest oxidant species an...
Article
To define changes in hematocrit (Hct) and the indications for blood transfusion in pediatric blunt splenic injury. Retrospective case series All children with blunt splenic injuries from 1990 to 1995 were studied (n = 74). Transfusion practices were reviewed for the whole group. Thirty children with isolated splenic injuries who were not transfused...
Article
Objective: To define changes in hematocrit (Hct) and the indications for blood transfusion in pediatric blunt splenic injury. Design: Retrospective case series Materials and Methods: All children with blunt splenic injuries from 1990 to 1995 were studied (n = 74). Transfusion practices were reviewed for the whole group. Thirty children with isolate...
Article
Ischemia/reperfusion (I/R) injury to the intestinal mucosa occurs in several commonly encountered clinical situations, such as necrotizing enterocolitis and nonocclusive mesenteric insufficiency. No clinically feasible technique is available for mucosal preservation during ischemia. The goal of this work was to determine whether the continuous intr...
Article
Selective left ventricular hypoplasia is a recognized feature of congenital diaphragmatic hernia (CDH). It is speculated that the herniated viscera act as a space occupying lesion that inhibit normal cardiac development. The purpose of this study was to determine the effect of two separate fetal surgical interventions on subsequent cardiac developm...
Article
Because nitric oxide (NO) dilates vascular smooth muscle cells, a deficiency of endogenous pulmonary nitric oxide production by nitric oxide synthase (NOS) has been suggested to be involved in the pathophysiology of pulmonary hypertension in congenital diaphragmatic hernia (CDH). Our aim was to determine whether experimentally induced CDH in rats r...
Article
In the pediatric population, there is strong evidence to suggest that a delay in treatment results in an increased risk of appendiceal perforation. However, it is not clear whether this delay arises from the parent seeking medical advice, the referring physician seeking surgical consultation, or the surgeon deciding to operate. To resolve this issu...
Article
Surfactant therapy given before the onset of ventilation (surfactant prophylaxis) has been shown to improve oxygenation, ventilation, and pulmonary hemodynamics in the lamb model of congenital diaphragmatic hernia (CDH). The aim of this study was to assess the efficacy of surfactant administered after the onset of ventilation ("surfactant rescue")....
Article
The in vitro effects of perfluorocarbon (PFC) on human neutrophil activation and chemotaxis were examined. Neutrophils were incubated, with and without PFC, and were analyzed for chemotaxis through 5-microns and 8-microns pore filters. Neutrophil activation was quantitated by flow cytometry. Activation studies showed that PFC-treated neutrophil sam...
Article
Teratomas are embryonal neoplasms consisting of tissues from at least two of the three germ layers. Teratomas can occur in almost any region of the body and in any organ, but they are most commonly observed in the paraxial and midline locations. Excluding teratomas of the testes, 75% to 80% of teratomas occur in females. Approximately 80% are benig...
Article
Left ventricular disproportion (decreased left-to-right ventricular internal diameter ratio) has been correlated with fetal or neonatal survival in cases of congenital diaphragmatic hernia (CDH). Because cardiac development is intimately related to lung development in the normally developing fetus, the authors sought to determine whether cardiac we...
Article
Since 1990 we have evaluated 12 children and teenagers in whom severe cardiorespiratory symptoms have developed due to failure of chest wall growth after very extensive pectus excavatum operations (removal of five or more ribs) at very early ages (< 4 years). Apparently these extensive procedures have removed or prevented growth center activity, wh...
Article
Congenital chylous ascites (CCA) results from maldevelopment of the intra-abdominal lymphatic system. Newborns present with respiratory distress and abdominal distention. Medical treatment of CCA is often prolonged. A newborn with CCA was treated with octreotide, a somatostatin analogue, resulting in rapid and complete resolution of the ascites.
Article
Experiments using animal models of neonatal respiratory distress syndrome have shown a decrease in pulmonary vascular resistance (PVR) with surfactant replacement, whereas studies with the lamb model of congenital diaphragmatic hernia (CDH) have demonstrated improvement in oxygenation and lung mechanics with this therapy. The aim of the present stu...
Article
Experimental tracheal ligation (CDH + TL) has been shown to reverse the profound lung hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The aim of this study was to determine whether this experimental therapy would correct the surfactant deficiency present in the fetal lamb model of CDH. The CDH lamb mo...
Article
Mycotic infections have become an increasingly common problem in immunosuppressed pediatric patients. The management of fungal infections requires not only the recognition of the potential for infection, but also of the types of organisms that can cause infection. The identification of the organ system(s) affected is important in directing therapy....
Article
To characterize the natural history and outcome of spontaneous pneumothorax in patients under age 16 years. This is a retrospective review in which all patients admitted to the authors' institution (between December 1980 and July 1993) with a diagnosis of spontaneous pneumothorax were included. Information was obtained from the hospital records. Th...
Article
The pathophysiologic features of congenital diaphragmatic hernia (CDH) include pulmonary hypoplasia, pulmonary hypertension, surfactant deficiency, and decreased pulmonary compliance. When the surfactant deficiency is corrected using exogenous surfactant therapy, the pulmonary compliance improves, but does not reach normal values. Quasistatic salin...
Article
A multi-institutional study was conducted by the Children's Cancer Group (CCG) to evaluate all soft tissue sarcomas diagnosed within the first month of life. A retrospective study by 11 CCG institutions of patient records from 1971 to 1991 were reviewed for demographic data, pathology, therapy, and outcome. 32 neonates with soft tissue sarcomas wer...