Complex spinal-paraspinal fast-flow lesions in CLOVES syndrome: analysis of clinical and imaging findings in 6 patients.

CLOVES syndrome is a complex disorder of congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal/scoliosis/spinal anomalies. We report the occurrence of spinal-paraspinal fast-flow lesions within or adjacent to the truncal overgrowth or a cutaneous birthmark in 6 patients with CLOVES syndrome.

Klippel-Trenaunay syndrome and spinal arteriovenous malformations: an erroneous association.

BACKGROUND AND PURPOSE: KTS is a rare limb overgrowth disorder with slow-flow vascular anomalies. This study examines the presumed association between KTS and spinal AVMs. MATERIALS AND METHODS: We performed a MEDLINE search of articles and reviewed textbooks of spinal diseases to study the association between KTS and spinal AVM. Our goal was to ascertain the basis on which the diagnosis of KTS was established and to evaluate the evidence of its association with spinal AVMs. In addition, the data base of the Vascular Anomalies Center at Children's Hospital Boston was queried for patients with KTS, and the association with spinal AVM was investigated. RESULTS: Twenty-four published reports on spinal AVMs in 31 patients with KTS were reviewed. None of these references provided solid evidence of the diagnosis of KTS in any patient. Clinical data were either incompatible with the diagnosis of KTS or were inadequate to establish the diagnosis. Alternative possible diagnoses (CLOVES syndrome and CM-AVM) were suggested by the first author for 9 of the patients reported in these articles. The medical records of 208 patients with the diagnosis of KTS were analyzed; not a single patient had clinical or radiologic evidence of a spinal AVM. CONCLUSIONS: An association between KTS and spinal AVM, as posited in numerous references, is most likely erroneous. The association has neither been reliably proved in the limited published literature nor encountered in a large cohort.

Infantile hemangiomas involving the neuraxis: clinical and imaging findings.

BACKGROUND AND PURPOSE: The neuroradiology and neurosurgery literature is replete with references to "hemangioma" involving the central nervous system (CNS). However, the number of cases of true infantile hemangiomas in the CNS reported to date is 15. Our purpose was to delineate the definition of infantile hemangiomas, determine their prevalence in the neuraxis, and describe their imaging characteristics and associations in this location. MATERIALS AND METHODS: We reviewed our Vascular Anomalies Center data base from 1999 through May 2008 to assess the prevalence of intracranial or intraspinal involvement within the total cohort of infantile hemangiomas. Fifteen patients were identified with infantile hemangiomas that involved the neuraxis. Two board-certified neuroradiologists reviewed the available imaging of these 15 patients, and a board-certified pathologist reviewed the available histopathology. Clinical records of all 15 patients were reviewed to identify the type of treatment and the treatment response. RESULTS: Of the 1454 patients listed with infantile hemangioma, 15 (approximately 1.0%) had involvement of the CNS. Eight patients had intracranial infantile hemangioma, 6 had intraspinal hemangioma, and 1 had both. In most instances, there was continuous extension into the neuraxis from an extracranial or extraspinal lesion. There were no cases of a CNS hemangioma without an accompanying extra-CNS tumor. Two patients had findings consistent with posterior fossa anomalies, cervicofacial hemangioma, arterial anomalies, cardiac defects, ocular abnormalities, and associated sternal or ventral defect. Of note, there were no brain or spinal parenchymal signal-intensity abnormalities, and there was no evidence of parenchymal invasion. CONCLUSIONS: CNS involvement by infantile hemangiomas is an unusual occurrence, which, when recognized, can help optimize patient management.

Visceral vascular anomalies.

Gastrointestinal endoscopy is an essential modality often used for initial diagnostic assessment and staging of visceral vascular anomalies, especially when bleeding is the presenting symptom. Some lesions have a pathognomonic appearance on endoscopy. Others are less clearly identifiable and require a multidisciplinary assessment, including histopathology, for a correct diagnosis. Proper application of nomenclature is crucial to prevent the institution of improper therapies. Advanced endoscopic methods, including endosonography and various hemostatic techniques, are useful to evaluate the depth and character of gastrointestinal wall involvement and to provide minimally invasive treatment when appropriate.

Aggressive multimodal treatment of pleuropulmonary blastoma.

Pleuropulmonary blastoma is a rare intrathoracic neoplasm almost solely confined to childhood. Survival is poor. The authors report 2 children with extensive intrathoracic disease who are long term survivors after multimodal therapy. Both children received multiagent neoadjuvant chemotherapy, followed by surgical resection to remove all gross tumor. Postoperative chemotherapy was given to both children; radiotherapy was also given in the second case because of a question of positive tumor margins. Experience supports the use of multimodal therapy, including an aggressive surgical approach in the potentially curative treatment of this tumor.

