Outcome of pediatric germ cell tumor with comparison of carboplatin and cisplatin based regimens: A 10-year analysis.

Carboplatin is being advocated more frequently for treatment of childhood germ cell tumors (GCT), due to less long-term toxicity, and demonstrable equivalence in outcome as compared to cisplatin. This analysis presents the survival of GCT in a low middle-income country and compares two different chemotherapeutic regimens. A retrospective analysis of patient case records was carried out over 10-years (January 2007-December 2016). Chemotherapy regimen used was bleomycin, etoposide, and cisplatin (PEb) for initial 6-½ years and carboplatin, etoposide, and bleomycin (CEb) subsequently. Ninety patients with GCT were treated over 10-years. Malignant GCT was diagnosed in 69 (77%) patients, with 21(23%) having teratoma. The chemotherapy protocol was PEb in 38 (42%), CEb in 28 (31%) patients, while 24 patients were treated with surgery only. Stage 4 tumor was observed in 19 (21%) patients. Relapse or disease progression was seen in 11(12%). Overall and event-free survival at 5-years for the entire cohort was 77% and 73%, being similar with PEb (OS:77%; EFS:72.5%) vs. CEb (OS:69%; EFS: 69%). Significantly better overall survival was noted for patients with gonadal GCT) and non-stage 4 disease, while event-free survival was significantly better in patients with non-stage 4 disease. The chemotherapeutic regimen (PEb vs. CEb), very high AFP (value ≥10,000 IU/L), and risk stratification (low, intermediate, or high-risk disease) did not affect survival significantly. Carboplatin-based strategy was equivalent in our cohort to cisplatin-based strategy, and could be used safely in the LMIC set-up. The overall survival is suboptimal, with delayed presentation, abandonment, and relapse being barriers to survival.

Challenges in the management of localized Ewing sarcoma in a developing country.

Survival in pediatric Ewing sarcoma (ES) lags in low- and middle-income countries (LMICs). This study analyzed factors contributing to a lower outcome in an LMIC center. A retrospective case review of children with localized ES treated from January 2011 till December 2017 was performed. Neoadjuvant chemotherapy with alternating cycles of vincristine, doxorubicin, cyclophosphamide; and ifosfamide, etoposide was administered 3-weekly for 48 weeks. Reassessment was planned for week 12, followed by local therapy (surgery/radiotherapy or both) tailed by adjuvant chemotherapy. Forty-eight patients with mean age 8 years (range: 0.7-14) were evaluated. Extremity and central axis tumors were seen in 25 (52%) and 23 (48%) patients. Three patients died of neutropenic sepsis and five abandoned therapy. Local therapy included primary surgery, radiotherapy and a combination of surgery and radiotherapy in 7 (16%), 20 (45%) and 17 (39%) patients. The 3-year event-free survival (EFS) and disease-free survival (DFS) for the cohort were 47.7 ± 11% and 57.6 ± 11.2%. Time to local therapy >16 weeks was associated with inferior DFS vs. local therapy administered within 16 weeks [46.6 ± 12.4 vs. 63.9 ± 19.4, p=.046]. Older age, axial site, large size and incomplete surgical resection did not predict relapse/progression. Patients who received wide local excision, as local therapy, had 100% DFS. Coordinated efforts to ensure timely therapy can improve outcome in pediatric ES. Abandonment and treatment-related mortality (TRM) are additional challenges that need to be tackled in LMICs.

Modified Duhamel's Two-Staged Procedure for Hirschsprung's Disease: Further Modifications for Improved Outcomes.

AIMS AND OBJECTIVES: We studied the short- and long-term outcomes and quality of life (QOL) in patients undergoing a two-staged modified Duhamel's procedure for Hirschsprung's disease. MATERIALS AND METHODS: Patients who had undergone this modified procedure, with initial Hartmann's procedure based on contrast enema, followed by bowel preparation and low colo-anal anastomosis below the dentate line were included. The patient who underwent this procedure over 10 years with a minimum 2-year follow-up were analysed based on an interview-based questionnaire. RESULTS: Of the 152 patients, 69 responded. Mean age at the time of interview was 7.72 ± 3.04 years with mean follow-up of 4.9 years (2-11 years). Perineal excoriation and soiling was present in 60.9% and 36.2% of patients initially which reduced to 0 and 4.3% by the end of 2 years. In the first 6 months, postoperative period, 15.9% of patients had constipation and 78.2% had altered stool consistency. Good fecal continence score was present in 97.1% of patients in the long term. About 95.4% had good QOL scores. There was no mortality in this series. CONCLUSIONS: Although short-term outcomes showed altered bowel function, soiling, and perineal excoriation, this improved significantly in the long term, with good QOL scores in the majority.

