Congenital Pulmonary Airway Malformation - A Histomorphological Spectrum of 15 Cases: A 5-Year Study from a Tertiary Care Center.

OBJECTIVE: Congenital pulmonary airway malformation (CPAM) is a rare developmental lung disease. The aim of this study is to analyze the histomorphological spectrum of CPAM in a series of 15 cases. MATERIALS AND METHODS: A retrospective descriptive study of 15 cases of CPAM was carried out from 2013 to 2018 in our hospital, and cases were classified based on the Stocker's classification. RESULTS: The age ranged from 4 days to 9 years (66.6% were infants). The left lung was most commonly involved (66.6%). The most common lobe was the left upper lobe (60%), followed by right lower lobe (20%). Grossly, cysts measured 0.2-5 cm, filled with mainly serous fluid with few having hemorrhagic and brownish mucoid secretions. On microscopy, single to multiple noncommunicating cysts of size 0.2-5 cm were seen, lined by ciliated columnar epithelium (60%), pseudostratified ciliated columnar epithelium (26.7%), mucin-secreting columnar epithelium (6.7%), and flattened epithelium (6.7%). Few cases showed smooth muscle (20%) and cartilage (13.3%) in the cyst wall. Chronic inflammation (73.3%) with dense histiocytic infiltrate (13.3%) was also seen. Emphysematous changes were also observed (13.3%). Cytomegalovirus inclusions (6.7%), zygomycete fungus (6.7%), and red hepatization (6.7%) were observed. The most common type was type II (60%), followed by type I (33.3%) and type IV (6.7%). CONCLUSION: Type II was the most common variant in this study. A careful observation should be done to look for fungal hyphae or viral inclusions.

Anaplasia in childhood rhabdomyosarcoma: An under reported entity.

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Anaplasia is a rare phenomenon seen in childhood RMS. The most common histologic subtype was Embryonal followed by Alveolar and spindle subtype. Design: A total of 11 cases of pediatric RMS were selected from January 2017 to June 2019 presenting at various sites. Out of 11 cases, 2 were further diagnosed as Embryonal, 2 as Alveolar, 2 as Pleomorphic, 1 as Spindle subtype and rest 4 as RMS-NOS based on morphology. All cases were positive for Desmin. The presence of cells with lobated, hyperchromatic nuclei at least three times larger than the tumor cell (anaplastic cells) was selected as the main criterion to diagnose Anaplasia. Results: Out of the total 11 cases, anaplasia was seen in 7 cases. Out of these seven, five cases showed Focal Anaplasia (FA) (71.4%) and 2 cases showed Diffuse Anaplasia (DA) (28.6%). Out of 2 cases of Embryonal RMS one exhibited focal anaplasia (50%). One case of Spindle RMS showed diffuse anaplasia, 2 cases of pleomorphic RMS showed focal anaplasia. Out of 3 cases of RMS- NOS, 2 exhibited focal anaplaisa and one displayed Diffuse anaplasia. Both Alveolar RMS had no features of anaplasia. Conclusion: Presence of Anaplasia is a frequent observation in pediatric RMS. Anaplasia is often under reported in pediatric RMS. Pathologist should be more aware of this rare phenomenon.

Histomorphological patterns of hepatoblastoma in a tertiary care hospital.

BACKGROUND: Hepatoblastoma (HB), rare malignancy in itself, is the most common primary liver tumor in children. Most common presenting features are abdominal distension or abdominal mass. Several patterns are associated with HB with different prognosis. Furthermore, some patterns have overlapping features with other childhood tumors. AIMS AND OBJECTIVES: The aim of this study is to discuss various patterns of HB which we came across in a tertiary care hospital during our study. H and E slides were reviewed with respect to different patterns, postchemotherapy changes including extramedullary hematopoiesis, necrosis, osteoid metaplasia, necrosis, and fibrosis. CONCLUSION: Different patterns of HB should be kept in mind by the pathologists to avoid any misdiagnosis.

Volvulus complicating dextrogastria in an infant.

We report eventration of right hemi-diaphragm resulting in gastric volvulus of the right sided stomach in an infant. The diagnosis of this rare association was made with contrast CT scan. Patient was initially managed with reduction of stomach, plication of right hemi diaphragm, anterior gastropexy and Ladds procedure, but required re-laparotomy after two months for recurrent volvulus.

Completely isolated, noncontiguous duplication cyst.

Enteric duplication cysts are hollow, epithelium-lined, cystic, or tubular structures that are intimately attached to a portion of the gastrointestinal tract. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply. Only two such previous cases have been reported in the literature so far. We report a third case of completely isolated, giant duplication cyst with a vascular pedicle in a four-day-old neonate with a review of the literature.

Carmi syndrome complicated by pharyngo-esophageal perforation.

