Intracranial neoplasms during the first year of life

During a 7-year period, from January 1976 to December 1982, 470 brain tumors were treated at the Hospital de Ni?os Ricardo Gutierrez in Buenos Aires, 40 of which occurred in infants under 1 years of age. Diagnosis was at best tentative because most cases were referred on account of symptoms and nonspecific signs, e.g., macrocephaly, vomiting, and altered behavior. The supratentorial location was predominant over the infratentorial, with a ratio of 4:1. Craniotomies with resection of tumor were performed on all but four infants. Optic chiasm pilocytic astrocytoma, superficial brain astrocytoma, and choroid plexus papilloma were the most common histologic types. Thirty-three infants (80%) were found to have hydrocephalus complicating the brain tumor, but only 15 of them were shunted, because 4 infants died, and in 14, hydrocephalus remitted after the operation. After 2-9 years of postoperative observation, 16 infants (40%) live tumor-free and have normal neurological examinations, 5 (12%) suffer moderate deficits, and 19 (48%) have died.     

Intracranial tumours in the first year of life. A report on 51 cases

Summary The authors report on 51 infants with intracranial tumours treated in an eleven-year period; these infants represent 13% of the total population of children with intracranial tumours who have been operated on in the same institution during the same period of time. Males (28 cases) were slightly more frequent. Astrocytomas (17 cases), medulloblastomas (12 cases), and ependymal tumours (5 cases) were the commonest histologic types.Signs and symptoms of increased intracranial pressure were by far the most frequent clinical manifestations, followed by seizure disorders. Thirty tumours were localized within the supratentorial, and 21 within the subtentorial compartment. The parasellar region (10 cases) and the lateral cerebral ventricles (8 cases) for the supratentorial tumours, the inferior cerebellar vermis and fourth ventricle (13 cases) for the infratentorial tumours appeared to be the preferred topographic locations.Craniotomies were carried out in 44 infants, with a total or radical removal of the tumour in 19 cases, a subtotal removal in 6 cases, and a partial removal in 17 cases. In 3 cases only a biopsy procedure was performed. Twenty-nine of these patients required an ancillary procedure such as CSF shunting. Three subjects underwent a biopsy procedure and 1 infant the insertion of a CSF shunting device only. Surgery was not performed in 5 cases.Overall, there were two surgical deaths. Two infants died before any surgical treatment could be performed. Radiation therapy was administered to 9 patients when they had reached three years of age. Chemotherapy was given to 21 infants, according to various Chemotherapic protocols.During the postoperative period 20 deaths (39%) were recorded. Two patients were lost to follow-up. From 1 to 10 years after the operation, 29 patients are still alive, 14 of them (28%) with a normal psychomotor development, 10 (20%) with some neurological or men tal deficits, and 5 (10%) with severe psychomotor retardation. There was no apparent correlation in this series between late outcomes and the histological type of the tumour.     

Intracranial neoplasms during the first year of life: analysis of one hundred consecutive cases

One hundred infants with intracranial tumors symptomatic during the 1st year of life were studied. They differed from older children in having a higher percentage of supratentorial tumors and in the fact that 90% of the tumors were of neuroectodermal origin. Vomiting, alteration of psychomotor development, and macrocrania were the most common presenting features. The "diencephalic syndrome" was seen in 5 infants, and subarachnoid hemorrhage due to tumor was diagnosed in 4. Computed tomography as the primary investigation is increasing the number of neoplasms diagnosed in this age group, although review of the skull roentgenograms in the series disclosed an abnormality in 92%. Eighty of the tumors were verified, 68 by a cranial operation and the rest at autopsy. Of the verified neoplasms, 20% were medulloblastomas, 12.5% were choroid plexus papillomas, and 10% were cerebellar astrocytomas. The cumulative average survival was 27 months but, for those who underwent a tumor operation, the average survival was 37 months. The operative mortality was 30%. Thirty-nine patients were irradiated, and this subset had a 5-year survival rate of 43%. The morbidity was high irrespective of radiotherapy; 60% of those who survived 1 year were moderately or severely disabled. Those infants receiving more than 5000 rads of whole brain radiation tended to have greater deficits in the long term. When analyzed separately, patients treated after 1970 had greatly improved mortality and morbidity rates.     