Clinical Presentation and Surgical Management of Neonatal Tumors: Retrospective Analysis.

AIMS: Neonatal tumors (NTs) include a group of diverse neoplasms. In this study, we reviewed our data for clinical presentations, management options, and outcome. MATERIALS AND METHODS: All patients from 0- to 1-month age presenting with solid tumors, from 2006 to 2018 were studied. The gender, presentation, location, type of tumor, and management were analyzed. The final diagnosis was made with histopathology in all cases. Hemangiomas and lymphangiomas were excluded from the study. RESULTS: A total of 32 neonates were studied. The most common tumor was sacrococcygeal teratoma (SCT,16) followed by teratoma at other sites including two cases of fetus-in-fetu, soft-tissue sarcoma (STS, 4), mesenchymal hamartoma (2), hemangioendothelioma (2), and other rare tumors. Three tumors were diagnosed antenatally; of whom, two were neither visible externally nor palpable. Complete surgical excision was done for all except in a case of ovarian cyst where near-total cystectomy was done. No patient received chemotherapy or radiotherapy. Six patients had postoperative complications, including two who had local recurrence requiring excision. There was one mortality. All the other patients are doing well during follow-up. CONCLUSION: NTs have varied presentations. SCT and STS were the most common benign and malignant tumor, respectively. Early diagnosis and complete surgical excision are often curative for all, regardless of the pathology with the minimal role of chemotherapy or radiotherapy.

Pediatric Trauma: Management and Lessons Learned.

AIM: The aim is to prospectively study 125 trauma patients admitted in the pediatric surgery ward in our institute. MATERIALS AND METHODS: Pediatric patients admitted in the ward after initial resuscitation in the triage room were included. Isolated neurosurgical and orthopedic injuries were excluded. X-ray cervical spine, hip, and chest and a focused assessment with sonography in trauma ultrasound were done for all patients. Computed tomography of the abdomen or chest was done where relevant. Injury profile and surgical intervention when needed were analyzed. RESULTS: Road traffic accidents and fall from height caused 73.6% of the injuries. School-going children were most commonly affected (60.8%). Distinctive injuries were noted such as abdominal wall hernias and delayed bladder perforation. All solid organ injury irrespective of grade treated conservatively. Forty percent of the children required surgical intervention. Five patients after laparotomy were found to have surgical conditions unrelated to trauma, whereas another 14 required delayed surgery. Five patients had injuries secondary to sexual abuse. All except two patients were discharged in a satisfactory condition and are doing well in the follow-up. CONCLUSION: In spite of extensive injuries and the need for multiple surgeries, children with trauma have a good prognosis. Close observation during admission and also in follow-up are essential, as many patients may require delayed surgery ≥1 week from injury.

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.

Consensus on the Management of Posterior Urethral Valves from Antenatal Period to Puberty.

The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.

Splenic Hemangiomatosis: An Uncommon Vascular Lesion in an Infant.

BACKGROUND: Vascular tumors of spleen represent a wide spectrum of lesions, ranging from benign to highly aggressive neoplasms. Among the benign tumors, localized hemangiomas are the most frequently encountered. Splenic hemangiomatosis is seldom seen especially in the pediatric population with anecdotal cases described in the literature. CASE REPORT: We present of 6-month-old child presenting with abdominal distention. The resected spleen was totally replaced with a hemangiomatous lesion. There were no other hemangiomatous foci outside the spleen. CONCLUSIONS: Hemangiomatosis limited to the spleen can occur in a child, and the present case demonstrates its characteristic histological features.

Abdominoperineal Pull-through with Simultaneous Extravesical Detrussoraphy: An Alternative Surgical Technique for Congenital Pouch Colon with High-grade Vesicoureteric Reflux.