Life-threatening anomalies of the thoracic duct: anatomic delineation dictates management.

Congenital anomalies of the thoracic duct are rare, poorly characterized, and difficult to manage. The spectrum of pathophysiologic perturbations, presenting symptoms, radiographic findings, and interventions performed in 4 patients are shown. Accurate anatomic delineation of the malformation was only possible by direct injection contrast lymphangiography. Therapies tailored to address the anatomic aberrations included intralesional sclerotherapy, surgical excision and ligation, lymphovenous anastomosis, and omental interposition to interrupt dysfunctional collateral lymphatics to the lung. Accurate anatomic diagnosis of central lymphatic channel anomalies by contrast lymphangiography facilitates an individualized multidisciplinary approach to repair.

Extrapleural thoracoscopic anterior spinal fusion: a modified video-assisted thoracoscopic surgery approach to the pediatric spine.

Video assisted thoracoscopic surgery (VATS) has recently been developed as an alternative to thoracotomy for anterior spinal surgery. We report a case in which an extrapleural dissection was combined with VATS to further improve this approach.

Perioperative considerations in a hypothyroid infant with hepatic haemangioma.

Hepatic haemangiomas in infants are rare. An infant with both a hepatic haemangioma and a severe hypothyroid condition, unresponsive to conventional thyroxine therapy, will be described. This case presented here is the perioperative management of a critically ill infant who had myocardial depression secondary to hypothyroidism and a hepatic haemangioma that required embolization. To our knowledge, this is the first published report describing intravenous triiodothyronine as a therapeutic modality to stabilize a hypothyroid infant prior to undergoing a general anaesthetic.

Nonfixation of an atretic colon predicts Hirschsprung's disease.

PURPOSE: After noting the colon to be nonfixed and coiled in the pelvis of a patient with colonic atresia and total colonic Hirschsprung's disease, the authors sought to determine whether this colonic position might be predictive of aganglionosis in the atretic colon. METHODS: The authors reviewed all cases of colonic atresia treated in their institution over the past 2 decades with regard to colonic orientation and the presence of aganglionosis. RESULTS: We identified 9 patients with colonic atresia. Two of these patients, as well as one patient with ileal atresia, also had long-segment Hirschsprung's disease. All 3 of these patients had foreshortened nonfixed colons located predominantly in the pelvis. None of the other 6 patients with colonic atresia had this configuration. Reviewing 12 reported cases from other institutions of total colonic aganglionosis associated with atresia, 7 appear to have had a pelvic nonfixed colon; colonic orientation could not be determined from the remaining reports. The diagnosis of Hirschsprung's disease was not established in any case before repair of the atresia, and each patient required a secondary enterostomy. CONCLUSIONS: An early gestational atresia, occurring before secondary retroperitoneal fixation of the colon at approximately 11 weeks may result in interruption of caudal migration of enteric nerves manifesting as Hirschsprung's disease distal to the atresia. Moreover, the aganglionosis may be predicted by a foreshortened, nonfixed colon coiled in the pelvis. Thus, biopsy of the colon should be performed at initial exploration in all cases of atresia in which the colon is not properly fixated to avoid immediate or delayed anastomosis to an aganglionic colon.

Costs and effectiveness of ultrasonography and limited computed tomography for diagnosing appendicitis in children.

BACKGROUND: A protocol of ultrasonography (US) followed by computed tomography with rectal contrast (CTRC) has been shown to be 94% accurate in the diagnosis of acute appendicitis in children. OBJECTIVE: To evaluate the changes in patient management and costs of a protocol using US and CTRC in the evaluation of appendicitis in children. DESIGN, SETTING, AND SUBJECTS: Prospective cohort study of 139 children between 3 and 21 years of age who had equivocal clinical findings for acute appendicitis seen in the emergency department of a large, urban pediatric teaching hospital between July 1998 and December 1998. PROTOCOL: Children with equivocal clinical presentations for acute appendicitis were prospectively evaluated with US. Patients with positive findings for acute appendicitis went directly to the operating room. Patients with negative or equivocal findings on US underwent CTRC. Surgical management plans were recorded before imaging, after US, and after CTRC. MAIN OUTCOME MEASURES: Surgical management plans before and after the imaging protocol as well as total hospital direct and indirect costs incurred or saved by each change in management were determined. Costs were obtained through the hospital's cost database and by ratios of costs to charges. RESULTS: Of the 139 children, the protocol resulted in a beneficial change in management in 86 children (61.9%), no change in management in 50 children (36.0%) and an incorrect change in management in 3 children (2.1%). US alone resulted in a beneficial change in management decision in 12/31 children (38.7%), while US followed by CTRC resulted in a beneficial change in management in 74/108 children (68.5%). The protocol resulted in a total cost savings of $78 503.99 or $565/patient. CONCLUSION: A protocol of US followed by CTRC in children with negative or equivocal US examinations results in a high rate of beneficial change in management as well as in total cost savings in children with equivocal clinical presentations for suspected appendicitis.

