Prevalence of neural tube defect and its identification during antenatal period: a cross-sectional study in eastern Indian state.

OBJECTIVE: To estimate the prevalence of neural tube defects among all birth outcomes in Odisha during 2016-2022. Additionally, to estimate the identification rate of neural tube defects during Pradhan Mantri Surakshit Matritva Abhiyan sessions. DESIGN: A population-based cross-sectional study with a household survey for neural tube defects using pictorial card as well as a hospital-based study for antenatal ultrasonography data. SETTING: The sample population was selected through multistage random sampling. In the first stage, one district from each zone was selected randomly. In the second stage, using simple random sampling, one community health centre and one urban primary health centre were selected from each district. In the third stage, the population from a block and ward were picked from the selected rural and urban settings, respectively. PARTICIPANTS: All married women in the reproductive age group (18-49 years) residing in these cluster villages in the selected districts were enrolled. RESULTS: The study surveyed 49 215 women and recorded 50 196 birth outcomes, including 49 174 live births, 890 stillbirths and 132 medical terminations of pregnancy. A total of 30 neural tube defect cases were detected. The overall prevalence rate of neural tube defect was 0.59 per 1000 birth outcomes. Spina bifida was the most prevalent neural tube defect with the prevalence of, followed by anencephaly and encephalocele. Despite 26 860 mothers receiving antenatal ultrasonography Pradhan Mantri Surakshit Matritva Abhiyan session, data on neural tube defects and other birth defects detected through these scans is unavailable. CONCLUSION: This study found a low prevalence of neural tube defect in Odisha, which is far lower compared with the older studies from India. There is an urgent need to strengthen the quality of antenatal care services provided under Pradhan Mantri Surakshit Matritva Abhiyan through better training regarding anomaly scans and better data keeping at public healthcare facilities. TRIAL REGISTRATION NUMBER: CTRI/2021/06/034487.

Congenital Intraoral Dermoid and Epidermoid Cyst with Orocutaneous Fistula Presenting with Life-threatening Airway Obstruction in an Infant.

Dermoid cysts and epidermoid cysts in the floor of the mouth are rare in the pediatric age group. In this case report, we have discussed the presentation, management, and literature review of a 6-month-old female child presenting with both dermoid and epidermoid cysts in the floor of the mouth with an orocutaneous fistula.

Congenital midline upper lip sinus in an infant.

Congenital lip sinus is a rare entity with upper lip sinus being rarer than the lower lip sinus. It can be an isolated entity or associated with cleft lip, palate or Van der Woude syndrome. Syndromic association requires proper evaluation and aggressive surgical treatment. Preoperative delineation of the sinus tract with ultrasound sonography or MRI is mandatory. Simple excision is sufficient in cases of isolated sinuses. In this article, we report an infant with upper lip sinus managed successfully with simple excision and reviewed the literature.

Assessment of Matrix Metalloprotease - 7 (MMP7) Immunohistochemistry in Biliary Atresia and Other Pediatric Cholestatic Liver Diseases.

Background and aims: Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy. The histopathological diagnosis is often challenging and an immunohistochemical marker is often sought as an adjunct. We evaluated MMP7 immunohistochemistry in BA and other non-BA pediatric cholestatic liver diseases. Materials and methods: MMP7 immunohistochemistry was applied in 5 age-matched normal control, 23 cases of BA and 43 cases of non-BA pediatric cholestasis including 16 cases of choledochal cyst (CC), and a multiplication score was obtained by multiplying the intensity and percentage positivity in the cholangiocytes. Results: BA showed a high mean MMP7 multiplication score which was significantly different from the normal control and other non-BA pediatric cholestatic diseases including CC (p value < 0.001). The sensitivity, specificity, positive, and negative predictive values of MMP7 immunohistochemistry were 91.3%, 93.02%, 87.5%, and 95.2% respectively. Conclusion: MMP7 immunohistochemistry may be an adjunct to histomorphology in BA.

Parosteal lipoma of rib causing widening of intercostal space in an adolescent.