Congenital pouch colon (CPC) is frequently associated with vesicoureteric reflux (VUR). These patients require long-term antibiotic prophylaxis and/or an additional surgical intervention for the management of the refluxing system. We propose a single-stage alternative approach in these patients. Two patients diagnosed to have CPC underwent pouch excision and an end colostomy at birth. Further evaluation revealed high-grade reflux in both the patients. At 6 months of age, definitive abdominoperineal pull-through (APPT) surgery along with extravesical detrusorrhaphy was performed. In the follow-up at 1 year, they are thriving well with no urinary complaints. Micturating cystourethrogram revealed complete resolution of VUR. This approach takes the advantage of the anesthesia for APPT and offers a relatively simple and quick solution for the refluxing system, thus, enabling the stoppage of antibiotic prophylaxis and obviating the need for a future endoscopy/surgery.

Juvenile Xanthogranuloma: Presenting as an Isolated Renal Involvement.

Juvenile xanthogranuloma (JXG) is a histiocytic disorder of childhood that mostly affects the skin although extracutaneous and visceral manifestations are well documented in English literature. The renal involvement is less commonly documented as a manifestation of xanthogranuloma and the reported cases occur in conjunction with cutaneous xanthogranuloma, especially in association with multiple skin lesions. We present a case of isolated renal JXG presenting as a renal mass with perinephric soft tissue and muscle involvement but without any cutaneous manifestation. This is the first case report of renal JXG without cutaneous involvement, to the best of our knowledge.

Role of feeding jejunostomy in major anastomotic disruptions in esophageal atresia: A pilot study.

AIMS: To investigate the role of feeding jejunostomy (FJ) in patients of esophageal atresia with anastomotic leak (AL) to decrease the degree of gastroesophageal reflux (GER) and its effect on anastomotic healing. MATERIALS AND METHODS: Twenty neonates, with major AL and severe GER after primary repair were managed with decompressing gastrostomy and transgastric FJ and analyzed prospectively. RESULTS: Male to female ratio was 1.7:1. Mean birth weight was 2.2 kg. Anastomotic gap ranged from 0 to 4 cm. The amount of leak was more than 20% of nasogastric feeds. Gastrostomy and FJ was done on an average of the 12(th) postoperative day, after observing the general condition, chest tube output, lung expansion, and ventilatory requirement. There was a drastic reduction in chest tube output and lung expanded in all patients. Average hospital stay was 36 days (8-80 days). Sixty percentage patients were discharged successfully on FJ. Esophagogram demonstrated healing and leak free patency after an average of 1.5 months. GER was noted in seven patients, four developed stricture, and one had pseudodiverticulum in follow-up. CONCLUSION: Decompressing gastrostomy and FJ can be an alternative to managing major ALs. It helps in healing of anastomotic dehiscence and in preserving the native esophagus.

Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: Basics of technique and its nuances.

AIM: To review the technique of thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (TREAT) and results reported in literature and with authors' experience. PATIENTS AND METHODS: The technique of TREAT was reviewed in detail with evaluation in patients treated at authors' institution. The patients were selected based on selection criteria and were followed postoperatively. The results available in literature were also reviewed. RESULTS: A total of 29 patients (8 females) were operated by TREAT. Mean age was 2.8 days (range 2-6 days). Mean weight was 2.6 kg (range 1.8-3.2 kg). There was a leak in four patients, and two patients had to be diverted. They are now awaiting definitive repair. Twenty-one patients have completed a mean follow-up of 1.5 years and are doing well except for two patients who had a stricture and underwent serial esophageal dilatations. The results from current literature are provided in tabulated form. CONCLUSIONS: TREAT is now a well-established procedure and currently is the preferred approach wherever feasible. The avoidance of thoracotomy is a major advantage to the newborn and is proven to benefit the recovery in the postoperative patient. The technique demonstrated, and the tweaks reported make the procedure easy and is helpful to beginners. The outcome is very much comparable to the open repair as proven by various series. Various parameters like leak rate, anastomotic stricture are the same. The outcome is comparable if you TREAT these babies well.

Antifungal properties of native Trichoderma isolates against Sclerotium rolfsii and Pythium aphanidermatum infecting tobacco.