Living on the edge: a phenomenological study of medically uninsured working Americans.

An estimated 35 to 50 million Americans have no medical insurance; the vast majority are employed persons and their dependents. This phenomenological study was developed to make visible the experience of working Americans living on the edge-forced to walk a fine line between health and illness without the safety net of medical insurance. A purposive sample of 12 individuals was asked, "What is it like to be working and without medical insurance? Based on textual analysis, using an adaptation of Colazzi's method, themes were grouped into four theme clusters: A Marginalized Life, Up Against Rocks and Hard Places, Making Choices-Chancing It, and Getting By-More or Less. These are illustrated through commentary and direct quotation to depict an overall sense of the experience. Implications for nurses charged with addressing the needs of the medically uninsured and for nursing as a whole are discussed.

Perforated appendicitis: prospective outcome analysis for 150 children.

BACKGROUND/PURPOSE: Controversy persists in the management of perforated appendicitis with regard to antibiotic choice and duration, operative timing, drain utilization, and wound closure. For 2 decades at the authors' institution, patients were treated with ampicillin, gentamicin, and clindamycin for 10 inpatient days, with drains in the abdomen, resulting in lower complication rates than most other published series. Managed care pressures have led to less aggressive medical management regimens with length of stay and financial factors viewed as principal outcome measures with little emphasis on clinical outcomes. In addition, there are little prospective data on clinical outcomes. The authors sought to determine whether our previously documented excellent quality outcomes could be maintained when modifications aimed at decreasing cost and length of stay in our protocol were instituted. METHODS: The authors monitored prospectively clinical outcomes in patients with perforated appendicitis treated according to their clinical practice guidelines over a 43-month period. Patients received a single antibiotic, piperacillin-tazobactam, intravenously for 10 days. They were permitted to go home with a percutaneous intravenous catheter for the final 5 days if medical and social criteria were met. Other practices from our earlier protocol were continued, including immediate operation, placement of Penrose drains, and primary wound closure. RESULTS: Of 150 patients treated on our protocol, major complications included intraabdominal abscess in 5 (3.3%), cecal fistula in 2 (1.3%), phlegmon in 3 (2.0%), wound infection in 4 (2.7%), and no small bowel obstructions requiring operation. None of these complications, nor their aggregate, were significantly more common than those reported in 373 patients treated over 11 years on the authors' prior protocol (chi2, P > .05). CONCLUSIONS: Prospective outcome analysis of our protocol shows that a single broad-spectrum antibiotic (allowing portions of therapy to be delivered less expensively on an outpatient basis) effectively can treat postoperative appendicitis with very few infectious complications. These outcome data provide baseline against which future protocols can be compared. All treatment modifications aimed at decreasing costs must be analyzed to ensure quality of care is not unduly compromised.

Endorectal pull-through abates gastrointestinal hemorrhage from colorectal venous malformations.

BACKGROUND/PURPOSE: Lower intestinal venous malformations are rare anomalies resulting from errors in vascular morphogenesis. These lesions may cause significant chronic and acute gastrointestinal hemorrhage. Venous malformations are unresponsive to angiogenesis inhibitors. Although these anomalies generally are incompletely resectable because of diffuse pelvic and mesenteric involvement, the authors sought to abate bleeding by excluding the lesion from the gastrointestinal lumen. METHODS: Three patients with circumferential transmural venous malformations of the colorectum, pelvis, and mesentery were identified. Imaging findings were similar among the patients and included circumferential septated bright signal on T2-weighted magnetic resonance imaging (MRI) contrast enhancement, and multiple phleboliths, seen best on computed tomography (CT). The lesion extended from the anus to the splenic flexure in 2 patients and throughout the entire colorectum in the other. Each had daily hematochezia for many years and required transfusions and chronic iron therapy. Although bleeding began in childhood in each patient, no therapy was successful until ages 7, 24, and 45. Colectomy, anorectal mucosectomy (through the pelvic venous malformation), and endorectal pull-through and anastomosis was performed (coloanal in 2 and ileoanal in 1). RESULTS: Bleeding essentially has been eradicated in all 3 patients with 10- to 57-month follow-up. One patient received a 3-unit transfusion intraoperatively, and the other 2 received none. The most recent patient to undergo surgery, who has residual venous malformation in the remaining 1 cm of anal mucosa, has some mild difficulty with fecal control if her diet results in loose stool. CONCLUSION: Colectomy with mucosectomy and endorectal pull-through should be considered for diffuse venous malformations of the colorectum before the development of large transfusion requirements.

