VOLUME 02, 1994

VOL 02 NO 01 JANUARY 1994

Fetal Neuroblastoma

Routine use of prenatal sonography will increase the incidental diagnosis of fetal neuroblastoma. Most are detected during the third trimester of pregnancy as cystic/solid suprarenal mass. The tumor does not cross the placenta but can metastize in utero to the fetal liver or placenta. After birth 50% of babies have elevated HMA/VMA levels. Most enjoy improved survival due to: lower stage of disease, cystic variety (in -situ), and higher stage IV-S (which has been associated with spontaneous inmuno-regression. Adverse biologic features are: diploid tumor karyotype (cytometry) and amplify N-myc oncogene. They can be very difficult to differentiate from neonatal adrenal hemorrhage; T2 of MRI can be of help. Are they neuroblastoma in-situ, and will they regress spontaneously without treatment are question waiting answer in the near future.

1- Pley EA, Wouters EJ, de Jong PA, Tielens AG, Clermonts GJ: The sonographic imaging of fetal adrenal neuroblastoma; a case report. European J Obst, Gyn, & Repr Biol 31(1):95-9, 1989
2- Hosoda Y, Miyano T, Kimura K, Oya T, Ishimoto K, Tanno M, Takeuchi H: Characteristics and management of patients with fetal neuroblastoma. J Pediatr Surg  27(5):623-5, 1992
3- Forman HP, Leonidas JC, Berdon WE, Slovis TL;  Wood BP;  Samudrala R: Congenital neuroblastoma: evaluation with multimodality imaging. Radiology 175(2):365-8, 1990
4- Anders D, Kindermann Gm, Pfeifer U: Metastasizing fetal neuroblastoma with involvement of the placenta simulating fetal erythroblastosis. Report of two cases.

J Pediatr 82(1):50-3, 1973

Prenatal CCAM

Congenital cystic adenomatoid malformation is a lung bud lesion characterize by dysplasia of respiratory epithelium caused by overgrowth of distal bronchiolar tissue. Prenatally diagnosed CCAM prognosis depends on the size of the lung lesion and can cause: mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and fetal hydrops (cardiovascular shunt). Classified in two types based on ultrasound findings: macrocystic (lobar, > 5 mm cysts, anechoic, favorable prognosis) and microcystic (diffuse, more solid, echogenic, lethal). Occurs as an isolated (sporadic) event with a low rate of recurrence. Survival depends on histology. Hydrops is caused by vena caval obstruction, heart compression and mediastinal shift. The natural history is that some will decrease in size, while others disappear. Should be follow with serial sonograms. Prenatal management for impending fetal hydrops has consisted of thoraco-amniotic shunts (dislodge, migrate and occlude), and intra-uterine fetal resection (technically feasible, reverses hydrops, allows lung growth). Post- natal management consist of lobectomy.

1- Revillon Y, Jan D, Plattner V, Sonigo P, Dommergues M, Mandelbrot L, Dumez Y, Nihoul-Fekete C: Congenital cystic adenomatoid malformation of the lung: prenatal management and prognosis. J Pediatr Surg 28(8):1009-11, 1993
2- Mashiach R, Hod M, Friedman S, Schoenfeld A, Ovadia J, Merlob P: Antenatal ultrasound diagnosis of congenital cystic adenomatoid malformation of the lung: spontaneous resolution in utero.J Clin Ultrasound 21(7):453-7, 1993
3- Taguchi M, Shimizu K, Ozaki Y, Kubota T, Aso T: Prenatal diagnosis of congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther 8(2):114-8, 1993
4- Adzick NS, Harrison MR: Management of the fetus with a cystic adenomatoid malformation. World J Surg 17(3):342-9, 1993

5- Adzick NS, Harrison MR, Flake AW, Howell LJ, Golbus MS, Filly RA: Fetal surgery for cystic adenomatoid malformation of the lung. J Pediatr Surg 28(6):806-12, 1993