Urolithiasis in the first year of life

Data on urolithiasis (UL) in infancy are limited. The objective of this study was to increase awareness of infant UL and to investigate the influence of possible risk factors in this very specific age group. Nonfasting, second-voiding urine samples were obtained to test for urinary excretions of calcium, oxalate, citrate, magnesium, uric acid, and creatinine. Blood analysis included calcium, phosphate, magnesium, uric acid, creatinine, sodium, potassium, chloride, and alkaline phosphatase. Patients received follow-up testing every 1–2 months; serial ultrasonography was used to track UL status. Fifty infants with a median age of 5 months were enrolled in the study. Hypercalciuria was detected in 9/47, hyperoxaluria in 5/39, hypocitraturia in 4/31, and cystinuria in 2/50 infants. We identified at least one metabolic abnormality in 46% of our patients; no metabolic abnormality was identified in 27 infants. Within a mean follow-up period of 14 months, 17 infants became stone free, stones increased in number in ten patients and decreased in number in 16, and recurrence was detected in seven. This study showed that UL could be detected in very early life, even in the newborn period, and could be the source of late childhood/adulthood UL. Infants with nonspecific symptoms such as restlessness may have UL and should undergo ultrasonographic examination. Metabolic evaluation of UL in this specific age group carries some diagnostic challenges, e.g. unsatisfactory data regarding normal ranges of urinary mineral excretion, and collection of 24-h urine samples.     

Intracranial neoplasms in the first year of life: results of a second cohort of patients from a single institution

The objective of this study was to describe the presentation and outcome of children with intracranial tumours under 1 year of age, and to compare the results with a previous cohort from the same paediatric neurosurgical unit. It is a retrospective review of all children under 1 year of age presenting with intracranial tumours between 1982 and 1997, with follow-up data from a multidisciplinary paediatric neuro-oncology clinic. Seventy-five children were diagnosed during the period of study. Overall survival at 5 years was 56% (31 of 55 eligible children), half of whom are in mainstream education. Earlier diagnosis and a dramatic reduction in peri-operative mortality compared to our previous cohort account for the improvements in the results of treatment for these children whose care can only be properly managed in a specialized paediatric oncology centre.     

Mitral valve replacement in the first year of life

From 1973 through 1987 25 patients underwent mitral valve replacement in the first year of life for mitral stenosis and mitral regurgitation. The patients with mitral stenosis included two with mitral arcade, two with supravalvular mitral stenosis with hypoplastic mitral valve, and one with parachute mitral valve. Included in the group of patients with mitral regurgitation were 12 with atrioventricular canal defect, six with chordal and leaflet defects, one with Marfan's syndrome, and one with bacterial endocarditis. Prostheses included 12 Bj?rk-Shiley (17 mm), seven St. Jude Medical (19 mm in four, 21 mm in three), five stent-mounted dura mater valves (12 mm to 16 mm), and one porcine xenograft (19 mm). In four patients the valves were placed in the left atrium in a supraannular location. There were nine operative (atrioventricular canal defect seven, mitral regurgitation two) and five late (atrioventricular canal defect four, mitral stenosis one) deaths, giving actuarial 1- and 5-year survival rates of 52% and 43%, respectively. All 6 patients with tissue valves died; the four with supraannular mitral valve replacement survived. Since 1983 operative mortality has been reduced to 0% (70% confidence limits 0% to 24%). Nine patients required a second mitral valve replacement for prosthetic stenosis 5 to 69 (mean 30) months after the original mitral valve replacement (one operative death). Because of improvements in repair of atrioventricular canal defect in infancy, the need for mitral valve replacement at atrioventricular canal defect repair has decreased. Although valvuloplasty has been advocated for repair of congenital mitral valve disease and is applicable in some infants with mitral regurgitation, mitral valve replacement is frequently unavoidable for congenital mitral disease and can now be accomplished at a low operative risk, even when the prosthesis has to be positioned supraannularly.     

