Congenital intrahepatic portosystemic shunt—An incidental rare anomaly

Congenital intrahepatic shunts are rare anomalies that are usually incidental in a child who undergoes an ultrasound examination for some other reasons. Early diagnosis is important, because the condition can lead to hepatic encephalopathy and hypoglycemia. Author’s would like to describe the findings of one such patient diagnosed to be having congenital intrahepatic shunt and discuss the clinical importance of this condition.     

Congenital lobar emphysema and sequestration—treatment by embolization

A case of congenital lobar emphysema associated with pulmonary sequestration, presenting with respiratory distress in infancy, is reported. The lobar hyperinflation was managed by emergency lobectomy. The ipsilateral lower lobe affected with vascular sequestration was salvaged by therapeutic embolization, thus avoiding the long-term sequelae of pneumonectomy. The case is reported for its rarity.     

Hemoglobin J—As a Cause of Congenital Hemolytic Anemia

Hemoglobin-J is a rare hemoglobin variant known to be clinically silent most of the times, only to be detected accidentally. Herein, the authors report a case of Hemoglobin-J manifesting as unstable hemoglobin detected during evaluation of hemolytic anemia in an 8 month-old-infant. Cation Exchange-High Performance Liquid Chromatography(CE-HPLC) was used to identify this variant after Hb electrophoresis was reported to be normal.     

Congenital microgastria —management with a Hunt-Lawrence jejunal reservoir pouch

A 6-month-old male presented with inability to tolerate feeds, failure to thrive, vomiting, and reccurent aspiration. He was diagnosed as having congenital microgastria. He was fed for the first 22 months of life via a jejunal feeding tube that frequently fell out; he also required multiple hospital admissions for asthma and reccurent respiratory infections. A Hunt-Lawrence pouch was anastomosed to his microstomach and 17 months later he has a normal appetite, feeds normally, and no longer has any vomiting or respiratory infections. The jejunal pouch has increased the capacity of his upper gastrointestinal tract and diminished his symptoms of gastroesophageal reflux.     

Congenital malformations at birth — A prospective study from south India

Congenital malformations were studied prospectively from September 1989 to December 1992 covering 12,797 consecutive deliveries. The overall incidence of malformations was 3.7% and it was 3.2% among live births and 15.7% among still births. Three hundred and ninety seven birth defects were observed among 308 live births and 72 among 45 still births. The incidence of malformation was significantly higher among male babies (p <0.001), still births (p < 0.001), low birth weights (p < 0.001) and preterm babies (p < 0.001). Consanguinity among parents of malformed babies was more common (p < 0.001). Musculo-skeletal malformations were the commonest (9.69 per 1000) followed by cutaneous (6.33 per 1000), genitourinary (5.47 per. 1000), gastrointestinal (5.47 per 1000), central nervous system (3.99 per 1000) and cardiac anomalies (2.03 per 1000). Musculoskeletal, cutaneous and genitourinary malformations were common among live born babies while central nervous system and gastrointestinal defects were common among still born babies. Antenatal infections and ingestion of drugs were not found to be significant factors in the causation of birth defects.     

Infantile Fibrosarcoma—An Important Differential Diagnosis of Congenital Vascular Tumors

We present the case of a female newborn with life-threatening bleeding of a ruptured infantile fibrosarcoma (IFS) and consecutive multiorgan dysfunction syndrome shortly after birth. After stabilization, the tumor could be treated without amputation due to surgery, laser therapy, and chemotherapy. The patient is free of tumor and doing functionally well after 4 years of follow-up.     

Management of Congenital Diaphragmatic Hernia in Newborn — Paradigm Shift and Ethical Issues

Management of congenital diaphragmatic hernia (CDH) begins soon after it is detected, whether antenatally or postnatally. Assessment of the severity of the condition, associated congenital anomalies, maternal health and related issues, weight of the fetus/baby, mode of delivery, timing of delivery, immediate appropriate management of the baby with CDH at birth, appropriate utilization of available treatment modalities as well as infrastructure of the treating institute have an impact on the outcome of the neonate. Survival without significant long-term/permanent morbidity is considered as good outcome. With advances in antenatal diagnosis, several legal and ethical considerations have cropped up. While on one hand there are proponents of early antenatal diagnosis and medical termination of pregnancy (MTP), on the other hand there are several socio-cultural groups who look upon human life as precious and argue against MTP. There is an ongoing ethical battle between maternal vs. fetal rights; there is no way to put a lid on the controversy whether the mother be allowed to choose in favor of MTP after being aware of the anomalous fetus or, we must attempt to save every fetus irrespective of the antenatal diagnosis of life-threatening anomalies. Notwithstanding, appropriate assessment of the condition, thorough counseling and sound evidence-based decisions could avert ethical dilemma in most cases. This review article provides information about the various choices available in the diagnostic and treatment armamentarium, though it should be kept in mind that the entire spectrum of management strategies may not be universally available.     

Wilms’ Tumor—Lessons and Outcomes—A 25-Year Single Center UK Experience

Wilms’ tumor (WT) is a common childhood renal cancer. A 25-year single center UK experience is reported. During 1985–2010, 97 children underwent immediate nephrectomy or delayed resection of tumor after chemotherapy. Survival, morbidity, and late effects following treatment are described. Tumor distribution was: Stage I, 25.7% (n = 25); Stage II, 24.7% (n = 24); Stage III, 26.8% (n = 26); Stage IV, 17.5% (n = 17); and Stage V, 5.2% (n = 5). Immediate nephrectomy was performed in 39% (n = 38) patients with elective delayed resection in 61% (n = 59) cases. Ten patients had cavotomy to excise tumor involving vena cava territory. Two cases required cardiopulmonary bypass. Tumor rupture was recorded in eight (8.5%) total operated cases—after immediate (n = 5/37), 13.5% vs delayed nephrectomy—(n = 3/57), 5.2%; X ² P = .154. From 2001 onwards, one case of tumor rupture was recorded at this center after the universal adoption of UKW3 and SIOP guidelines advocating preoperative chemotherapy and delayed nephrectomy for all WT. Three treatment-related deaths occurred—hepatic veno-occlusive disease (n = 2) with actinomycin D and a single WT fatality due to vascular injury. Overall survival was 84.5% (82/97 cases). Two patients developed “late malignancies” —thyroid cancer and a basal cell carcinoma. This study demonstrates excellent survival for WT comparable with national outcomes and international cooperative studies. Adverse events with chemotherapy and surgery, including “late onset,” second malignancies deserve special consideration.     

Neonatal abstinence syndrome—postnatal ward versus neonatal unit management

Aim  

The aim of this cohort study was to test the hypothesis that caring for infants with neonatal abstinence syndrome (NAS) with their mothers on the postnatal ward rather than admit them to the neonatal unit would reduce treatment duration and length of hospital stay.     

Perineal hamartoma with accessory scrotum, anorectal anomaly, and hypospadias—a rare association with embryological significance: case report

A rare case of a newborn male with a perineal hamartoma, accessory scrotum, anorectal anomaly, hypospadias, and bifid scrotum is reported, with discussion of its embryological significance. Only three other cases with such a combination of anomalies have been reported in the English literature.     

Peripherally inserted central venous lines versus central lines in surgical newborns — A comparison

Objective  

To compare the insertion characteristics, utilization profile, life span and the complication rates of Central lines (CL) and Peripherally inserted central lines (PICL).