We describe a neonate having congenital pyloric atresia (CPA) associated with epidermolysis bullosa (EB), also known as Carmi syndrome. The case is unusual as it was complicated with pharyngo-esophageal perforation (PEP) and a definite family history of EB in pervious generations could be recorded.

Covered exstrophy with visceral sequestration: case report and review of literature.

We report a case of a male newborn with asymmetrical epispadic diphallia and congenital urethral fistula, and exomphalos minor. A segment of bowel was attached in the region of the pubis; with no communication to the underlying bowel. This was excised; histological examination revealed this to be a colonic remnant. The excision of the examphalos minor and approximation of the pubis and the rectus sheath over the bladder was also done; urethroplasty is planned at a later date. A comprehensive review of the available literature revealed only eight such cases before this; the possible embryology is discussed.

Forme fruste choledochal cyst.

Two girls presenting with features of pancreatitis were diagnosed to have minimal dilatation of extra hepatic biliary duct (EHBD) associated with pancreatico-biliary maljunction (PBMJ). A high degree of suspicion is required to diagnose this condition that has been termed Forme fruste choledochal cyst (FFCC). Both did well with pancreatico-biliary disconnection procedure and reconstruction of biliary channel using enteric conduit.

Neuroblastoma with concomitant giardiasis: report of a case with diagnosis by fine needle aspiration cytology.

BACKGROUND: Diagnosis of two pathologies, including a neoplasm and infectious condition, by fine needle aspiration (FNA) cytology in the same patient is rare. CASE: A 2-year-old, male child presented with fever, abdominal pain and abdominal mass. Imaging findings were strongly in favor of a neuroblastoma. FNA smears from the mass revealed fecal material containing numerous trophozoites of Giardia lamblia. FNA was repeated in view of the imaging findings. Repeat smears showed a small round cell tumor with rosettes and background filamentous/fibrillar material consistent with a neuroblastoma. Chemotherapy reduced the mass considerably. Histopathology of the resected residual mass revealed a ganglioneuroma in addition to remnants of neuroblastoma. The patient was free of disease two years after the initiation of chemotherapy. CONCLUSION: When FNA cytology shows an infectious pathology in the clinical and imaging setting of a tumor, FNA should be repeated so that an important component of the diagnosis is not missed.

Esophageal atresia: tragedies and triumphs over two decades in a developing country.

During a period of two decades, 1972-1991, 303 patients with esophageal atresia and/or tracheo-esophageal fistula were treated at the department of Pediatric Surgery, SMS Medical College, Jaipur, India. More than half of our patients fall into Waterston's risk category C. To evaluate the improvement in our results, the patients were divided into four phases of 5 years each. Over the period of observation, the incidence of new cases as well as the number of associated anomalies has considerably increased. With time, more patients are being diagnosed and referred early for treatment. This has resulted in more patients being treated by primary repair with or without a gastrostomy. Extrapleural approach and single layer end-to-end anastomosis has been practised in all cases. Results have shown a steady improvement primarily because of early recognition, improved perioperative care and newer antibiotics. Although postoperative pulmonary complications and anastomotic leak have shown a decreasing trend, but the presence of severe associated anomalies, pulmonary complications and sepsis still remain the major killers in our set-up. Although our results may not match those from the developed countries, we have still come a long way, improving our overall survival rate of 4.6% in 1972-76 to 45.7% in 1987-91. Through the present article, we wish to highlight the practical problems faced in the management of these patients in underprivileged developing countries.

Pulmonary blastoma.

A three-and-half-year-old boy presented with recurrent chest infections, fever and weight loss of three month duration not responding to antibiotics. The chest X-ray and CT scan revealed a large well-circumscribed mass in right upper thorax with collapse of right upper lobe. A preoperative diagnosis could not be made even after fine needle aspiration cytology. Thoracotomy and right upper lobectomy was done and the biopsy report was an inflammatory pseudotumor. The child remained well for three months after which his symptoms and the mass recurred. The histopathology slides were reviewed and revealed a biphasic malignant tumor suggestive of Pulmonary Blastoma (PB). Patient received four cycles of chemotherapy followed by re-exploration. The recurrent tumour could only be excised partly and the child succumbed to persistent shock postoperatively. The final histopathological diagnosis was confirmed as PB. Primary pulmonary neoplasms in children are rare and of these PB which is even rarer, constitutes less than 15%. The report highlights that the lack of familiarity with this entity still causes error in the diagnosis of PB.

Mediastinal enteric duplication cyst containing aberrant pancreas.

