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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Multiple neural tube defects may not be very rare.

BACKGROUND: Multiple neural tube defects (MNTDs) are a rare occurrence. Although the clinical incidence is small, MNTDs raise some interesting embryological queries. AIM: This study aims to investigate the morphological and clinical variations observed in neonates presenting with multiple neural tube defects and associated central nervous system anomalies. MATERIALS AND METHODS: This is a prospective study carried out at our institute to assess clinical and morphological variation in patients presenting with multiple neural tube defects. RESULTS: Among the 263 patients with a neural tube defect, who presented to our outpatient department and emergency departments, only 10 cases of MNTDs were identified. Thus, incidence of MNTDs in the cohort of patients affected with NTD was 0.038 %. Among the 10 patients, 9 had double neural tube defects and 1 patient had three neural tube defects. CONCLUSIONS: Multiple neural tube defects may not be very rare in the general population, especially the populations with high incidence of neural tube defects. Multisite closure theory has the versatility to explain various combinations of neural tube defects, but better insights into the molecular pathways governing this tightly regulated process can provide us the missing link in establishing the pathogenesis of multiple NTDs. It can also provide us with an opportunity to prevent NTDs or treat them in utero by pharmacological modulation of these signaling pathways.

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.

Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula.

OBJECTIVE: The role of preoperative contrast-enhanced computerized tomography (CT) of chest with three-dimensional (3D) reconstructions was evaluated in neonates with esophageal atresia and tracheoesophageal fistula. METHODS: This was a prospective study which investigated 30 cases of esophageal atresia with tracheoesophageal fistula. All patients were evaluated preoperatively with contrast-enhanced spiral CT using a low-dose CT protocol. 3D CT reconstruction images were evaluated for the type of esophageal atresia, the distance between the upper and lower esophageal pouches, origin, level and position of the fistula, and the presence or absence of any other cardiac, pulmonary or mediastinal lesions and the findings were correlated with the findings at surgery. The radiation dose for each patient was calculated using the formula-Effective dose (E) = DLP × (E/DLP)age. RESULTS: All the 30 cases had type-C esophageal atresia with tracheoesophageal fistula as per Gross classification. The exact site of the fistula could be identified only in 26 (80 %) cases. The mean gap between the upper pouch and lower fistula was 0.95 ± 0.57 cm (range 0.2-2.8 cm) on CT scan and 1.38 ± 0.61 cm (range 0.5-3.2 cm) at surgery. On statistical analysis, the correlation was found to be significant (p < 0.0001). In addition, lung pathology (consolidation), cardiac pathology and vertebral anomaly were also detected on CT scan in some cases. The mean radiation dose for the neonates who underwent CT chest was calculated to be 1.79 mSv which is significantly high. CONCLUSION: Though preoperative CT scan of chest has many advantages, it involves significant exposure to ionizing radiation and risk of radiation-induced cancer in the future. Additionally in 20 % of cases, the fistula could not be located on CT scan. The most common variety of esophageal atresia and tracheoesophageal fistula is Gross type C (86 %) that has low to intermediate gap (97 %) and can be anastomosed primarily. Thus, CT scan can provide good anatomical delineation, but may not help in surgical decision making. Hence, performing CT in these cases would unnecessarily expose the neonates to ionizing radiation. Therefore, there is no role for CT scan in the routine preoperative assessment of EA with distal TEF.

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.

Rare association in a female DSD case of phallus, accessory phallic urethra, perineal lipoma and anterior ectopic anus.

Disorders of sex differentiation (DSD) are a heterogeneous and broad spectrum group of diseases with a varied appearance. Presence of an accessory phallus with a phallic urethra in association with a normal vagina in a female is an extremely uncommon anomaly. We present a rare case of a genotypically female child with a normal urethra and vagina in association with a phallus, accessory phallic urethra, perineal lipoma and anterior ectopic anus.

Transilluminating testicular mass.

Testicular tumor in prepubertal age group is uncommon. Most of the testicular tumors are benign in this age group and present as painless, hard testicular mass. We present a case where a 7 year male child presented to us with painless scrotal swelling, which was cystic and transilluminating, clinically mimicking as hydrocele. On ultrasonography, the mass was solid. Orchidectomy was performed and histopathology revealed mature cystic teratoma.

Glial heterotopia of maxilla: A clinical surprise.

Glial heterotopia is a rare congenital mass lesion which often presents as a clinical surprise. We report a case of extranasal glial heterotopia in a neonate with unusual features. The presentation, management strategy, etiopathogenesis and histopathology of the mass lesion has been reviewed.