Maria Di Lorenzo

Complications of lymphangiomas in children.

One hundred ninety-three cases of lymphangioma presenting in children were reviewed over a 10-year period. The diagnosis was established before 5 years of age in 87.0%. The distribution of lymphangiomas was: cervical (31.4%), craniofacial (18.9%), extremity (18.9%), trunk (9.2%), intraabdominal (9.2%), cervicoaxillothoracic (4.9%), multiple (3.8%), cervicomediastinal (2.2%), and intrathoracic (1.6%). Of 164 patients undergoing primary therapy, total excision was performed in 77.4% and partial excision in 20.7% with recurrence rates of 11.8% and 52.9%, respectively. Thirty-eight percent of all operations for lymphangiomas were performed for resection of recurrent or residual disease. The incidence of postoperative complications was 31.3%. Forty-four additional procedures were required to manage these complications. Sclerotherapy with 50% dextrose was used as an adjunct in 9.5%. Recurrences were seen after total resection in 17.6% of operations in which 50% dextrose was used and 11.8% in which it was not used. Seroma formation was noted in 3.5% of total resections and 40.0% of resections for recurrent disease in which adjunctive sclerotherapy with 50% dextrose was used. Seromas occurred in 9.8% of wounds in which local drains were used and 3.6% of wounds in which drains were not used. Infectious complications occurred after 6.6% of operations using perioperative antibiotics and 3.5% not using antibiotics. The management of lymphangiomas in children remains a difficult problem. Despite being benign lesions, lymphangiomas are attended by a high rate of morbidity from complications of the disease and its management. Complete excision still offers the best chance for complete cure. Sclerotherapy with 50% dextrose is not beneficial in the management of recurrent disease or postoperative seromas. Local drains and perioperative antibiotics do not appear to diminish the incidence of seromas and infectious complications, respectively.

Childhood primary pulmonary neoplasms

Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.

Blunt Pancreatic Injuries in Children: The Role of Percutaneous External Drainage in the Treatment of Pancreatic Pseudocysts

During the past 10 years, 26 cases of blunt pancreatic trauma were diagnosed in our institution. In 42.3% (11/26) the accident was bicycle-related. Seventy-three percent of patients were seen within 48 hours of injury. The most frequent clinical presentations included abdominal pain, tenderness and vomiting. Diagnosis of pancreatic injury was suggested by hypermylasemia in most cases. Associated trauma was seen in seven patients (26.9%) and it was intraabdominal in four (15.3%). Computerized tomography (CT) scan is the single most useful radiologic investigation in evaluating pancreatic trauma. Ultrasound, although less accurate than CT scan in determining the severity of the initial injury, is useful in the evaluation and treatment of pancreatic pseudocysts. Pancreatic pseudocysts developed in ten patients. Spontaneous resolution occurred in five (50%). In three patients, percutaneous external drainage (PED) was successful in treating pancreatic pseudocysts without complications or recurrence at 11, 19, and 31 months. PED is a suitable form of treatment in selected cases of pancreatic pseudocysts. Results in children are better than in the adult population, probably due to the absence of primary pancreatic pathology. We believe that PED should be considered the primary therapeutic procedure for traumatic pancreatic collections prior to more invasive surgical treatment, when there is no evidence of pancreatic duct transection on CT scan.

A twenty-year experience with thyroid carcinoma in children

During the past 20 years, 23 patients (7 males, 16 females) were operated on for thyroid carcinoma in our institution. The average age was 13.6 years (range, 22 months to 27 years). Our series includes papillary carcinoma in 11, follicular carcinoma in four, and medullary thyroid carcinoma in eight patients. Follow-up ranged from 8 months to 20.3 years, with an average of 7.5 years for well-differentiated carcinomas and 4.3 years for medullary thyroid carcinomas. All patients are presently alive with no evidence of progressive disease. Patients with papillary and follicular carcinomas underwent partial thyroidectomy; those with medullary carcinoma underwent total thyroidectomy. Serious complications included three permanent hypoparathyroidism and two tracheostomies, all after secondary neck explorations. The overall results observed in our series of patients seem to support the current conservative approach to well-differentiated thyroid carcinoma, reserving total thyroidectomy for medullary cancer of the thyroid. A more aggressive search for familial medullary carcinoma through use of pentagastrin stimulation leads to early detection and more effective therapy.

