A case of arteriovenous malformation of the hand and its outcome.

INTRODUCTION AND IMPORTANCE: AVMs are rarest congenital anomalies constituting around 1% of pediatric population. Arteriovenous malformations originate at 4th to 10th weeks of intra uterine life due to persistence of primitive arteriovenous shunts. Though the exact prevalence is unknown, upper limb constitute around 10 % of all AVMs. Hand being the most common region affected after head and neck. CASE PRESENTATION: Author is presenting here a rare case of palmar arteriovenous malformation in child since birth, hindering her routine daily activities, and its unique way of surgical management. CLINICAL DISCUSSION: Hand arteriovenous malformations (AVMs) are challenging to treat due the necessity to maintain function and aesthetic appearance. CONCLUSION: The risk of fatal bleeding makes resection of AVMs a challenging and stressful procedure, but however in the presence of available efficient equipments to control the bleeding and expertise of the available treating surgeons, primary surgical resection can be considered a feasible option in a tertiary care pediatric center.

Congenital duodenojejunal junction obstruction; report of two rare cases.

INTRODUCTION AND IMPORTANCE: Congenital obstruction of duodenojejunal junction is a rare unexplored pathologic entity. Most of the cases reported so far are regarding extrinsic band or narrower attachment of ligament of Treitz, which will be presenting with vomiting in neonates and children without malrotation. CASE PRESENTATION: Author is reporting here two rare cases of congenital intrinsic duodenojejunal junctional obstruction and their management in toddlers. CLINICAL DISCUSSION: Duodenojejunal junction, is an embryologically, pathologically and radiographically, yet unexplored region except anatomically and surgically. Only few pathologies have been described in this region so far CONCLUSION: An abnormal embryogenesis could be speculated and contemplated to be the reason for such rare congenital duodeno jejunal junctional obstruction which should be managed promptly as per required surgical techniques.

A case of ganglioneuromatosis in a child and its outcome.

INTRODUCTION: Ganglioneuromas (GNs) are hamartomatous tumors that originate in sympathetic ganglia and adrenal glands. Rarely, they might originate in enteric nervous system affecting its motility. Clinically they present with varying symptoms of pain abdomen, constipation and bleeding. Nevertheless, patients can remain asymptomatic for many years. CASE PRESENTATION: Author is reporting here a case of ganglioneuromatosis of intestine in a child and its effective management by a simple surgical procedure, yielding good result without morbidity. CLINICAL DISCUSSION: Intestinal ganglioneuromatosis is a rare form of benign neurogenic tumor characterized by hyperplasia of ganglion cell nerve fibers and supporting cells. CONCLUSION: Intestinal ganglioneuromatosis, diagnosed only after histopathological examination, should be managed either by conservative or surgical procedure, depending upon the clinical scenario by attending paediatric surgeon.

A rare case of anterior mid line neck cyst in a child.

INTRODUCTION: Mid line neck swellings are common in paediatric as well as in adults. They can be of three categories; inflammatory, neoplastic, and congenital. CASE PRESENTATION: A child with history of a nodular swelling over the anterior midline neck, its unusual diagnosis and its management has been discussed here. CLINICAL DISCUSSION: Many of Non thyroidal lesions can mimic and present like thyroid nodules. Clinical examination with preoperative work-up will help to differentiate such lesions, in planning subsequent surgical intervention and hence avoiding iatrogenic damage to the thyroid gland. CONCLUSION: Among the diverse and numerous mid line neck lesions, the clinical assessment can only aid in justifying the surgical procedure in these lesions.

A decompressing abdominal mass and its outcome in a child-case report.

Introduction: Mesenteric cysts are benign tumours occurring in both adults as well as in children; can be single or multiple, unilocular or multilocular. Importance: Pure chylous cysts as presenting as decompressing mass are rarely reported so far. Case presentation: a 2year old male child presenting with vague fullness of the abdomen and occasional vomiting since 7months of age, on imaging studies revealed it to be giant mesenteric cyst. Clinical discussion: Chylous cysts are rare variants of mesenteric cysts with an incidence of 7.3-9.9% in general population. Conclusion: The clinician should have high index of suspicion for a possible chylous mesenteric cyst for a clinically decompressing abdominal cyst which should be explored promptly.

Testicular torsion; clinical diagnosis or imaging diagnosis?

Testicular torsion is the most common urosurgical emergency in infants, children, and young adolescents, resulting in irreversible ischemic injury within hours; hence demanding urgent surgical attention. It has a bimodal distribution with the highest incidence occurring after puberty, followed by the neonatal period peak. Though the imaging modalities are helpful in the diagnosis, nothing can supersede the clinical findings and judgment.

Bell clapper deformity in female?

INTRODUCTION: Indirect inguinal hernia is one of the most common congenital anomaly common in males, but not uncommon in females. In females, inguinal hernia mostly indirect, containing ovary presenting as a palpable labial mass is common, resulting in strangulation, torsion, and infertility if left untreated. IMPORTANCE: The bell clapper deformity (BCD) is an important anatomical risk factor for intra vaginal testicular torsion, its presence in females is hardly been speculated. CASE PRESENTATION: A 6 months female baby presenting with tender labial mass, its management has been discussed here. CLINICAL DISCUSSION: The bell clapper deformity, in which the tunica vaginalis inserts high on the spermatic cord, leaving the testis free to rotate and get torted, however its presence in females has never been explored. CONCLUSION: Bell clapper deformity's existence in females yet to be speculated before exploration.

A rare case of jejunal atresia.