Fetal Intestinal Obstruction

The fetal gastrointestinal tract (foregut, midgut and hindgut) undergoes ventral folding between 24-28 days' gestation. By the 5- 6th wk the stomach rotates to the right and the duodenum occludes by cell proliferation. Recanalization of the duodenum occurs around the 8th wk. The midgut rotation takes place during the 6-11th wk and the final peritoneal closure by 10th wk. The fetal GI tract begins ingestion and absorption of amniotic fluid by the 14th wk. This fluid contributes to 17% effective nutrition; proximally obstructed gut can cause growth retardation. Fetal intestinal obstruction is caused by: failure of recanalization (duodenal atresia), vascular accidents (intestinal atresias), intrauterine volvulus, intussusception, or intraluminal obstruction (meconium ileus). Esophageal obstruction causes polyhydramnios, absent visible stomach and is related to tracheo-esophageal anomalies. Duodenal obstruction seen as two anechoic cystic masses is associated to aneuploidy (trisomy 21) and polyhydramnios. Jejuno- ileal obstruction produces dilated anechoic (fluid-filled) serpentine masses and bowel diameter of 1-2 cm. Large bowel obstruction is most often caused by meconium ileus, Hirschsprung's disease or imperforate anus. The colon assumes a large diameter and the meconium is seen echogenic during sonography. In general the method of delivery is not changed by the intrauterine diagnosis of intestinal obstruction. Timing can be affected if there is evidence of worsening intestinal ischemia (early delivery recommended after fetal lung maturity).

1- Knochel JQ, Lee TG, Melendez MG, Henderson SC: Fetal anomalies involving the thorax and abdomen. Radiol Clin North Am 20(2):297-310, 1982
2- Ewer AK, McHugo JM, Chapman S, Newell SJ: Fetal echogenic gut: a marker of intrauterine gut ischaemia? Arch Dis Child 69(5 Spec No):510-3, 1993
3- Touloukian RJ: Diagnosis and treatment of jejunoileal atresia. World J Surg 17(3):310-7, 1993
4- Grosfeld JL, Rescorla FJ: Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 17(3):301-9, 1993
5- Weissman A, Goldstein I: Prenatal sonographic diagnosis and clinical management of small bowel obstruction. Am J Perinatol 10(3):215-6, 1993
6- Mandell J, Lillehei CW, Greene M, Benacerraf BR: The prenatal diagnosis of imperforate anus with rectourinary fistula: dilated fetal colon with enterolithiasis [see comments] J Pediatr Surg 27(1):82-4, 1992
7- Hertzberg BS, Bowie JD: Fetal gastrointestinal abnormalities. Radiol Clin North Am 28(1):101-14, 1990

8- Langer JC, Adzick NS, Filly RA, Golbus MS, deLorimier AA, Harrison MR: Gastrointestinal tract obstruction in the fetus. Arch Surg 124(10):1183-6; discussion 1187, 1989

VOL 02 NO 02 FEBRUARY 1994

Meconium Ileus

Meconium Ileus (MI) is a neonatal intraluminal intestinal obstruction associated with Cystic Fibrosis (10-20%). The distal ileum is packed with an abnormally thick, viscous, inspissated meconium. The meconium has a reduced water content the result of decreased pancreatic enzyme activity and a prolonged small bowel intestinal transit time. MI can be classified as simple or complicated. Simple MI appears in the first 48 hrs of life with abdominal distension and bilious vomiting. Complicated MI is more severe (< 24 hrs) with progressive abdominal distension, respiratory distress, and peritonitis. X-Ray findings are: dilated bowel loops, absent air-fluid levels, "soap-bubble" granular appearance of distal ileum due to a mixture of air with the tenacious meconium. Therapy consists of Gastrografin enema for simple cases: hyperosmolar solution draws fluid to the bowel lumen causing an osmotic diarrhea. Operative therapy is reserved for failed gastrografin attempts and complicated cases (associated to volvulus, atresias, gangrene, perforation or peritonitis). Surgical procedures has included: ileostomy with irrigation, resection with anastomosis, and resection with ileostomy (Mikulicz and Bishop- Koop). Post-operative management includes: 10% acetylcysteine p.o., oral feedings (pregestimil), pancreatic enzyme replacement, and prophylactic pulmonary therapy. Long-term prognosis depends on the degree of severity and progression of cystic fibrosis pulmonary disease.