Cerebral cavernous angiomas in the first year of life

Two rare cases of cerebral cavernous angiomas in two infants, 9 and 6 months old, respectively, are reported and the other 11 cases in the literature concerning patients in the first year of life are reviewed. Cavernous angiomas of the brain occur rarely in the first year of life and present with seizures and head enlargement. On computed tomographic scan they typically appear as large, hyperdense, unenhanced masses, with large cysts and must be differentiated from tumors more common in infants, such as teratomas, ependymomas, and mixed tumors. Total removal is often possible, even with large lesions, because of the small amount of bleeding and the well-defined limits of the mass.     

Repair of aortic coarctation in the first year of life

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.     

Surgery of pelviureteric obstruction in the first year of life

Twenty-nine out of 108 patients (26%) were under 1 year of age at the time of surgery for congenital pelviureteric obstruction. The clinical features and diagnosis, management, follow-up and results in these patients are reviewed. Clinical presentation was usually with an abdominal mass or urinary infection but an increasing number of cases were diagnosed after maternal ultrasonography had shown hydronephrosis. Ultrasonography, together with renal nuclide scan, were considered to be the most appropriate imaging modalities to define anatomy, determine function and document obstruction. Pyeloplasty was carried out successfully in 28 patients (two bilateral) and one patient had a nephrectomy. Sixteen pyeloplasties were managed by a nephrostomy (with or without a stent), the tubes usually being removed 10-12 days postoperatively. Fourteen pyeloplasties were managed by a wound drain only but one required a secondary nephrostomy although eventual recovery was satisfactory. Renal nuclide scan was found to be the most appropriate follow-up test and the overall results of surgery were satisfactory.     

The spectrum of cholelithiasis in the first year of life

Cholelithiasis is being diagnosed with increased frequency in the very young. We encountered 12 patients over a 5-year period in whom cholelithiasis was diagnosed prior to age 1 year. The earliest diagnosis was made in utero during the seventh gestational month. While most of the children suffered from complex medical disorders, 4 of the 12 had no recognizable predisposing factors. Eight of the 12 were treated at some point prior to diagnosis with central parenteral nutrition. Three presented with infectious complications requiring prompt operative intervention. Two children underwent elective cholecystectomy nearly 2 years after the diagnosis for symptoms of biliary colic. Of the remaining seven, spontaneous stone resolution was documented in three, while four remain asymptomatic after a minimum of years follow-up. The spectrum of presentations in this series highlights the uncertainties regarding the etiology, natural history, and treatment of this increasingly more common disorder.     

Brain tumors during the first twenty-four months of life

One hundred patients with brain tumors were diagnosed and treated during the first 24 months of life. They represent 16% of 608 children with brain tumors treated from 1952 through 1984. The most common histological type of brain tumors during the first 24 months are benign astrocytoma, medulloblastoma, and choroid plexus papilloma. The tumor location is distributed relatively evenly among the cerebellum and the 4th ventricle, the cerebral hemisphere (including the lateral ventricle), and the suprasellar region and 3rd ventricle. The chief presenting signs and symptoms are a full fontanelle, macrocephaly, changes of behavior, and delayed developmental milestones. Localizing signs are infrequent. Ninety-two patients underwent craniotomy with a one-month surgical mortality rate of 12%. The surgical mortality rate was 5.6% among 36 recent patients diagnosed by computed tomography. The 5-year survival rate is 41% in the patients younger than 12 months and 74% in patients diagnosed during the 2nd year of life. This study indicates the validity of Collins' rule for medulloblastomas during the first 24 months of life, but not for benign or malignant astrocytomas.     