Sirenomelia in an identical twin: A case report

Sirenomelia, or the mermaid syndrome, is the most extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, anal imperforation, and renal agenesis or dysgenesis. Because of the resultant oligohydramnios, these infants most often have Potters facies and pulmonary hypoplasia. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. It has been suggested that the association of the most extreme form of caudal regression, sirenomelia, with monozygotic twinning may represent developmental arrest of the primitive streak, with creation of a second primitive streak that gives rise to the usually normal twin. The embryology of the various presentations of the caudal regression syndrome may be further delineated by studying infants with this dramatic and fatal syndrome.

Malignant small-cell tumor of the thoracopulmonary region: ‘Askin tumor’

Malignant small-cell tumor of the thoracopulmonary region (MSCT) is an uncommon neoplasm in children. We describe five cases diagnosed since 1981 that fulfill the criteria put forth by Askin et al. Surgery was performed for diagnosis or therapy in all patients. Two patients underwent open lung biopsy, only because of tumor extent. The other three had chest wall resections. All patients received radiotherapy and chemotherapy. Three patients presenting initially with extensive disease died at intervals of 2.5 to 7 months after diagnosis. Two patients are alive and disease-free at 16 and 24 months postdiagnosis. All five cases were reviewed for standard histology and differential immunohistochemistry. Electronmicroscopy and tissue cultures were done in 3 of the 5 patients. All five patients were neuron-specific enolase-positive. MSCT is an exclusion diagnosis not always readily made. Clinical course and pathologic expertise may point to the correct diagnosis.

Management of an infant with diffuse bullous pulmonary lesions using high-frequency oscillatory ventilation.

PurposeTo describe the anesthetic and ventilatory management of an infant with diffuse pulmonary bullous lesions.Clinical FeaturesFour successive operations were scheduled for an infant with diffuse pulmonary bullous lesions. At the age of seven weeks, conventional positive pressure ventilation during laparotomy for intestinal occlusion led to arterial desaturation. This was corrected by returning to spontaneous respiration and deep inhalation anesthesia with halothane. Based on our ICU experience and due to a potential impaired oxygenation during conventional ventilation, we chose high-frequency oscillatory ventilation (HFOV) for bilateral sequential thoracotomies for bullectomies at the age of five months. We elected the same ventilatory mode for laparotomy for intestinal obstruction secondary to a polyp at the age of six months. This ventilatory mode was combined with total intravenous anesthesia and epidural analgesia and provided optimal oxygenation and ventilation as well as vital signs stability.ConclusionHigh frequency oscillatory ventilation is a safe technique that may be used in the operating room in cases where conventional ventilation failed to provide satisfactory gas exchange.RésuméObjectifDécrire la technique d’anesthésie et de ventilation chez un enfant souffrant de lésions bulleuses pulmonaires bilatérales.Aspects cliniquesUn enfant porteur de lésions pulmonaires bulleuses diffuses a dû subir quatre opérations successives. À l’âge de sept semaines, la ventilation habituelle en pression positive utilisée durant une laparotomie pour occlusion intestinale a abouti à une désaturation artérielle. Le traitement a consisté en l’utilisation d’une technique d’anesthésie par inhalation d’halothane en respiration spontanée. En nous basant sur notre expérience aux soins intensifs et sur le fait que la ventilation traditionnelle pourrait provoquer une hypoxie, nous avons choisi d’utiliser la ventilation à haute fréquence par oscillations pour deux thoracotomies séquentielles pour bullectomies à l’âge de cinq mois. Nous avons choisi la même technique de ventilation pour une nouvelle laparotomie pour occlusion intestinale sur un polype à l’âge de six mois. Ce mode ventilatoire associé à une anesthésie intraveineuse totale et à une analgésie épidurale a permis d’assurer l’oxygénation et la ventilation du patient tout en maintenant la stabilité des paramètres vitaux.ConclusionLa ventilation à haute fréquence par oscillations est une technique sécuritaire et utilisable en salle d’opération lorsque la ventilation classique ne permet pas d’assurer des échanges gazeux satisfaisants.

Multiple small bowel perforations in leukemia secondary to epstein-barr virus lymphoma with survival: A case report

This is the case report of a 4-year-old white boy who was diagnosed as having acute lymphoblastic leukemia (ALL) in November 1985. While in remission and on maintenance chemotherapy, he developed a primary Epstein-Barr virus (EBV) respiratory infection in October 1986. On October 27, 1986 a plain abdominal radiograph taken for abdominal distention showed free air. At celiotomy, multiple nodules were noted to stud the small bowel. Central necrosis of these nodules with perforations were present in the distal small bowel. Resections and end-to-end anastomoses were performed. Three days later the patient again had a similar acute abdominal episode. At reexploration, similar lesions in the liver, kidney, duodenum, proximal jejunum, and colon were found. Liver biopsy as well as intestinal resections and end-to-end anastomoses were performed, along with a loop ileostomy. Polymorphic B-cell lymphoma positive for EBV was found in the specimens. After cessation of chemotherapy and institution of abdominal radiotherapy, the hepatic and renal lesions were seen to resolve on computed tomography scan. The patients course was complicated by the development of cervical and mediastinal abscesses that were drained, and E coli sepsis accompanied by chronic diarrhea requiring intravenous hyperalimentation. By January 1988, he appeared to be recovering. His ileostomy was closed in March 1988. Despite cessation of chemotherapy since October 1986, the patient is now well and in complete remission.

