Comparison of inguinal hernia and asymptomatic patent processus vaginalis in term and preterm infants.

INTRODUCTION: The aim of this study was to evaluate the characteristics of inguinal hernia (IH) and patent processus vaginalis (PPV) in term and preterm infants less than the age of 6months. METHOD: Between January 2004 and December 2012, 246 term and 165 preterm infants underwent laparoscopic herniorrhaphy within the first 6months of life. Preoperative clinical presentation and intraoperative anatomical findings during the laparoscopic procedure were evaluated. Additionally, initial side of hernia, laterality of IH and PPV were analyzed in term and preterm infants. RESULTS: In the group of term infants, most infants presented with a primary right-sided IH (58.5%) versus 17.9% left-sided and 23.6% bilateral IH. Babies with primary unilateral IH were found to have a contralateral PPV in 41.0% of cases. A difference between left-sided PPV and right-sided PPV could not be identified. In the group of preterm infants, initial bilateral presentation was predominant (38.8%) versus right-sided (30.3%) and left-sided IH (30.9%). Infants with primary unilateral IH were found to have a contralateral PPV in 56.4%. We identified a slight difference between left-sided PPV (54.0%) and right-sided PPV (58.8%). CONCLUSION: IH is predominantly right sided in term infants, whereas preterm infants mostly present with bilateral IH. The incidence of PPV was found to be significantly higher in the preterm group. Regarding the incidence of a contralateral PPV in term and preterm infants, no difference between initial left-sided and right-sided IH could be identified between both groups.

Neuroblastoma in monozygotic twins with distinct presentation pathology and outcome: is it familial or in utero metastasis.

To date ten sets of monozygotic twins with neuroblastoma have been reported in the literature. Twin-to-twin in utero metastasis have been proposed as the mechanism of tumor development in the second twin; based on similar pathology, presence of metastatic disease, absence of a primary tumor, and/or later presentation in the second twin. Hereditary neuroblastoma has not been described in this context. We propose that primary neuroblastoma can occur in monozygotic twins without twin-twin transmission; due to the different ages of presentation, histology, ploidy, and tumor behavior.

Extrahepatic vitelline vein aneurysm: prenatal diagnosis and follow up.

Umbilical vein varix is a well-described prenatal anomaly in which the prognosis remains unclear. We describe a very rare venous malformation that mimicked an umbilical vein varix consisting of a persistent vitelline vein. From 2003 to 2010, three patients were referred starting at 20 weeks gestation to our prenatal centers for an umbilical vein varix diagnosis. Fetal follow up was unremarkable, with the exception of the dilated vein size (mean: 35 mm at 33 weeks gestation). After birth, the three children presented with thrombosis from the aneurysmal sac to the portal trunk. All the children underwent surgical thrombectomy and resection of the aneurysmal sac after birth. Operative findings showed no umbilical vein but an abnormal dilated and thrombosed vein coming from the umbilicus to the portal vein following the right vitelline vein trajectory. One child was treated with systemic heparin. Median follow up is 5.6 years. Currently, one patient has a normal portal flow. The other two have persistent portal vein thrombosis with portal cavernoma and portal hypertension. This malformation is rare and should be considered in cases of early diagnosed umbilical vein varix whose diameter is greater than 20mm. We advocate an early surgical thrombectomy with heparinization to prevent portal vein thrombosis.

Ex utero intrapartum treatment in the management of giant cervical teratomas.

PURPOSE: The purpose of this study is to present the outcome and technical details of the Ex Utero Intrapartum Treatment (EXIT) procedure performed in the management of the fetus with a giant cervical teratoma. METHODS: A retrospective review of the medical records of patients undergoing the EXIT procedure between September 1995 and September 2010 was performed. RESULTS: Eighty-seven EXIT procedures were performed. In 20% of cases (17/87), the indication was giant cervical teratoma. There were 10 females and 7 males. Polyhydramnios was present in 82%. Median gestational age at EXIT was 35 weeks (range, 30-39 weeks). Median birth weight was 2.5 kg (range, 1.7-3.7 kg). Access to the airway under placental support was established in all cases via direct laryngoscopy/bronchoscopy in 8 patients (47%) and via surgical exploration (tracheostomy or retrograde intubation) in 9 patients (53%). The mortality rate under placental support was zero. Seven patients had the tumors resected immediately after the EXIT, 6 patients had the resection later, and 4 patients died before resection. The neonatal mortality rate was 23% (4/17 patients). Patients who died had severe pulmonary hypoplasia that resulted from the upward traction by the giant cervical mass on the airway and compression of the lungs against the thoracic apex. CONCLUSIONS: We conclude that the EXIT procedure continues to be the optimal delivery strategy for patients with prenatally diagnosed giant cervical teratomas and potential airway obstruction at birth. A thorough evaluation of the prenatal images and an experienced multidisciplinary team are key factors for an effective approach to the obstructed fetal airway.