一岁以内婴儿法洛四联症纠治术

目的 总结婴儿法洛四联症(TOF)的纠治经验，以提高婴儿TOF纠治术的成功率。方法 1999年3月一2001年11月我科收治43例小于1岁TOF患者，术前均经二维超声心动图或心导管造影检查确诊，均在体外循环下行纠治手术，其中5例行急诊手术。结果 手术近期死亡1例(2．3％)，30例患者随访1—18个月，恢复良好，紫组消失，生长发育明显加快。结论 小于1岁婴儿行TOF纠治术是安全可靠的，对反复缺氧发作的TOF患者，强调早期手术；对缺氧发作呈持续状态的患者要考虑行急诊手术。改善手术及体外循环方法，加强术后监护是提高婴儿TOF纠治术成功的关键。     

Health care of preterm infants in the first year of life

The home environment of preterm infants and their use of health services after discharge from hospital were investigated. Of the mothers of preterm infants studied 49% were not educated beyond primary school level, 38% were unmarried and 20% of these families had marked social problems, and 56% lived in grossly overcrowded conditions. Only the extent of unmarried motherhood and marked social problems were greater in preterm infants than in a control group of term infants. Attendance at primary care services for illness was no different for the preterm and term groups, although hospital admissions were most frequent in the preterm infants. Three preterm infants died in the first year. Only 65% of preterm infants and 55% of controls had completed weight charts on their preschool record card at 12 months of age. Three preterm and 2 control infants had not been fully immunized. In spite of poor social circumstances, the use of health services by the mothers of preterm infants was most encouraging.     

Urgent laparoscopic surgeries in children of the first year of life

One hundred and ninety-two urgent laparoscopic operations in 179 infants (including 31 newborn infants) were performed. Indications for surgery were intestinal obstruction (108 children), acute diseases of abdominal organs (61), and gynecological diseases (10). The rate of conversion in 3 groups was 10.9, 4.6% and 0, respectively. In all the cases conversion was performed due to technical difficulties, there were no intrasurgical complications, diagnostic value of the method reached 100%. Combined laparoscopic surgeries were performed in 25.1% cases. Intestinal invagination was the most frequent cause of laparoscopy. Comparative analysis of open (28) and laparoscopic (85) desinvagination demonstrated the advantages of the latter one both during and after surgery. Laparoscopy is the useful method for diagnosis and treatment of acute abdominal disease requiring urgent surgery in infants.     

Postnatal development of renal function during the first year of life

Several aspects of renal function varyconsiderably during the 1st year of life and differ markedly from the equivalent values in the adult. Glomerular filtration rate (GFR) increases little, prior to the time an infant reaches a conceptional age of 34 weeks, the point in renal development from which the absolute GFR (ml/min) increases gradually to mature values when linear growth is completed during adolescence. GFR corrected for body size is not comparable with adult normal values until after 12 months of age; therefore, whether GFR is estimated from S_cr or measured by timed urine collection, there is no easily recalled range of normal values for infants. One must know the changes in the renal function of normal infants that take place following birth during the 1st year of life. Despite several attempts to do so, renal function during the 1st year of life cannot be assessed from urine flow rate. A urine flow rate of less than 1 ml/kg per hour may be normal and appropriate and may not be harmful either to preterm or full-term infants with normal GFR. Impaired concentrating ability of the neonatal kidney is probably of no clinical significance in all but the most extreme circumstances and is not a major factor in an infant becoming dehydrated, developing hypernatremia or being at greater risk of acute renal injury. Acid-base status in infants must be interpreted appropriately to know when alkali therapy should be introduced to avoid growth failure secondary to true metabolic acidosis. When plasma renin activity is measured in the infant with renal failure of hypertension, one must compare the result with the normal range of values related to postnatal age of normal infants.Presented at the annual meeting of the American Society of Pediatric Nephrology, 5 May 1986, organized by Dr. R. N. Fine, Los Angeles.