Parosteal lipomas are typically seen in adults and affect mostly the diaphysis of the long bones of the upper and lower limbs. Only a few cases have been reported in children until now and none were reported involving ribs. A female adolescent patient presented with swelling on the left upper back over the scapular region for the past 4 years without any neurological deficit. MRI revealed a hyperintense lesion on the left paravertebral region extending from D5 to D12 and into the intercostal space, causing it to widen. The patient underwent surgical excision. The lipomatous mass was seen entering in to the intercostal space, causing it to widen. However, the pleura was not breached. In this report, we have performed a literature review compiling all cases of parosteal lipoma affecting the ribs.

Effect of preoperative estrogen on complications after proximal hypospadias repair: A randomized controlled trial.

Introduction: Proximal hypospadias repair has many postoperative complications such as urethrocutaneous fistulae, wound dehiscence, and urethral stricture. The beneficial effect of estrogen to promote wound healing has been known. We designed a study to determine whether preoperative stimulation of tissue with estrogen can reduce the postoperative complications associated with wound healing in patients undergoing hypospadias repair. Methods: Patients with proximal hypospadias requiring two-stage repairs (chordee correction followed by urethral tubularization) were randomized to estrogen and control groups before the second stage of surgery. In the former, topical estrogen cream (0.5 mg of estriol) was applied to the ventral penis for a month, whereas normal saline gel was applied to the latter; the urethroplasty was carried out thereafter. Patients were followed up for complications. Results: There were 29 patients in the estrogen group and 31 in the placebo group after the exclusion criteria were met. There was no significant difference in the overall postoperative complications between the estrogen group (44.8%) and the placebo group (51.6%). The occurrence of urethrocutaneous fistula (37.9% vs. 51.6%) and dehiscence (41.4% vs. 45.2%) was not significantly different between the estrogen and placebo groups. Neourethral stricture occurred in four patients in the estrogen group, while none of the patients in the placebo group developed stricture. Conclusions: The preoperative application of topical estrogen cream to the ventral penis failed to demonstrate any significant effect on wound healing and complications.

Urethral Caruncle with Associated Renoureteric Anomalies.

A urethral caruncle is a rare differential diagnosis for a prolapsed mass at the urethral meatus. The aetiopathogenesis of this entity is unclear. We report a three-year-old female patient who presented to a tertiary care teaching hospital in India in 2019 with intermittent bleeding from the introitus for a month. Investigations revealed a urethral caruncle and associated renal anomalies which has not been described in the previous literature. The patient was discharged with a prescription of a Sitz bath twice daily and advised to apply betamethasone (0.1%) cream locally once daily. There was a significant improvement after six weeks of therapy; at two-years follow-up, the lesion had disappeared completely.

Congenital Short Bowel Syndrome With Annular Pancreas Presenting as Neonatal Intestinal Obstruction.

Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with features of intestinal obstruction and failure to thrive. The abdominal radiograph was suggestive of a central gasless abdomen, and a provisional diagnosis of malrotation of the gut with volvulus was considered. On exploration, the duodenum was hugely dilated with annular pancreas, which was not obstructing the duodenum. The length of the intestine was 20 cm from the duodenojejunal junction to the ileocecal junction. Duodenal web was ruled out. Total parenteral nutrition (TNP) was started postoperatively. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome; however, the challenges are entirely different in developing countries.

Expression of CD56 is Not Limited to Biliary Atresia and Correlates with the Degree of Fibrosis in Pediatric Cholestatic Diseases.

BACKGROUNDS AND AIMS: CD56 immunostain is used as an adjunct to aid in the preoperative diagnosis of biliary atresia (BA) by liver biopsy. We aimed to study the expression of CD56 in different pediatric cholestatic diseases thereby evaluating its utility in the diagnosis of BA. METHODS: We performed immunohistochemistry for CD56 on 35 cases of pediatric cholestatic diseases and five age-matched controls. CD56 expression was assessed by a multiplication score (percentage positivity x intensity) in the biliary epithelium. RESULTS: The multiplication score between BA and choledochal cyst was not significantly different. High scores were also encountered in other cholestatic disorders. The score showed a significant negative association with serum albumin and a significant positive correlation with the serum ALT level. Very significant positive correlation between the score and portal fibrosis was obtained. CONCLUSION: CD56 expression is an infidel marker for the histological diagnosis of BA and rather provides a clue to the disease status in pediatric cholestatic diseases.

Loss of interest for training in paediatric surgery in India.