INTRODUCTION: Intestinal atresia more common in the small bowel, apart from large intestine. Jejunal atresia characterized by complete occlusion of the intestinal lumen, is a rare congenital anomaly occurring in 1 in 12,000 live births. IMPORTANCE: The jejunal atresia can be single or multiple occurring anywhere from the ligament of Treitz to the jejuno-ileal junction, requiring immediate surgical attention to prevent mortality and morbidity among these neonates. CASE PRESENTATION: A rare case of jejunal atresia in neonate and its management has been discussed here. CLINICAL DISCUSSION: Surgical excision of the involved bowel and end to end anastomosis of the normal bowel is definitive treatment. CONCLUSION: The morbidity associated with post-operative hypo persistaltic bowel can be minimised by adding oral prokinetics in controlled manner.

An unusual manifestation of a foreign body airway in a toddler.

Foreign body (FBA) in the airway is a common cause of sudden onset of respiratory distress in children. Prompt recognition and early intervention is required to minimize the potentially serious and sometimes fatal consequences.

An Unusual Case of Neonatal Perianal Lesion and Its Management.

Infantile hemangiomas (IHs) are common vascular tumors occurring in infants. The IHs have a characteristic natural course. Most of the uncomplicated IHs undergo spontaneous involution, with a small proportion of cases requiring intervention. Perianal hemangiomas are rarely reported in infants.

Congenital right diaphragmatic defects: Our institutional experience.

BACKGROUND: The descriptive clinical study was conducted to analyse the clinical profile as well as the outcome of congenital right diaphragmatic defects among children including neonates in a tertiary care referral neonatal and paediatric centre in southern Karnataka, India. MATERIALS AND METHODS: This retrospective and prospective observational clinical study was conducted from January 2005 to August 2019, over a period of 14.7 years in a tertiary care referral neonatal and paediatric centre. Clinical characteristics and risk factors of 33 children including neonates admitted and diagnosed with congenital right diaphragmatic defects were assessed both pre- and postoperatively. Neonates and children with acquired right diaphragmatic hernia defects and the left-sided diaphragmatic defects were not included in this clinical study. RESULTS: For statistical as well as clinical analysis, 33 study subjects were grouped into four groups, depending on the pre-operative and intraoperative findings as well as on their final diagnosis. Group I comprised right congenital diaphragmatic hernia (RCDH) (n = 18), Group II comprised RCDH with sac (n = 6), the babies with diagnosis of right diaphragmatic eventration were included in Group III (n = 7), whereas babies with other right-sided diaphragmatic hernia defects diagnosis were included in Group IV (n = 2). CONCLUSION: Right-sided congenital diaphragmatic defects, though rare, do carry excellent survival if referred early and managed in a tertiary care neonatal and paediatric centre as that of left diaphragmatic defects.

Early neonatal lobectomy for ILE; is it feasible?

INTRODUCTION: Congenital lobar emphysema (CLE) also called congenital lobar overinflation and infantile lobar emphysema is a rare congenital anomaly of lung often presents in the neonatal period, with hyperinflation of one or more pulmonary lobes. IMPORTANCE: Author is reporting here an uneventfully done earliest ever neonatal lobectomy for ILE.

A rare case of chest wall reconstruction in a child.

INTRODUCTION: Ewing sarcoma/primitive neuroectodermal tumour (ES/PNET) is the most common malignant tumour of the chest wall in children and young adults. Chest wall defect left after complete resection of the involved ribs and chest wall defect requiring reconstruction, is surgically challenging for cosmetic as well as for functional purposes especially in growing children. IMPORTANCE: A rare but feasible and simple technique for a case of chest wall tumour reconstruction has been described here with its successful outcome with available composite muscular vascularised flap.

Persistent Left Superior Vena Cava: A Rare Association.

Persistent left superior vena cava (PLSVC) is the most frequent abnormality in the general population with the frequency of 0.1% to 0.5%. It results from the failure of the involution of the left anterior cardinal vein. Right and Left SVC can coexist together in 80% to 90% of cases. Association of PLSVC with ano rectal malformation (ARM) is very rarely reported. Hence, here is a report of a unique case of PLSVC in a female neonate with ARM.

A Terminal Colovesical Fistula in an Anorectal Malformation.

Anorectal malformations are relatively common anomalies encountered in pediatric surgical practice. They are usually recognized at birth with absent anal canal or failure to pass meconium and hence can lead to life threatening bowel obstruction without surgical intervention. We are reporting a rare case of non-syndromic, sporadic, terminal colovesical fistula associated with high anorectal malformation not associated with congenital pouch colon in a neonate.

Pedunculated Perianal Mass in a Neonate: A Rare Case Report.

Congenital perianal pedunculated masses are a rare occurrence in neonates. They are benign in nature and can be excised completely for definitive diagnosis. Here is a report of a rare case of pedunculated perianal mass occurring in a neonate managed successfully.

Giant Colonic Lithobezoar: A Rare Case Report.

Bezoar is defined as the accumulation of undigested foreign bodies or nutrients in the gastrointestinal tract. Lithobezoar, the accumulation of stones in the digestive tract, is commonly seen in the stomach. We report a case of giant colonic lithobezoar in a child.

Stridor in a child: It's diagnostic challenges.

INTRODUCTION: Foreign body aspiration (FBA) is an extremely serious life threatening condition in infants and children. CASE REPORT: We are reporting a case of multiple foreign bodies lodged in respiratory tract in an infant. DISCUSSION: FBA is the most common cause of accidental death among infants and toddlers. CONCLUSION: History suggestive of or even suspicion of FBA is enough for subjecting the child for further confirmatory test such as rigid endoscopy in spite of negative imaging modalities.