1- Rescorla FJ,Grosfeld JL: Contemporary management of meconium ileus. World J Surg 17(3):318-25, 1993
2- Kalayoglu M, Sieber WK, Rodnan JB, Kiesewetter WB: Meconium ileus: a critical review of treatment and eventual prognosis. J Pediatr Surg 6(3):290-300, 1971
3-  Littlewood JM: Cystic fibrosis: gastrointestinal complications. Br Med Bull 48(4):847-59, 1992
4- Fakhoury K, Durie PR,Levison H, Canny GJ: Meconium ileus in the absence of cystic fibrosis.Arch Dis Child 67(10 Spec No):1204-6, 1992
5- Docherty JG, Zaki A, Coutts JA, Evans TJ, Carachi R: Meconium ileus: a review 1972-1990. Br J Surg 79(6):571-3, 1992

6- Del Pin CA, Czyrko C, Ziegler MM, Scanlin TF, Bishop HC: Management and survival of meconium ileus. A 30-year review. Ann Surg 215(2):179-85, 1992

Alarming Hemangiomas

Hemangioma is the most common tumor of infancy characterized by proliferation of capillary endothelium. Natural history is of rapid post-natal proliferative growth (8-18 mo) to a slow but inevitable regression during the next 5-8 years (involutive phase). Most are small and harmless. Alarming hemangiomas are associated with heart failure and thrombocytopenic coagulopathy (Kasabach-Merritt syndrome). Management consist of: (1) high dose steroids (rate response 30-60%), (2) radiation therapy, (3) surgery, and recently (4) alpha-interferon. Alpha-interferon inhibits the proliferation of endothelial cells, smooth muscle cells and fibroblast. Minimal side effects such as fever, elevation of liver function tests and flu-like syndrome has been reported. It is given subcutaneously, early withdrawal can cause re-growth of the lesion and has been found successful for severe complicating hemangiomas.

1.  Fishman S J, Mulliken JB: Hemangiomas and Vascular Malformations of Infancy and Chidhood. Pediatric Clinics of North America.  40(6), 1177-1200, 1993
2.  Hatley RM, et al: Successful Management of an Inffant witha Giant Hemangioma of the Retroperitoneum and Kasabach-Merritt Syndrome Syndrome With Alpha-Interferon. J Ped Surg 28(10), 1356-59, 1993
3.  MacArthur CJ, et al: The Use of Interferon Alpha-2a for Life-Threatening Hemangiomas.  Arch Otolaryngol Head and Neck Surg 121:690-693, 1995
4.  Ohlms LA, et al: Interferon Alpha-2a Therapy for airway  Hemangiomas. Ann Otol Rhinol Laryngol 103(1), 1-8, 1994
5.  Ricketts RR, et al: Interferon Alpha-2a for the Treatment of Complex Hemangiomas of Infancy and Childhood. Ann Surg 219(6): 605-614, 1994

6- Soumekh B: Interferon Alpha Therapy for hemangiomas. Ann Otol Rhinol laryngol. 105(3): 201-205, 1996


Chronic Intestinal Pseudo-Obstruction (CIPO) is a rare disorder of intestinal motility in infants and children characterized by recurrent attacks of abdominal pain, distension, vomiting, constipation and weight loss in the absence of obvious mechanical lesions. The disease can be familial or sporadic. Suggested etiology is degeneration of enteric nervous or smooth muscle cells. The diagnosis is based an history, physical exam, radiographies and motility studies. X-Ray hallmarks are: absent strictures, absent, decreased or disorganized intestinal motility, and dilated small/large bowel loops. Associated conditions identified in 10-30% of patients are bladder dysfunction (megacystis) and neurological problems. Histologic pattern portrayed: myenteric plexus hyperplasia, glial cell hyperplasia, and small ganglion cells (hypoganglionosis). Management is primary supportive: intestinal decompression (NG), long-term TPN and antibiotic prophylaxis. Motility agents are unsuccessful. Venting gastrostomy with home parenteral nutrition has shortened the high hospitalization rate associated to this disease process. A similar condition can be seen in early fed prematures due to immaturity of intestinal motility.