Background Despite the sizeable Indian paediatric population, few students have opted for postdoctoral (Magister Chirurgiae [MCh]/Diplomate of the National Board of Examinations [DNB]) courses in the past decade. We analyse the apparent loss of interest for training in paediatric surgery in India and suggest remedies. Methods We did a combination of an online questionnaire-based survey and several interviews among students, teachers and practitioners of paediatric surgery. The results were collated and analysed. Results Information from 238 questionnaires, 35 interviews and 75 feedbacks were distilled. About 83% of respondents agreed to a definite loss of interest among students in paediatric surgery, largely because of poor exposure during undergraduate/postgraduate courses and absence of a department in medical colleges. The blanket increase in seats has led to vacancies. The low saleability of paediatric surgery is linked to high personnel and infrastructure investment, modest remunerative potential and poor insurance cover for patients. Besides increasing public awareness, strategic governmental patronage in postdoctoral training (e.g. establishing a department in medical colleges, moderating the number of seats, encouraging performing training centres and rationalizing the bond after the training course) and facilitation of patient care (e.g. insurance cover for congenital conditions and paediatric surgeon in neonatal care units in district hospitals) is suggested. Conclusions The loss of interest in paediatric surgery among medical trainees is real and urgently requires a multipronged strategy by the medical fraternity, professional organizations and regulatory bodies across government and non-governmental sectors to facilitate a revival and cater to the sick surgical child in the future.

Awareness of Common Pediatric Surgical Entities among Practicing Indian Pediatricians.

AIM: Common pediatric surgical entities often get a delayed referral to the pediatric surgeon in the average Indian scenario. This study was conducted to assess the awareness about management of select common pediatric surgical entities among practicing pediatricians. MATERIALS AND METHODS: An online multiple-choice questionnaire consisting of twenty questions related to the diagnosis and management of common pediatric surgical entities encountered by pediatricians in their routine office practice was prepared, and the electronic link was circulated among the practicing pediatricians of India. Each question had one correct response. RESULTS: One hundred and seventy-five responses were obtained, collated, grouped, and analyzed. Overall, 35% of all responses were correct. Individually, 56% of respondents marked 51%-75% of answers correctly; only 7% scored above 75%. Some clinical observations were disparate: 65% were unfamiliar with preputial adhesions and 51% would discharge a preterm neonate with inguinal hernia without a surgical consult. There is a tendency toward unnecessary imaging (60% - localization of undescended testis, 91% - wet umbilicus, and 51% - postanal dimple) and overzealous medical management (propranolol for the involuting hemangioma). However, 82%-88% concurred on the standard conservative or surgical management in index conditions such as hypospadias and umbilical problems. CONCLUSION: There was a concurrence in the principles of management of common pediatric surgical entities between pediatricians and pediatric surgeons in 35%. Misdiagnosis, unwarranted investigations, overzealous medical management, and delayed surgical consults figured in 65%. A systematic professional interaction between the physicians and surgeons would facilitate a coordinated management. A larger study would yield more meaningful data.

Diminution of the Primary Cilia From the Intrahepatic Cholangiocytes in a Pediatric Choledochal Cyst.

Choledochal cyst (CC) is a cystic disease predominantly involving the extrahepatic biliary tree. Biliary atresia (BA), another disorder of the extrahepatic biliary tree, is sometimes considered to be in the same spectrum as pediatric CC. Recently, the absence and/or the structural abnormality of the primary cilia in the intrahepatic cholangiocytes have been implicated in the pathogenesis of BA. We aimed to evaluate the expression of primary ciliary proteins in the intrahepatic cholangiocytes in cases of pediatric CC and compare it with normal control and BA. We performed immunohistochemistry for primary ciliary proteins (acetylated-α-tubulin and double-cortin domain containing 2) on the liver biopsies of control liver (n=5), pediatric CC (n=13), and BA (n=14). We also compared the expression with various clinical, biochemical, histopathologic (portal fibroinflammation and ductal plate malformation), and immunohistochemical (proliferative index) data. There was significant loss of primary cilia from the intrahepatic cholangiocytes in cases of CC and BA as compared with the normal control by both immunostains (CC: P=0.003 and 0.001, respectively; BA: P=0.001 and 0.001, respectively). There was no significant difference between the CC and BA in terms of ciliary protein loss. The loss of the ciliary proteins occurred irrespective of the proliferative (MIB-1 labeling) index, portal fibroinflammation, or ductal plate malformation. The loss of cilia did not correlate with the clinical follow-up in cases of pediatric CC. The loss of primary cilia from the intrahepatic cholangiocytes may be crucial in the etiopathogenesis of pediatric CC.

