Kanishka Das

Tracheobronchial foreign bodies in infants

OBJECTIVE Tracheobronchial foreign body aspiration (FBA) is a common cause of respiratory distress between 1 and 3 years of age. Literature on airway foreign bodies in this age group is abundant; however no study has addressed this problem in infants exclusively. This study aimed to review the clinical presentation, management and outcome of infants with tracheobronchial foreign bodies at a referral tertiary care hospital over a decade. METHODS 102 infants who underwent bronchoscopy for suspected FBA from 1997 to 2007 were retrospectively reviewed. Details of demographic data, clinical features, radiologic and bronchoscopic findings, postbronchoscopy events and eventual outcome were analysed. RESULTS The mean age was 10.5 months. 8 (7.8%) were 0-6 months of age, the youngest being 2 months. Males outnumbered (72:30) females. 10 (9.8%) presented secondarily after treatment elsewhere, 6 of them were initially misdiagnosed. The onset-presentation interval ranged from 1 day to 3 months, 41 (40.2%) reporting within a day and 19 (18.6%) a week after onset. 20 (19.6%) had no history suggestive of FBA but harboured airway FBs at bronchoscopy. The clinical triad of cough, respiratory distress and stridor was highly predictive of FBA. 8 (7.84%) had no abnormal physical findings while 8 (10.81%) had grossly normal chest radiographs. All the patients underwent emergency/elective rigid bronchoscopy (Karl Storz system) under general anesthesia as in-patients. The physical findings did not always correlate with radiology or bronchoscopic location of the FB. A peanut cotyledon was the commonest FB retrieved across infancy; in 5 (4.9%) no FB was found/identifiable. 2 required postbronchoscopy mechanical ventilation and 1 a repeat bronchoscopy. There were 6 complications but no mortality in the series. The average hospital stay was 1.4 days. At a weeks follow-up, all were asymptomatic and well. CONCLUSIONS The clinical presentation, diagnosis and management of FBA in 102 infants are presented and certain peculiar features are described. A high index of suspicion coupled with a combination of history, physical signs and radiology is more conclusive than any of them in isolation. Availability of expertise and endoscopic equipment ensures a favourable outcome without significant morbidity and mortality.

Hirschsprung's disease, associated rare congenital anomalies

Objective: Hirschsprung’s disease may be associated with a number of congenital anomalies of which Down’s syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung’s disease.Methods: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung’s disease encountered over two years was performed.Results: Besides Down’s syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed.Conclusion: Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung’s disease.

Segmental dilatation of sigmoid colon in a neonate: atypical presentation and histology

Segmental dilatation of the colon is a rare disorder of colonic motility in children, often presenting with severe constipation in older infants, children, and occasionally adults. It may mimic the commoner Hirschsprung disease clinicoradiologically but differs in that the ganglion cell morphology and distribution are typically normal in the colon. We report a neonate with segmental dilatation of the sigmoid colon who had an atypical clinical presentation and describe certain abnormalities in bowel histology (hypertrophied muscularis propria, nerve plexus, and ganglion cells located within the circular layer rather than the normal myenteric location), for the first time in the English literature.

Preputial adhesions — A misunderstood entity

ObjectiveTo assess the trends and outcomes in referrals of ‘phimosis for circumcision’ to a tertiary care pediatric surgical department.MethodsThis is a prospective study of 100 consecutive children of presumed phimosis referred for circumcision. They were assessed and classified by the senior author as having either preputial adhesions or phimosis. The patients in the former group underwent outpatient preputial adhesiolysis while those in the latter group were offered circumcision primarily. All were followed up till resolution.ResultsAll 100 were referred with a non-retractile prepuce and an additional reason — preputial ballooning at voiding, dysuria or suspect UTI. Eight (mean age −58 months, referred for preputial ballooning at voiding −6/8 and dysuria −2/8) had phimosis and were primarily offered circumcision. The remaining 92 (mean age — 22 months, referred for preputial ballooning at voiding −52/92, dysuria −28/92 and suspect UTI −12/92) had preputial adhesions. In the latter group, none has a documented urinary infection on specific investigations. These 92 underwent outpatient preputial adhesiolysis. Seventy three (79 %) required a single sitting, 11 (12%) required 2 −4 sittings and 4(4.5%) required 5−8 sittings over a follow up period of 1 −24 months (median −3 weeks). 4/92(4.5%) were deemed non-responders to adhesiolysis and were circumcised later.ConclusionThe majority of children referred with ‘phimosis for circumcision’ to this tertiary pediatric surgical centre were actually physiologic preputial adhesions that were managed with outpatient preputial adhesiolysis. This study underscores a lack of awareness amongst referring primary care physicians regarding preputial adhesions and the potential for an erroneous diagnosis of phimosis translating into unnecessary circumcisions in many young boys.