[Intracerebral neurenteric cysts in newborn infants]. / Quistes neuroentericos intracerebrales en neonatos.

INTRODUCTION: Intracranial neurenteric cysts are very infrequent congenital lesions. Within the nervous system, they are most commonly located in the rachis. Another frequent site is the craniocervical junction. Few cases of supratentorial location have been reported. A differential diagnosis is required to distinguish them from other cysts. They are not often diagnosed in the paediatric age. The clinical features they give rise to are due to the mass effect or episodes of chemical meningitis. Magnetic resonance imaging is the best diagnostic method although on many occasions they cannot be distinguished from arachnoid cysts. Treatment consists in surgery with complete resection of the membranes in order to prevent recurrences. CASE REPORTS: We report two cases of infants who, in the last weeks of gestation, presented supratentorial intracerebral cystic lesions that resembled arachnoid cysts. A neonatal magnetic resonance scan confirmed the existence of such supratentorial cysts with septae inside them. After presenting an increase in the cranial perimeter, surgical treatment was undertaken with a neuronavigation-guided craniotomy and debridement of the cysts. The cysts contain mucus and have thick membranes. Pathological study results are consistent with a neurenteric cyst. One of the patients presented complete resolution after the intervention with good cerebral expansion. In the second case, there was persistence of an adjoining arachnoid cyst, in which placement of a cyst-peritoneal shunt was necessary with full resolution. CONCLUSIONS: Most intracranial cysts are arachnoid cysts, but there are other cystic lesions that must be treated by surgical means so that they can be completely excised and sent for pathological analyses.

Primary anastomosis for meconium peritonitis: first choice of treatment.

PURPOSE: Newborn surgery for meconium peritonitis (MP) is sometimes very difficult owing to severe adhesions and bleeding. The aim of this study was to reveal the benefit of primary anastomosis (PA) for MP by comparing PA with multistep operations (MO). PATIENTS AND METHODS: We retrospectively reviewed 38 patients with MP who underwent surgery in our institution from 1983 to 2009. From 1983 to 2000, we essentially used MO. After 2001, we used PA with the exception of 1 patient. We performed MO on 20 patients (group A) and PA on 18 patients (group B). RESULTS: Mortality was 4 in 20 in group A and 1 in 18 in group B. Three patients in group A and 2 in group B required reoperation because of complications. After 2001, 14 of 16 patients underwent PA. Of the 2 patients for whom PA could not be performed, one was postresuscitation from cardiopulmonary arrest and the other was an extremely low-birth-weight infant. The only mortality among the patients who underwent PA occurred in a very low-birth-weight infant who died from intraoperative hepatic hemorrhage. CONCLUSION: PA can be performed for almost all patients with MP except for extremely low-birth-weight infants.

Synchronous ipsilateral Bochdalek and Morgagni diaphragmatic hernias: a case report.

The etiology of congenital diaphragmatic hernia (CDH) is unknown. Phenotypic patterns of CDH defects provide clues about normal diaphragm development and the pathophysiology of CDH. We report a case of a patient who was diagnosed with CDH postnatally and was found on imaging to have simultaneous Bochdalek and Morgagni hernias on the right side. During the operative repair of these defects, an additional left-sided Morgagni-type defect was also found. To the best of our knowledge, this form of CDH has not been previously reported.

Placental anomalies in children with infantile hemangioma.

Evaluation of the placenta provides some important insights into pathophysiologic changes that take place during the prenatal and intrapartum process. We investigated the pathogenic significance of placental features and their relationship to the development of infantile hemangioma in order to obtain a better understanding of its cause. Placental specimens were reviewed from 26 singleton pregnancies of women whose offspring weighed less than 1500 g. A group of 13 neonates who developed infantile hemangioma in the immediate neonate period were compared with 13 healthy preterm infants of comparable postconception age who had no infantile hemangioma. Pathologic placental changes were analyzed in both groups. Gross lesions with disturbance of the utero-placental circulation were found in all placentas from children who developed infantile hemangioma, including massive retroplacental hematoma in two infants, extensive ischemic infarction in seven, and large dilatated vascular communications, severe vasculitis, chorioamnionitis and funiculitis in four. Placental features included percentages greater than 25% of avascular villi, platelet and fibrin aggregates, and multifocal disease involving more than one histologic section. Examination of 13 placentas of low-birth-weight infants without infantile hemangioma only showed abnormal placentation in one and isolated villous dismaturity in two. The higher ratio of placental pathologic findings in patients with infantile hemangioma suggests that reduced placental oxygen diffusive conductance contributes to fetal hypoxic stress and that hypoxic/ischemic changes in the placenta could be related to infantile hemangioma development via vascular endothelial growth factor and placental growth factor expression, among others, within the villious vessels and throphoblasts.