Isolates of Trichoderma native to tobacco rhizosphere, were evaluated for their antifungal properties against Sclerotium rolfsii and Pythium aphanidermatum, fungal pathogens causing collar rot and damping off diseases in tobacco. Isolate TvJt1 showed maximum HCN production (0.14 OD) followed by ThHt1 and minimum by isolates ThJnt and ThRt1 (0.08 and 0.09 OD). Indole acetic acid (IAA) production among the isolates varied from 6.48 to 14.82 pg ml(-1). Isolate ThJO1 produced maximum siderophore (13.48 m mol ml(-1), whereas isolate TvJt1 produced minimum content (6.24 m mol ml(-1)). Isolate ThJt1 showed maximum and significantly higher chitinase activity (62.12 p kat m;(-1)) as compared to other isolates, followed by isolate TvHt2. Isolate TvHt2 showed maximum and significantly higher ß-1, 3-glucanase activity (9.94 n kat ml(-1)) when compared to all other isolates. Isolate ThJt1 showed second highest activity of ß-1, 3-glucanase (6.75 n kat/ml). In dual culture, inhibition of mycelial growth of Pythium by isolate TvHt2 was maximum (72.45%) followed by ThJt1 whereas inhibition of mycelial growth of S. rolfsii by these two isolates was at a par (78.36% and 76.17%) and significantly higher than some isolates. Isolates TvHt2 and ThJt1 showed maximum and significantly higher inhibition of Pythium and Sclerotium growth (80.24% and 76%) by production of non-volatiles. Inhibition of Pythium and Sclerotium growth by production of volatile compounds by Trichoderma isolates ranged from 54.36 to 72.6% and 66.24 to 84.24% respectively. Hence, further investigation with potential isolates ThJt1 and TvHt2 in tobacco nursery may help to explore these bioagents for control of collar rot and damping off diseases in tobacco.

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.

Nephrectomy in children: Comparison of stress response to laparoscopic and open methods.

AIM: To evaluate and compare the extent of surgical stress following laparoscopic nephrectomy (LN) and open nephrectomy (ON) in children. MATERIALS AND METHODS: Twenty consecutive children undergoing nephrectomy were randomized to LN or ON groups. Acid-base balance, blood glucose, acute phase proteins (C-reactive protein [CRP]) and inflammatory markers (interleukin-6 [IL-6]) were measured pre-operatively, as well as 4 and 24 h after surgery. The differences between the two groups were analyzed statistically (significance value for P < 0.05). RESULTS: The overall acid base status was more stable in LN. The fall in pH 4 h after surgery was more in ON (P = 0.440) and the difference in pH in ON 4 h and 24 h post-operatively was statistically significant (P = 0.002). In LN, significant difference was found in the base excess mean pre-surgery (mean -3.280 mEq/L) and 4 h post-surgery (mean -7.480 mEq/L) (P = <0.05), as well as between 4 h and 24 h after surgery (mean -2.660 mEq/L) (P = 0.011). The acute rise in CRP 24 h post-operatively in the ON (88.972 mg/L) was significantly higher when compared to both the pre-operative and 4 h post-operative values (P < 0.05). This rise was however, not statistically significant when compared to the 24 h post-operative value in LN (46.399 mg/L) (P = 0.062). The rise in IL-6, 24 h post-procedure in LN (mean 44.444 pg/ml) was statistically lower than that in the open group (mean 343.333 pg/ml) (P = 0.041). CONCLUSIONS: The stable acid-base status and lesser rise of CRP and IL-6 in LN lead to the conclusion that surgical stress caused by LN is less than ON.

Relationship of ß-catenin and postchemotherapy histopathologic changes with overall survival in patients with hepatoblastoma.