Heteropagus Twins: A Tale of Two.

Parasitic twin is a rare occurrence, with rachispagus being the rarest. The surgical approach and timing are not standardized. We describe the management of two cases of rachispagus and review the sparse literature on this entity. Both cases were operated early because of the social burden on the family as well as difficulty in nursing care. The first patient had associated major systemic malformations in the autosite (cerebral, spinal and cardiac), and succumbed in the postoperative period. The entity was isolated in the second who is doing well.

Acute limb ischemia following iatrogenic femoral artery injury during orchiopexy.

Orchiopexy for undescended testis is a standard procedure in pediatric patients. Intraoperative complications during orchiopexy are rare. Major complications reported include injury to vas deferens or testicular vessels, leading to testicular atrophy. Damage to the femoral artery has not been described in the literature as a complication during orchiopexy. In this report, we describe a case where injury to the right femoral artery leading to acute limb ischemia occurred while performing orchiopexy.

Xanthogranulomatous cystitis masquerading as bladder tumor in a child.

Xanthogranulomatous cystitis affecting the urinary bladder is extremely rare, and only around thirty adult cases and two pediatric cases have been reported in the literature. The treatment is predominantly surgical as the lesion is mostly infiltrative and mimics malignancy. We report probably the third pediatric case, who presented with symptoms of urinary tract infection and urinary retention and was initially suspected as bladder tumor on imaging. The diagnosis was confirmed on histopathology, and the child responded well to aggressive antibiotic therapy alone.

Intracranial, Extradural, Hemangiopericytoma in a Neonate.

Intracranial infantile hemangiopericytoma (HPC) is a rare, sparsely documented neoplasm with a relatively favorable prognosis than its adult counterpart. We describe a neonatal extradural, intracranial, infantile HPC managed with near-total excision.

The Presence of Pericholedochal Hyaline Cartilage in Biliary Atresia: A Report and A Review.

Background: The presence of cartilage in extra hepatic biliary tree is an unusual finding. An isolated presence of the cartilage is possibly heterotopic or occurs as a metaplastic response to the inflammatory insult.Material and methods: We had examined the liver biopsy and the resected specimen of a biliary atresia (BA) after Kasai procedure.Results: There was hyaline cartilage around the common hepatic and common bile duct in a 3-months-old male infant with distal obstructive cholangiopathy on liver biopsy and had positive serum IgM for cytomegalovirus (CMV). Similar findings could not be documented in the pericholedochal tissue of any of the 25 other pediatric cases operated for BA or choledochal cyst and three neonatal autopsies performed for liver-related deaths.Conclusion: Peri-bile duct cartilage is a unique finding and could represent an unusual form of heterotopia or connective tissue metaplasia.

Outcome Analysis of Patients of Congenital Hydrocephalus with Ventriculoperitoneal Shunt at a Tertiary Care Hospital in North India.

INTRODUCTION: Ventriculoperitoneal (VP) shunt is a commonly performed neurosurgical procedure in pediatric surgical practice. This study is aimed at determining the outcome of congenital hydrocephalus patients who underwent shunt surgery at our center. MATERIALS AND METHODS: A total of 278 operated cases of congenital hydrocephalus with a minimum follow-up of 6 months were analyzed in terms of complications. Hydrocephalus associated with intracranial hemorrhage, neural tube defects, and ventriculitis were excluded from the study. RESULTS: Complications were observed in 32.0% (89/278) of cases. Shunt block and chamber migration were the most common complications (29.21%). Abdominal wound complications were seen in 4.49% of cases. Shunt exposure due to erosion of overlying skin was noted in 6 patients. Shunt infection was diagnosed in only 9 patients (4.4%). New-onset seizures were seen in 4 cases. Overall, 21% of patients required redo shunt and there were 2 mortalities. CONCLUSION: VP shunt continues to have high complication rates. Shunt migration and new-onset seizure disorders are important late complications. In spite of inherent problems, shunt surgery is the procedure of choice until a safer effective alternative is available.

Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy.

Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods: We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion: The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.