1- Hyman PE, Di Lorenzo C, McAdams L, Flores AF, Tomomasa T, Garvey TQ 3d: Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction [see comments].  Amer J Gastroent.  88(6):832-6, 1993
2- Di Lorenzo C, Flores AF, Reddy SN, Snape WJ Jr,  Bazzocchi G,  Hyman PE: Colonic manometry in children with chronic intestinal pseudo-obstruction. Gut  34(6):803-7, 1993
3- Gil Vernet JM, Casasa JM, Boix Ochoa J, Salas A, Broto J, Marhuenda C: Intestinal dysmotility-pseudo-obstruction. [Spanish] Cirugia Pediatrica  5(2):87-95, 1992
4-  Peck SN, Altschuler SM: Pseudo-obstruction in children. Gastroenterology Nursing  14(4):184-8, 1992
5- Devane SP, Ravelli AM, Bisset WM, Smith VV, Lake BD, Milla PJ: Gastric antral dysrhythmias in children with chronic idiopathic  intestinal pseudoobstruction. Gut  33(11):1477-81, 1992
6- Navarro J, Sonsino E, Boige N, Nabarra B, Ferkadji L, Mashako LM, Cezard JP: Visceral neuropathies responsible for chronic intestinal pseudo-obstruction syndrome in pediatric practice: analysis of 26 cases. Journal of Pediatric Gastroenterology & Nutrition 11(2):179-95, 1990
7- Glassman M, Spivak W,Mininberg D, Madara J: Chronic idiopathic intestinal pseudoobstruction: a commonly misdiagnosed disease in infants and children. Pediatrics  83(4):603-8, 1989

8- Fonkalsrud EW, Pitt HA, Berquist WE, Ament ME: Surgical management of chronic intestinal pseudo-obstruction in infancy and childhood. Progress In Pediatric Surgery  24:221-5, 1989

VOL 02 NO 03 MARCH 1994

IH in Prematures

The incidence of inguinal hernia(IH) in premature babies (9-11%) is higher than full-term (3-5%), with a dramatic risk of incarceration (30%). Associated to these episodes of incarceration are chances of: gonadal infarction (the undescended testes complicated by a hernia are more vulnerable to vascular compromise and atrophy), bowel obstruction and strangulation. Symptomatic hernia can complicate the clinical course of babies at NICU ill with hyaline membrane, sepsis, NEC and other conditions needing ventilatory support. Repair should be undertaken before hospital discharge to avoid complications. Prematures have: poorly developed respiratory control center, collapsible rib cage, deficient fatigue-resistant muscular fibers in the diaphragm that predispose then to potential life-threatening post-op respiratory complications such as: need of assisted ventilation (most common), apnea and bradycardia, emesis, cyanosis and re-intubation (due to laryngospasm). Independent risk factors associated to this complications are (1) history of RDS/bronchopulmonary dysplasia, (2) history of patent ductus arteriosus, (3) low absolute weight (< 1.5 Kg), and (4) anemia (Hgb < 10 gm- is associated to a higher incidence of post-op apnea). Postconceptual age (sum of intra- and extrauterine life) has been cited as the factor having greatest impact on post-op complications. These observation makes imperative that preemies (with post conceptual age of less than 45 weeks) be carefully monitored in-hospital for at least 24 hours after surgical repair of their hernias. Outpatient repair is safer for those prematures above the 60 wk. of postconceptual age. The very low birth weight infant with symptomatic hernia can benefit from epidural anesthesia.