Are routine cranial ultrasounds necessary in premature infants greater than 30 weeks gestation?

The purpose of this study was to validate the recommendation of the American Academy of Neurology and the Child Neurology Society that screening cranial ultrasonography be performed routinely on all infants of less than 30 weeks gestation at 7 to 14 days of age and again between 36 and 40 weeks postmenstrual age, and, by using this practice parameter, to determine the number of babies with a clinically significant abnormal screening cranial ultrasound (US) who would otherwise have been missed. A retrospective study of 486 infants of 30 to 33 weeks gestation born January 1, 1999 to June 30, 2004 was done. All had screening cranial ultrasounds. Grade III and/or grade IV intraventricular hemorrhage (IVH) occurred in 4 (0.8%) infants of 30 to 31 weeks gestation. Infants with significant IVH had either risk factors for brain injury or symptoms that would eventually warrant US during their hospitalization. Seven (1.4%) infants had periventricular leukomalacia (PVL). All infants with a final diagnosis of PVL had pre- and/or perinatal risk factors associated with PVL. There was a significant trend toward fewer abnormal cranial ultrasounds from 30 to 33 weeks gestation (p=0.04). Our study supports the recommendation by the American Academy of Neurology and the Child Neurology Society that screening US can be limited but suggests that the gestational age cut off should be 30 weeks or less.

Magnetic resonance imaging assessment of brain maturation in preterm neonates with punctate white matter lesions.

INTRODUCTION: Early white matter (WM) injury affects brain maturation in preterm infants as revealed by diffusion tensor imaging and volumetric magnetic resonance (MR) imaging at term postmenstrual age (PMA). The aim of the study was to assess quantitatively brain maturation in preterm infants with and without milder forms of WM damage (punctate WM lesions, PWML) using conventional MRI. METHODS: Brain development was quantitatively assessed using a previously validated scoring system (total maturation score, TMS) which utilizes four parameters (progressive myelination and cortical infolding, progressive involution of glial cell migration bands and germinal matrix tissue). PWML were defined as foci of increased signal on T1-weighted images and decreased signal on T2-weighted images with no evidence of cystic degeneration. A group of 22 preterm infants with PWML at term PMA (PWML group) were compared with 22 matched controls with a normal MR appearance. RESULTS: The two groups were comparable concerning gestational age, birth weight and PMA. TMS was significantly lower in the PWML group than in the control group (mean TMS 12.44 +/- 2.31 vs 14.00 +/- 1.44; P = 0.011). Myelination (mean 2.76 +/- 0.42 PWML group vs 3.32 +/- 0.55 control group, P = 0.003) and cortical folding (3.64 +/- 0.79 vs 4.09 +/- 0.43, P = 0.027) appeared to be significantly delayed in babies with PWML. CONCLUSION: Conventional MRI appears able to quantify morphological changes in brain maturation of preterm babies with PWML; delayed myelination and reduced cortical infolding seem to be the most significant aspects.

The morphological analysis of the haematopoietic microenvironment of the bone marrow at foetuses from preterm pregnancies.

The aim of the study was morphological assessment of the haematopoietic microenvironment of the bone marrow in foetuses from preterm pregnancies. For the morphological assessment the bone marrow was taken from sternum during an autopsy examination. After standard preparation, cellular elements of individual developmental lines were identified with the application of immunohistochemical methods. Evaluation of the bone marrow stroma elements was performed on the basis of configuration of the vascular sinuses, connective tissue elements, mutual topographic relations and integrin occurrence. The assessment of the argentophilic fibres was carried out with impregnation with silver according to the Gomori method. The configuration of the vascular sinuses was defined with morphometric evaluation with MicroImage-Olympus Kit. Identification of the 'young vascular forms' was performed with immunohistochemical methods with the use of endothelial cell antibodies and factor VIII. The presence of integrin was discovered in individual megakaryocytes, endothelial cells of sinus vessels and singular cells forming a stroma. However, no differences were found in the quantity of argentophilic fibres. The study revealed that ontogenetic development of haematopoietic tissue not only consists in alterations connected with the maturing process and cellular differentiation, but in topographic changes as well.