AIMS: Histopathologic spectrum and expression of ß-catenin were analyzed in patients with hepatoblastoma, diagnosed over a period of 14 years. These were correlated with the survival outcome. The morphologic features subsequent to chemotherapy were also analyzed. METHODS AND RESULTS: Histomorphologic features were studied on paraffin-embedded sections. There were 24 cases with 15 fetal, 4 embryonal, 4 macrotrabecular, and 1 of small cell subtype. Follow-up was available in 20 cases (mean = 16.8 mo). ß-catenin immunostaining performed by indirect immunoperoxidase method revealed 14 cases with nuclear and 10 cases with cytoplasmic positivity. Statistical analysis revealed no significant correlation between morphologic subtype and survival. Significant difference in survival was noted with respect to tumor stage, mitotic index, and ß-catenin staining pattern. Cases with nuclear expression had a mean survival of 71.54 ± 8.1 months in comparison with 14.71 ± 6.5 months in cases with cytoplasmic expression. Besides osteoid and cartilage formation, interesting postchemotherapy findings were the presence of tumoral maturation, hepatocellular carcinoma-like areas, peliotic-like foci, and "glomeruloid clusters." CONCLUSIONS: Nuclear ß-catenin expression is not a poor prognostic factor and this might be indicative of different genetic alterations in hepatoblastoma in the Indian subcontinent. There was no significant correlation between histologic subtype and osteoid differentiation with survival. The histopathologic changes observed were peliotic-like areas, tumoral maturation, hepatocellular carcinoma-like changes, and glomeruloid clusters besides the well-established features of osteoid differentiation after chemotherapy.

Quality of life of very young spina bifida patients after initial surgical treatment.

OBJECTIVE: The objectives of this study were to assess the quality of life (QOL) of operated patients with spina bifida (SB) in their initial years and to elucidate the factors affecting the QOL and the timing of proposed interventions. PATIENTS AND METHODS: We studied 18 patients operated on for SB, aging 3 years or more at the time of assessment. The quality of life score was calculated for all the patients by the parental-administered questionnaire method. The impact of all these factors on the social and school lives of these patients was studied. RESULTS: Eleven patients were having neurogenic bladder. Thirteen patients were having stool problems which ranged from mild to severe constipation and soiling. Five patients were having lower limb deformities interfering with normal ambulation. Ten patients were able to walk on their own without any help, four patients were using either crutches or help of the caregiver for ambulation while the remaining four patients were non-ambulatory. Twelve patients were attending school. The calculated QOL scores ranged from 2 to 15. The Barthel Index (BI) ranged from 15 to 100. The QOL questionnaire score and BI were showing significant positive correlation. CONCLUSIONS: The patients operated on for SB commonly have the neurological sequelae. The QOL score identifies the important problems in the initial years after surgery which can help in initiating and maintaining rehabilitative services in these patients. The treatment of urinary and fecal incontinence very early in life will allow these children to have a normal social and school life.

A novel variant of esophageal atresia with tracheo-esophageal fistula with a crossed-over proximal esophageal pouch: a diagnostic dilemma.

Diagnosis of esophageal atresia (EA) with tracheo-esophageal fistula (TEF) can be easily done in newborns with symptoms of excessive salivation and failure to pass infant-feeding tube (IFT) into the stomach. There are many reports in the literature which describe the diagnostic pitfall of this IFT test. We describe a case of a neonate with EA with TEF in which the upper atretic esophageal pouch had grown long enough to reach up to the level of diaphragm, thus allowing the IFT to go up to significant distance from the alveolus giving the radiograph as if the tube is in the upper part of stomach, thus creating a diagnostic dilemma.

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.

Revisiting the histopathologic spectrum of congenital pulmonary developmental disorders.

A retrospective analysis of lobectomy, pneumonectomy, cystectomy specimens (10 years) and stillbirth/neonatal autopsies (5 years) was carried out to analyze the histologic spectrum of congenital pulmonary developmental disorders/malformations. The autopsy data was analyzed to identify the lesions that are more diffuse and not amenable to surgical management. A total of 166 cases of pulmonary developmental disorders/ malformation were found. Out of 2,155 stillbirth/neonatal autopsies, there were 105 cases of pulmonary hypoplasia, 2 cases of congenital pulmonary lymphangiectasis, 2 cases of extralobar sequestration, and 3 cases of congenital pulmonary airway malformation (CPAM). Among the surgical specimens, there were 21 cases of CPAM, 11 cases of intralobar sequestration, 5 cases of congenital lobar emphysema and 17 cases of bronchogenic cyst. All these malformations have been described in reference to the latest updated classification of developmental disorders/malformations.