First report of surgery for anencephaly in twin pregnancies: Technical notes and postoperative management.

Anencephaly, the most severe form of neural tube defect, has no known cure, and in most cases, patients die before or shortly after birth. To date, no surgical intervention has been reported in the management of anencephaly. This study presents a case of dichorionic-diamniotic twin pregnancy in which 1 twin was anencephalic and describes the surgical management of this complex case. We aimed to share the problems experienced during the follow up of a patient who survived for a long time after surgery. We also aimed to highlight several clinical issues, including the challenges of managing anencephaly in twin pregnancies, problems experienced during the follow up process in our case, diagnosis of brain death in anencephaly cases, and ethical dilemmas related to organ donation. This case is notable because of the challenging nature of the surgical procedure and complexity of postoperative care. By highlighting the difficulties encountered during the follow up period, we hope to provide insights to health professionals that can inform the management of similar cases in the future.

The association of early postnatal weight loss with outcome in extremely low birth weight infants.

BACKGROUND: To compare outcomes of extremely low birth weight (ELBW) infants having different weight losses in the first 3 days of life. METHODS: One hundred and twenty six ELBW infants were evaluated retrospectively for weight loss percentages on the third day of life compared to their birth weight. We examined the weight loss on the third day of life compared to the birth weight for the ELBW infants and tested its association with mortality and morbidities. The mortality was subgrouped as overall mortality and mortality in the first 7 days of life. The morbidities were patent ductus arteriosus (PDA), intraventricular hemorrhage (IVH) and bronchopulmonary dysplasia (BPD). BPD was defined as need for supplemental oxygen at 36 weeks' postconceptional age. We grouped the infants into four quartiles according to weight loss percentage on the third day of life: Group 1 (Quartile 1), infants with weight loss of 0-3% of birth weight; Group 2 (Quartile 2); infants with weight loss of 3.1-7.5%, Group 3 (Quartile 3), infants with weight loss of 7.51-12%; and Group 4 (Quartile 4), infants with weight loss of more than 12%. The mortality and morbidities were analyzed according to these groups and other risk factors. RESULTS: Overall mortality and mortality in the first 7 days of life were significantly higher in Groups 1 (36% and 27%) and 4 (43% and 24%), compared to Groups 2 (10% and 10%) and 3 (18% and 9%), respectively. CONCLUSION: Weight loss less than 3% and more than 12% was significantly associated with an increase in mortality. There was a positive correlation between weight loss on the third day of life and IVH. CONCLUSION: Inappropriate weight loss in ELBW infants is associated with increased mortality and IVH. Appropriate weight loss can improve outcomes in this population.

Giant cell tumour of humerus: an unexpected lesion in a skeletally immature patient with Hodgkin's disease.

Giant cell tumour (GCT) of bone is a benign aggressive tumour of the bone. It typically presents in persons aged 20 - 40 years. It is rare in adolescents and children. In most cases, GCT of bone occurs in the metaphyseal and epiphyseal regions of long bones. However, in children with open physes, GCT of bone may be centered in the metaphysis and may abut the physis. GCT is most commonly found in the distal femur, proximal tibia, and distal radius. It is not reported after treatment of a cancer in childhood. To the best of authors' knowledge, the coexistence of giant cell tumour of the bone and Hodgkin disease has not been reported in the literature. We report an atypical case of giant cell tumour of humerus in a skeletally immature child treated for Hodgkin's disease (HD), which had initially presented as a bone cyst radiologically.

Neurodevelopmental outcomes of very low birth weight preterm infants treated with poractant alfa versus beractant for respiratory distress syndrome.

BACKGROUND: Some controlled trials have shown significant differences in short-term clinical outcomes between poractant alfa and beractant in infants with respiratory distress syndrome (RDS). There is, however, no study showing the differences in long-term outcomes with these treatments. AIM: To determine and compare the neurodevelopmental outcomes of preterm infants with RDS treated with poractant alfa or beractant at 2 years of age. METHODS: This was a prospective, longitudinal, single-center cohort study of infants born at ≤ 1,500 g and/or ≤ 32 weeks between 2008 and 2009 who received either poractant alfa (n = 113) or beractant (n = 102) for RDS. Neurological and developmental assessments were performed at a corrected age of 18 to 24 months. RESULTS: About 33 of 113 infants (29.2%) in the poractant alfa group had neurodevelopmental impairment compared with 36 of 102 (35.2%) in the beractant group, and the results did not differ between the groups (p = 0.339). Similarly, no significant difference was found in the percentage of infants with cerebral palsy (11.5 vs. 16.7%, respectively; p = 0.275). CONCLUSION: Our findings suggest that poractant alfa and beractant are similar in terms of neurodevelopmental outcomes when used for the treatment of RDS in preterm infants.

Postnatal risk factors associated with hearing loss among high-risk preterm infants: tertiary center results from Turkey.

The aim of this study was to determine the postnatal risk factors associated with hearing loss as well as the prevalence of hearing loss among high-risk preterm infants in newborn hearing screening (NHS). We performed a retrospective study of high-risk preterm infants born with a gestational age ≤32 weeks and/or a birth weight ≤1,500 g. A NHS procedure was performed by automated auditory brainstem response (AABR) and automated evoked otoacoustic emission (TEOAE). Infants who failed TEOAE or AABR or both tests were referred to a tertiary audiology center for diagnosis confirmation and management. Postnatal risk factors associated with hearing loss were evaluated and compared for preterm infants with and without hearing loss. 1,360 high-risk preterm infants were assessed. Permanent hearing loss was found in 19 (1.4%) infants. Multivariate analysis revealed that proven sepsis (p = 0.019), mechanical ventilation ≥5 days (p = 0.024), loop diuretics (p = 0.001), patent ductus arteriosus ligation (p = 0.018) and operation for retinopathy of prematurity (ROP) (p = 0.034) were significant related factors for the hearing loss. This study showed a low prevalence of hearing loss and an association between operation for ROP and hearing loss in preterm infants, which has not been defined previously. Our results suggest that every neonatal intensive care unit should determine their own risk factors and take precautions to prevent hearing loss for these high-risk preterm infants.

Congenital laryngeal cyst: a rare cause of polyhydramnios.

Congenital laryngeal cyst is a rare cause of airway obstruction that may require urgent diagnosis and treatment. We report a case of a neonate having history of polyhydramnios and severe respiratory distress at birth. A laryngeal cyst detected during intubation. The outcome of laryngoscopic treatment of the cyst was favorable.

A rare cause of hematemesis in newborn: fibrocystic breast disease of mother.

Hematemesis in a healthy newborn is most often caused by swallowed maternal blood. Maternal blood due to fibrocystic breast disease in human milk has not previously been reported in the literature. We report here a newborn case with hematemesis in which the mother had fibrocystic breast disease, and we want to emphasize this rare entity. Physicians should be aware of this rare condition, and fibrocystic breast disease of the mother should be included in the differential diagnosis of newborns with hematemesis.

Fetal cervical neuroblastoma: prenatal diagnosis.

Neuroblastoma is the most frequent extracranial solid tumor in childhood, but it is seldom diagnosed prenatally. It usually presented with adrenal masses. Presentation of other localization is extremely rare. We report a case of cervical neuroblastoma identified at 20 weeks of gestational age. This is the third case diagnosed antenatally on neck region in the literature. Additionally, it is the first case that extended to the brain. We also discussed the literature for cervical neuroblastoma detected prenatally.