Performance of a Simplified Wall Motion Score Index Method for Noncardiologists to Assess Left Ventricular Ejection Fraction

Backgrounds. For emergency physicians performing a goal-directed transthoracic echocardiogram (TTE), a reliable estimate of LVEF must be obtained rapidly. We compared rapid LVEF estimates obtained from short axis sections to those obtained from apical sections using two methods of evaluation. Methods. The TTEs of 6 patients were interpreted by 16 echo-proficient readers (PRO group) and 105 novice readers (NOV group). LVEF was assessed by each group. The strategies consisted of either a global visual estimation (VIS) of LVEF or semiquantitative (SQ) methods. Results. Using RNV and BIP as a reference standard, NOV readers performed better with the SQ method than global visual estimation. For NOV readers, best agreement was achieved with the 234C sequence in low LVEF situations, but with the BMA series in normal LVEF settings. Neither series of views was better than the other in the setting of mild LVEF depression. Conclusion. Semi-quantitative method was superior to global visual estimation of LVEF in NOV group in most of the LVEF ranges.

Assessment of LVEF using a new 16-segment wall motion score in echocardiography

Background Simpson biplane method and 3D by transthoracic echocardiography (TTE), radionuclide angiography (RNA) and cardiac magnetic resonance imaging (CMR) are the most accepted techniques for left ventricular ejection fraction (LVEF) assessment. Wall motion score index (WMSI) by TTE is an accepted complement. However, the conversion from WMSI to LVEF is obtained through a regression equation, which may limit its use. In this retrospective study, we aimed to validate a new method to derive LVEF from the wall motion score in 95 patients. Methods The new score consisted of attributing a segmental EF to each LV segment based on the wall motion score and averaging all 16 segmental EF into a global LVEF. This segmental EF score was calculated on TTE in 95 patients, and RNA was used as the reference LVEF method. LVEF using the new segmental EF 15-40-65 score on TTE was compared to the reference methods using linear regression and Bland–Altman analyses. Results The median LVEF was 45% (interquartile range 32–53%; range from 15 to 65%). Our new segmental EF 15-40-65 score derived on TTE correlated strongly with RNA-LVEF (r = 0.97). Overall, the new score resulted in good agreement of LVEF compared to RNA (mean bias 0.61%). The standard deviations (s.d.s) of the distributions of inter-method difference for the comparison of the new score with RNA were 6.2%, indicating good precision. Conclusion LVEF assessment using segmental EF derived from the wall motion score applied to each of the 16 LV segments has excellent correlation and agreement with a reference method.BACKGROUND Simpson Biplane method and 3D by transthoracic echocardiography (TTE), radionuclide angiography (RNA), and cardiac magnetic resonance imaging (CMR) are the most accepted techniques for left ventricular ejection fraction (LVEF) assessment. Wall motion score index (WMSI) by TTE is an accepted complement. However, the conversion from WMSI to LVEF is obtained through a regression equation which may limit its use. In this retrospective study, we aimed to validate a new method to derive LVEF from the wall motion score in 95 patients. METHODS The new score consisted of attributing a segmental EF to each LV segment based on the wall motion score and averaging all 16 segmental EF into a global LVEF. This segmental EF score was calculated on TTE in 95 patients and RNA was used as the reference method. LVEF using the new segmental EF 15-40-65 score on TTE was compared to the reference methods using linear regression and Bland-Altman analyses. RESULTS The median LVEF was 45% (interquartile range 32-53%; range from 15 to 65%). Our new segmental EF 15-40-65 score derived on TTE correlated strongly with RNA-LVEF (r=0.97). Overall, the new score resulted in good agreement of LVEF compared to RNA (mean bias 0.61%). The standard deviations (SDs) of the distributions of inter-method difference for the comparison of the new score with RNA was 6.2%, indicating good precision. CONCLUSION LVEF assessment using segmental EF derived from the wall motion score applied to each of the 16 LV segments has excellent correlation and agreement with a reference method.