Microlaparoscopy with conscious sedation in adolescents with suspected pelvic inflammatory disease.

STUDY OBJECTIVE: To develop a protocol for emergency department microlaparoscopy with conscious sedation in adolescents with clinically suspected pelvic inflammatory disease (PID), and to evaluate the feasibility and tolerability of microlaparoscopy in this population. DESIGN: Prospective study involving adolescents and young adults age 13 to 24 meeting clinical criteria for uncomplicated PID. Laparoscopy subjects underwent microlaparoscopy in the Children's Hospital Emergency Department (ED) and comparison subjects were admitted for treatment of PID. Chi-square, Mann-Whitney U tests, Wilcoxon Rank Sum tests, and repeated measures of analysis of variance (MANOVA) were used for analysis. RESULTS: Twenty-four patients were enrolled: 6 laparoscopy subjects and 18 comparison subjects. Laparoscopy and comparison subjects did not differ with respect to age, mean white blood cell (WBC) count, mean temperature, or mean erythrocyte sedimentation rate. Mean surgical induction time was 13.5 minutes, operative time 19.0 minutes, and total procedure time 32.5 minutes. Mean requirement for midazolam was 2.8 mg and for fentanyl 225 microg. Pain assessment over the first 90 minutes did not differ significantly between laparoscopy and comparison subjects. Four of 6 laparoscopy subjects (67%) and 10 of 18 comparison subjects (56%) were diagnosed with PID (p = NS). CONCLUSIONS: ED microlaparoscopy appears to be feasible, safe, and well tolerated in this small sample of adolescents and young adults with suspected PID.

Prolonged local myometrial blockade prevents preterm labor after fetal surgery in a leporine model.

BACKGROUND/PURPOSE: Postoperative premature labor remains the foremost limiting factor to the development of fetal surgery. Most attempts at controlling this complication have involved the use of drugs delivered systemically to the mother. This study assessed the effects of prolonged local anesthetic blockade of the myometrium on preterm delivery after open fetal surgery. METHODS: Eighteen New Zealand rabbits at 23 days' gestation (term, 31 to 33 days) were divided in three groups. In group I (n = 6), the most proximal fetuses of both uterine horns were submitted to open amputation of a forelimb; in a few animals, one of the uterine horns was empty, hence, only one fetus was manipulated. In groups II (n = 5) and III (n = 7), an identical surgical procedure was performed. In group II, immediately before hysterotomy, the myometrium was injected with 0.5 mL of 0.5% bupivacaine along the incision line. In group III, only saline was injected. In group II, before uterine closure, the incised area of the myometrium was injected with 1.5 mL of a novel suspension of biodegradable polylactic-co-glycolic acid microspheres loaded with 75% w/w bupivacaine and 0.05% w/w dexamethasone. This suspension previously has been shown to provide peripheral nerve blockade for approximately 5 days. In group III, microspheres without any drug were injected. RESULTS: Abortion rates were significantly different among the groups: 83.3% (five of six) for the does in group I, zero in group II, and 71.4% (five of seven) in group III (P < .05). The absence of abortions observed in group II occurred despite the fact that the fetal mortality rate was significantly higher in this group (87.5%, seven of eight fetuses) than in groups I (0) and III (33.3%, 4 of 12 fetuses, P < .05). CONCLUSIONS: Prolonged local blockade of the myometrium with bupivacaine inhibits preterm labor after fetal surgery in rabbits. The high fetal mortality rate observed in this study may be caused by "transplacental" transfer of the local anesthetic to the fetus. Notably, the abortifacient effect of a dead fetus was completely suppressed by the local blockade. Studies using microspheres with local anesthetics that do not cross the placenta, in animal models with longer gestational periods, are warranted.