1- Rescorla FJ, Grosfeld JL: Inguinal hernia repair in the perinatal period and early infancy: clinical considerations. J Pediatr Surg 19(6):832-7, 1984
2- Harper RG, Garcia A, Sia C: Inguinal hernia: a common problem of premature infants weighing 1,000 grams or less at birth. Pediatrics 56(1):112-5, 1975
3-Gollin G, Bell C, Dubose R, Touloukian RJ, Seashore JH, Hughes CW, Oh TH, Fleming J, O'Connor T: Predictors of postoperative respiratory complications in premature infants after inguinal herniorrhaphy. J Pediatr Surg 28(2):244-7, 1993
4- Peutrell JM, Hughes DG: Ex-premature infants can safely have outpatient inguinal herniotomies [letter; comment]J Pediatr Surg 27(11):1487-8, 1992

5- Melone JH, Schwartz MZ, Tyson KR, Marr CC, Greenholz SK, Taub JE, Hough VJ: Outpatient inguinal herniorrhaphy in premature infants: is it safe? [see comments]J Pediatr Surg 27(2):203-7, 1992; discussion 207-8


The FDA has recommended labeling changes for the use of anectine (succinylcholine chloride- depolarizing muscle relaxant). Anectine should not be used for routine, elective pediatric surgery. A series of cardiac arrests and deaths (36 cases reported in the last three years) after the use of anectine in children with a previously undiagnosed myopathy has prompted this issue. Children with myopathies have increase sensitivity to succinylcholine developing rhabdomyolysis, hyperkalemia and cardiac arrest (mortality 55%). Healthy children undergoing surgery can have occult forms of myopathy that cannot be known beforehand. Otherwise, anectine is indicated in instances of rapidly securing the airway: emergency intubation, laryngospasm and full stomach.

1- O'Connor RL, Weinstock A: Further considerations on succinylcholine-induced cardiac arrest [letter; comment] Anesth Analg 76(5):1167-8, 1993

2- Rosenberg H, Gronert GA: Intractable cardiac arrest in children given succinylcholine [letter] [see comments] Anesthesiology 77(5):1054, 1992

Splenic Cysts

Splenic cysts in children are either considered true epidermal (congenital), pseudocysts (post-traumatic), or infectious (echinococcus) in etiology. They are rare, benign, solitary cysts often producing few symptoms. They may present as a palpable mass in the left side of the abdomen or during evaluation for another abdominal problem. Ultrasound (large unilocular sonolucent cyst) is the most important diagnostic method, and can be supplemented by CT-Scan. The lining of the cyst is a flattened endothelium surrounded by fibrous tissue. This mesothelium can produced carcinoembryonic antigen (CEA). Indications for surgery are: (1) risk of complications (rupture, bleeding), (2) size greater than 5 cm., (3) infectious etiology, and a (4) symptomatic child (pain, mass or splenomegaly). Their management formerly total splenectomy has changed to: interventional sonography with fluid aspiration (catheter placement), or partial splenic decapsulation (cystectomy); the result of recognition of the physiologic importance (hematologic and immunologic) of the spleen, together with the development of radiological imaging and operative surgery. Long term follow-up with radionuclide scans is recommended.

1-   Topilow AA, Steinhoff NG: Splenic pseudocyst a late complication of trauma.      Journal of Trauma 15:260-263, 1975
2-   Gray Robbins F, Yelin AE, Robert W, et al: Splenic epidermoid cysts. Ann Surg 187:231- 235, 1978
3-   Davis CE, Montero JM, Van Horn CN: Large splenic cyst. Ann Surg 173:686-692, 1971
4-   Martin JW: Congenital splenic cysts.  Am J Surg 96:302-308, 1958
5-   Edmond RE, Rochon BR, McPhail JF: Case report: a traumatic splenic pseudocyst historical review, diagnosis, and current mode of treatment.  The journal of Trauma 30:349-352, 1990
6-   Brown MF, Ross AJ, Bishop HC, et al: Partial splenectomy: the preferred alternative for the treatment of splenic cysts. J Ped Surg 24:694-696, 1989
7-   Millar JS: Partial excision and drainage of post-traumatic splenic cysts.  Br J Surg 89:477- 478, 1982
8-   Moir C, Guttman F, Jequier S, et al: Splenic cysts: aspiration, sclerosis, or resection.  J Ped Surg 24:646-648, 1989
9-   Khan AH, Bensoussan AL, Ouimet A, et al: Partial splenectomy for benign cystic lesions of the spleen.  J Ped Surg 21:749-752, 1986
10-  Soderstorm D: How to use cytodiagnostic spleen puncture. Acta Med Scand 199:1-5, 1972
11-   Quinn SF, vanSonnenberg E, Casola G, et al: Interventional radiology in the spleen. Radiology 161:289-291, 1986
12-  Goldfinger J, Cohen MM, Steinhardt MI, et al: Sonography and percutaneous aspiration of splenic epidermoid cyst. J Clin Ultrasound 14:147-149, 1986