OBJECTIVE: The purpose of the study is to report a unique association of clinical and pathological findings in a neonate. Foregut enteric duplication cysts--rare developmental anomalies that are associated with midline vertebral fusion anomalies. METHODS: We had a neonate with foregut duplication cyst who presented at birth with respiratory distress. The child also had associated communicating hydrocephalus. The patient underwent excision of the duplication cyst along with a ventriculo-peritoneal shunt. RESULT: The excised specimen revealed a duplication cyst lined by aberrant pancreatic tissue. CONCLUSION: The present case demonstrates histologically the presence of both pancreatic and gastric tissue.

Immature gastric teratoma.

Gastric teratoma, a very rare tumor of infancy is essentially benign. Immature (embryonic) elements have been described in only three of the sixty-six cases described previously. This report concerns with one "immature" gastric teratoma seen in infancy.

Dengue viral infection.

PIP: Dengue viral infection produces a spectrum of disease. For example, mild dengue disease is characterized by biphasic fever, myalgia, arthralgia, leukopenia, and lymphadenopathy, while dengue hemorrhagic fever is an often fatal disease characterized by hemorrhages and shock syndrome. The disease, especially in its severe form, is seen more often among children than among adults. With focus upon India, dengue's etiology, epidemiology, pathology, pathogenesis of dengue hemorrhagic fever, clinical manifestations of both the mild and severe forms of dengue viral infection, diagnosis, differential diagnosis, treatment, prevention, and prognosis are discussed.^ieng

Inguinal herniotomy in children: a decade's experience.

We reviewed our experience with 1369 inguinal herniotomies in 1340 children performed over the last one decade. Different grades of surgeons were assigned work according to the complexity of cases. Except for the minor scrotal hematoma, other complications were hardly seen. Recurrences were seen in only 2 cases. Careful training and supervision of junior staff in the technique of inguinal herniotomy has led to results that compare favorably to those of specialized units in developed countries.

Abdominal tuberculosis in children: experience over a decade.

This presentation deals with 110 surgically proven cases of abdominal tuberculosis in the pediatric age group. The protean clinical profiles and complications of the disease entity made the clinical diagnosis difficult; the investigations were also found non-pathognomonic. The most common type of pathology seen was adhesive variety followed by nodal type. Strictures of the small bowel were uncommon and hyperplastic variety was rarely seen in the present series. The pathogenesis relating to various varieties has been suggested based on the vast experience from a single institution.

Teratomas in pediatric age group: experience with 75 cases.

The clinicopathological features of 75 children under the age of 12 years with teratomas are reviewed. Tumors arose in the following anatomic sites: sacrococcygeum (n = 49), ovary (n = 10), Testis (n = 5), oral cavity (n = 3), retroperitoneum (n = 2) and others (n = 6). Fifty five (74%) presented within the first year of life. Excluding the gonadal tumors, male-female ratio was 2:5. Majority of the tumors had only mature tissues. Such patients and those 9 patients in whom the histology was not specified, underwent excision alone and had 95% early survival rates. Five patients had admixture of mature and immature tumors. Nine patients had malignant tissues. Germ cell tumors containing only malignant component, but no mature or immature teratomatous tissues were excluded from the series. The patients with immature and malignant tissues underwent multimodal therapy including surgical excision, multiagent chemotherapy (VAC regimen) and at times radiotherapy. Mortality in patients with immature and malignant teratomas was 20 and 66.7%, respectively. Besides histology, the only factor which affected prognosis, especially in case of sacrococcygeal teratomas was the age at the time of presentation. Our experience highlights the importance of early recognition and complete surgical excision of teratomas in the pediatric age group.

Pattern of childhood trauma. Indian perspective.

Although infectious diseases are still the chief cause of death in children in a developing country like ours, but a definite increase in incident and related mortality due to trauma has been noted in the last decade. The problems relating to pediatric trauma are peculiar to our setup and differ considerably to the severe multiple organ trauma met with in the high velocity vehicular accidents, seen in developed Western countries. The present study identifies patterns of childhood trauma from our region. It comprises 2100 patients admitted over a 3 years period to Pediatric Surgical Unit. Cranial injuries were the most commonly encountered injuries followed by the abdominal and skeletal injuries. Fall from the house roofs is the commonest mode of injury, although road traffic accidents are also recognized to be on the increase. The overall mortality was 7.7%; its chief determinant being the presence of severe head injury. A few important epidemiological factors involved were identified.

Antenatally diagnosed wilms' tumour.

Wilms' tumour (WT) is seldom seen in a neonate and prenatal diagnosis is rare. We present a case of antenatally diagnosed left sided WT with features of hydrops foetalis in a girl baby. Emergency LSCS was done at 34 weeks of gestation for foetal distress. Patient required mechanical ventilation for birth asphyxia and congestive cardiac failure. After stabilization, gross total resection of the tumour was done on day 4 of life. Histopathology HPE confirmed classical WT (stage I). Unfortunately, the patient died on the second postoperative day despite all supportive measures.