Alobar Holoprosencephaly Associated with Meningomyelocoele and Omphalocoele: An Unusual Coexistence.

Holoprosencephaly is a rare congenital disorder which results from failure of cleavage or incomplete differentiation of the forebrain structures at various levels or to various degrees. Depending on the degree of involvement, it is classified into 4 types: Alobar, Semilobar, Lobar and Middle interhemispheric fusion variant. A male child was born to 28-year-old female at 34 weeks of gestation. The mother on antenatal follow-up was detected to have a fetus with multiple congenital anomalies on Ultrasonography (USG) done at 34weeks of gestation. The baby died after 12 hours of birth. A complete autopsy was performed. On external examination, multiple congenital anomalies were seen including cleft lip and palate, absent nasal bridge, proptosis of right eye, micropenis, left undescended testis, bilateral rocker bottom feet, omphalocele and sacral meningomyelocele. Internal examination of the brain revealed hydrocephalus and features of alobar holoprosencephaly. This case is presented for its rarity. In addition, it is unusual for a fetus with alobar holoprosencephaly to survive till term as this is the most severe type. Though facial malformations are usually present in a case of holoprosencephaly, its association with sacral meningomyelocele and omphalocele has rarely been described in literature.

A Survivor of Neonatal Intestinal Mucormycosis.

Gastrointestinal mucormycosis (GIM) is a rare opportunistic fungal infection. One third of all patients are children and of these, 50% are infants. The most common clinical mimic is necrotizing enterocolitis (NEC). It has to be differentiated from this entity as the treatment is entirely different. High index of suspicion by the clinicians and the pathologists aids in early diagnosis and immediate treatment. If untreated, it has a frequent fatal outcome. Very few survivors of GIM are found in literature. We report a rare case of a surviving neonate of GIM.

An Unusual Presacral Cyst in An Infant.

The neurenteric cyst is a rare developmental lesion arising as a result of persistence of the neurenteric canal. It usually presents in the second and third decade. Lesions occurring in children are rare. Majority of the cases presented with signs of spinal cord involvement. This is a rare case of neurenteric cyst in an infant who presented with a presacral mass. A nine-month-old female had an antenatally diagnosed sacrococcygeal lesion. Radiology suggested a diagnosis of sacrococcygeal teratoma. Histology was typical of a neurenteric cyst. We report this case to create awareness about this rare cystic lesion and inclusion of this entity in the differential diagnosis of cystic masses in children at this location.

Fetus in fetu: an institutional experience.

The objective of this article is to present an analysis of cases of fetus in fetu (FIF) diagnosed at our institute and to compare our data to that of the published literature. This is a retrospective analysis of cases of FIF diagnosed at our institute from January 2000 to December 2012. Details of clinical and investigational data pertaining to cases of FIF were retrieved from hospital records, and were analyzed and summarized. Literature was reviewed and our data were compared to that of published cases. We evaluated seven cases (4 males and 3 females) of FIF during the study period. The age of patients ranged from two days to 15 years. The most common site of occurrence in our study was the retroperitoneum, with the most common presenting feature being an abdominal mass. Radiology showed a solid-cystic mass. Pathologic findings were diagnostic of FIF. Most findings of our study matched with those reported in literature. The condition of FIF must be included in the differential diagnosis of abdominal masses, especially in children. This entity must be differentiated from the more commonly occurring teratoma, which can be done based on its unique clinical, radiologic, and pathologic characteristics. To our knowledge, this is the longest series of cases of FIF reported from a single institute.

Sirenomelia with oesophageal atresia: a rare association.

We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.

Multifocal nephrogenic adenoma ­ a mimicker of malignancy.

The authors report a multifocal nephrogenic adenoma of urinary bladder in an 11-y-old girl. Nephrogenic adenomas occur almost exclusively in the urinary bladder in children and are rarely multifocal. Less than 30 cases of nephrogenic adenomas have been reported in children and very few of these are multifocal. This report aims at drawing attention to this rare entity in children.