13-  Touloukian RJ, Seashore JH: Partial splenic decapsulation: a simplified operation for splenic pseudocyst. J Pediatr Surg 22(2):135-7, 1987

VOL 02 NO 04 APRIL 1994

Bilateral Cryptorchidism

The undescended testis is the most frequent disorder of male sexual differentiation affecting 0.8% of boys by age one year. The etiology is varied but many cases represent disturbances of the hypothalamic-pituitary-gonadal axis and may represent a forme fruste of hypogonadotrophic hypogonadism. Bilateral cryptorchidism occurs in 10-30% of cases. Management consists of hormonal therapy a/o surgical orchiopexy. Human Chorionic Gonadotropin (HCG) is superior to Gonadotropin Releasing Hormone (GnRH) and placebo in the treatment of bilateral cases, with success rates of 23%. Regression analysis showed treatment is more successful the younger the child; after six months of age medical treatment with LHRH and HCG has produced descent in half managed cases. A recent study in young male rat revealed that the fertility defect is partially prevented by early orchiopexy and adjunctive hormonal therapy is probable of little additional benefit. Infertility is common in patients with history of bilateral cryptorchidism even after successful prepubertal orchiopexies, and azoospermia is present in 18% of bilateral cases pexed as children. Although the undescended testis experience a substantial increase of developing later malignancy, the absolute risk is so small that does not appear to justify special surveillance after surgery. Laparoscopy for the impalpable and bilateral undescended testis is of value to diagnosed testicular absence, identify intra-abdominal testis, and transect vessels for later Stephen-Fowler approach.

1- Snyder HM 3d: Bilateral undescended testes. Eur J Pediatr 152 Suppl 2:S45-6, 1993
2- Zerella JT, McGill LC: Survival of nonpalpable undescended testicles after orchiopexy. J Pediatr Surg 28(2):251-3, 1993
3- Diamond DA, Caldamone AA: The value of laparoscopy for 106 impalpable testes relative to clinical presentation. J Urol 148(2 Pt 2):632-4, 1992
4- P:eloquin F, Kiruluta G, Quiros E: Management of an impalpable testis: the role of laparoscopy. Can J Surg 34(6):587-90, 1991
5-  Lawson A, Gornall P, Buick RG, Corkery JJ: Impalpable testis: testicular vessel division in treatment. Br J Surg 78(9):1111-2, 1991

6- Cortes D, Thorup J: Histology of testicular biopsies taken at operation for bilateral maldescended testes in relation to fertility in adulthood. Br J Urol 68(3):285-91, 1991


Achalasia in children is an uncommon esophageal motor disorder distinguished by clinical, radiological and manometrics features. Incidence is estimated in 0.1 cases/year per 100,000 population under 14 years of age. Clinical presentation is characterized by progressive dysphagia, regurgitation, weight loss, chest pain and nocturnal cough. Infants exhibit failure to thrive. Diagnosis is established by barium swallow and confirmed by manometry and motility studies. Ba swallow shows' esophageal dilatation, motility alteration and a small caliber (bird-beak) cardio-esophageal junction. Manometry reveals elevated E-G sphincter pressure, non- peristaltic esophageal contraction and failed relaxation of lower esophageal sphincter upon swallowing. Videoflouroscopy can be of help in the screening of esophageal motors disorders. Esophageal pneumatic balloon dilatation is not an effective method of treatment in children due to the high rate of recurrence of symptoms. Primary therapy is surgical (Heller's modified esophagomyotomy), and results are similar after a transabdominal or thoracic approach. Many authors favor a concomitant antireflux procedures in these patients. Nifedipine can be of help as a short management in preparation for surgery. Long-term result presents' a connection between achalasia and malignant disease of the esophagus.