Periscapular bronchogenic cyst

Periscapular location of bronchogenic cysts is extremely rare with four cases reported in literature. We report a relatively late presentation in a ten-year-old boy that was clinically mistaken for a typical sebaceous cyst. The definitive treatment is total excision after preoperative imaging to detect the occassional intrathoracic extension. Histologically they are lined by pseudostratified ciliated columnar epithelium, which can rarely undergo malignant transformation.

The distal level of normally innervated bowel in long segment colonic Hirschsprung's disease

PurposeThe definitive surgical management of Hirschsprung’s disease (HD) depends on an unambiguous histological diagnosis of bowel aganglionosis, as well as an accurate assessment of its proximal extent from the rectum or ‘leveling’. This study aimed to evaluate and compare the transition zone in rectosigmoid and long segment colonic forms of HD.MethodsOne hundred and fifteen cases of rectosigmoid Hirschsprung’s disease (RSHD) and 20 cases of long segment colonic Hirschsprung’s disease (LScHD) managed from 1998 to 2008 with a consensus prospective protocol were included in this clinicopathological analysis.ResultsIn RSHD, the radiologic transition zone (rTZ)/gross appearance at laparotomy correlated with the histological ‘leveling’ in the majority (96%); the histologic transition zone was also short and predictable. In contrast, the rTZ in LScHD was conclusive in only 53%; overall the radiologic and histological transition zones concurred in only 26%. The histologic transition zone extended proximally for an unpredictable length before normal circumferential innervation was noted.ConclusionsA stringent intraoperative histological evaluation of the colon as outlined is more helpful than radiology to establish the distal level of normally innervated bowel in LScHD and achieve an accurate leveling during a colostomy/pull through.

Sporadic synchronous ganglioneuromas in a child—case report and review

An 8-year-old girl presented with a relatively asymptomatic abdominopelvic mass that was detected in the neonatal period. She harbored a presacral mass with intraspinal extension and a right posterior mediastinal mass; all were excised completely and were ganglioneuromas. The report discusses the maturation of neuroblastoma-ganglioneuromas and details the staged management of sporadic synchronous ganglioneuromas in a child, probably the first in published English literature.

Fetal rhabdomyoma of the lower extremity

We report a rare case of cellular fetal rhabdomyoma in a 9‐year‐old male, in the unusual location of right lower thigh. These tumors are more common in the head and neck region; and this case the second such case to be reported in the thigh. Fetal rhabdomyoma is a benign tumor of the skeletal muscle, showing varying degrees of skeletal muscle maturation. The present report discusses the histopathological features, the differential diagnosis and the importance of making the correct diagnosis for proper management of this rare entity. Pediatr Blood Cancer 2009;52:881–883.

Primary pleural non-Hodgkin lymphoma in a child--an exceedingly rare disease.

Primary pleural lymphomas are very rare. Two types are described in the literature: primary effusion lymphoma, in the setting of human immunodeficiency virus infection, and pyothorax-associated lymphomas, with a strong Epstein-Barr virus association. We report a rare case of a primary pleural lymphoma in a 12-year-old immunocompetent girl who presented with a hemorrhagic pleural effusion and had plaque-like thickening of the pleura. The histologic and immunophenotypic findings conformed to that of a diffuse large B-cell lymphoma (CD20 positive).

Hemihypertrophy, renal dysplasia and benign nephromegaly.

Hemihypertrophy is associated with malignant visceral abdominal neoplasms in childhood. Benign nephromegaly and nephroblastomas are both known to occur with hemihypertrophy; however, association with renal dysplasia has not previously reported. We describe an infant presenting with recurrent haematuria who had segmental hemihypertrophy, ipsilateral renal dysplasia and contralateral benign nephromegaly. Although debated, renal dysplasia may predispose to and predate malignant change. Rational management and optimal surveillance of renal dysplasia and benign nephromegaly in hemihypertrophy is discussed.