1- Emblem R, Stringer MD, Hall CM, Spitz L: Current results of surgery for achalasia of the cardia. Arch Dis Child 68(6):749-51, 1993
2- Allen KB, Ricketts RR: Surgery for achalasia of the cardia in children: the Dor-Gavriliu procedure. J Pediatr Surg 27(11):1418-21, 1992
3- Nihoul-Fekete C, Bawab F, Lortat-Jacob S, Arhan P: Achalasia of the esophagus in childhood. Surgical treatment in 35 cases, with special reference to familial cases and glucocorticoid deficiency association.
Hepatogastroenterology 38(6):510-3, 1991
4- Levine ML, Moskowitz GW, Dorf BS, Bank S: Pneumatic dilation in patients with achalasia with a modified Gruntzig dilator (Levine) under direct endoscopic control:results after 5 years. Am J Gastroenterol 86(11):1581-4, 1991
5-  Vane DW, Cosby K, West K, Grosfeld JL: Late results following esophagomyotomy in children with achalasia. J Pediatr Surg 23(6):515-9, 1988
6- Lemmer JH, Coran AG, Wesley JR, Polley TZ Jr, Byrne WJ: Achalasia in children: treatment by anterior esophageal myotomy (modified Heller operation). J Pediatr Surg 20(4):333-8, 1985
7- Buick RG, Spitz L: Achalasia of the cardia in children.Br J Surg 72(5):341-3 , 1985

8- Berquist WE, Byrne WJ, Ament ME, Fonkalsrud EW, Euler AR: Achalasia: diagnosis, management, and clinical course in 16 children. Pediatrics 71(5):798-805, 1983

Christmas Tree Deformity

This anomaly consists of a proximal high jejunal atresia with the blood supply of the distal jejunum and ileum supplied by the ileocolic and marginal vessels. The small intestine distal to the atresia is coiled in a spiral-like fashion around a rudimentary mesentery similar to an apple-peel or Christmas tree. The etiology is a intrauterine vascular accident (block) after the take off of the first branch of the superior mesenteric artery. Most are premature infants with associated malrotation. Management consist of tapering the proximal jejunum and anastomosing it to the distal bowel peel. Malabsorption can be a post-op problem until the blood supply and intestinal length improves. Parenteral nutrition has improved survival rates of this patients.

1- Zerella JT, Martin LW: Jejunal atresia with absent mesentery and a helical ileum. Surgery 80(5):550-3, 1976
2- Hull JD 3d, Kiesel JL, Proudfoot WH, Belin RP: Agenesis of the dorsal mesentery without jejunoilial atresia ("apple peel small bowel"). J Pediatr Surg 10(2):277-9, 1975
3- Weitzman JJ, Vanderhoof RS: Jejunal atresia with agenesis of the dorsal mesentery. With "Christmas tree" deformity of the small intestine. Am J Surg 111(3):443-9, 1966
4- Touloukian RJ: Diagnosis and treatment of jejunoileal atresia.
World J Surg 17(3):310-7, 1993
5- Turnock RR, Brereton RJ, Spitz L, Kiely EM: Primary anastomosis in apple-peel bowel syndrome. J Pediatr Surg 26(6):718-20, 1991

6- Ahlgren LS: Apple peel jejunal atresia. J Pediatr Surg 22(5):451-3, 1987

VOL 02 NO 05 MAY 1994

IA: Males Decisions

The most important decision in the initial management of Imperforate Anus (IA) male patient during the neonatal period is whether the baby needs a colostomy and/or another kind of urinary diversion procedure to prevent sepsis or metabolic derangements. Male patients will benefit from perineal inspection to check for the presence of a fistula (wait 16-24 hours of life before deciding). During this time start antibiotherapy, decompress the GI tract, do a urinalysis to check for meconium cells, and an ultrasound of abdomen to identify urological associated anomalies. Perineal signs in low malformations that will NOT need a colostomy are: meconium in perineum, bucket-handle defect, anal membrane and anal stenosis. These infants can be managed with a perineal anoplasty during the neonatal period with an excellent prognosis. Meconium in urine shows the pt has a fistula between the rectum and the urinary tract. Flat "bottom" or perineum (lack of intergluteal fold), and absence of anal dimple indicates poor muscles and a rather high malformation needing a colostomy. Patients with no clinical signs at 24 hours of birth will need a invertogram or cross-table lateral film in prone position to decide rectal pouch position. Bowel > 1 cm from skin level will need a colostomy, and bowel < 1 cm from skin can be approach perineally. Those cases with high defect are initially managed with a totally diverting colostomy. Diverting the fecal stream reduces the chances of genito-urinary tract contamination and future damage.

1- Pena A: Anorectal malformations. Semin Pediatr Surg 4(1):35-47, 1995
2- Pena A: Management of anorectal malformations during the newborn period. World J Surg 17(3):385-92, 1993
3- Pena A: Posterior sagittal approach for the correction of anorectal malformations. Adv Surg 19:69-100, 1986
4- Pena A: Surgical treatment of high imperforate anus. World J Surg 9(2):236-43, 1985

5- deVries PA, Pena A: Posterior sagittal anorectoplasty. J Pediatr Surg 17(5):638-43, 1982

Pyloric Stenosis Revisited

Reviewing 137 consecutive cases of Pyloric Stenosis during a 6.5 year period managed by the author at the region of Bayamon, and dividing the patients into the three most commonly encountered metabolic disturbances: hypochloremia, alkalosis and hypochloremic alkalosis, we found that the most important factor (p< 0.001) determining the probability of developing a metabolic disturbance was age at diagnosis; the older the child the higher the probability of developing hypochloremic alkalosis. Neither age, sex, race, birth weight, gestational age, firstborn, the presence of a palpable pyloric muscle (olive), or the hospital stay showed any association to the metabolic derangements characteristics of this condition. Post-op vomiting(22%) was a self-limiting event which resolved during the first 48 hrs after surgery upon resuming the oral feeding schedule. Persistent vomiting (4%) after myotomy is caused by an associated allergic gastropathy.


1- Lugo-Vicente HL, Torres-Rivera CN: Pyloric Stenosis: 137 Consecutive Cases. Boletin Asoc Med PR Vol 84 (10): 249-252, 1992

Vascular Access: Neonates

Vascular access is indicated to administer fluid, drugs, and nutrients in sick neonates. Peripheral venous cannulation is the initial preferred method. Complications associated to peripheral teflon catheter use are: extravasation with skin loss, phlebitis, and bacterial colonization. Within the first two weeks of life, umbilical vessel cannulation should be considered. Central venous catheter placement should be considered for long term delivery of TPN, antibiotics or venous sampling. Routes of access are: the external jugular veins, facial veins, internal jugular veins, the saphenous veins and the subclavian veins. Catheters are of silastic material (Broviac) or polyurethane. Complications associated to central vein catheters are:(1)sepsis- most common and serious, no other source of infection and positive blood culture. They are associated to fibrin sheath formation and thrombosis within the catheter. Most are managed with antibiotics, for fungi- catheter must be removed. (2) Mechanical- dislocation, occlusion, and breakage. (3) thrombosis- the result of hypercoagulability.

1- Reynolds J: Comparison of percutaneous venous catheters and teflon catheters for intravenous therapy in neonates. Neonatal Netw 12(5):33-9, 1993
2- Jones GR, Konsler GK, Dunaway RP, Lacey SR,Azizkhan RG: Prospective analysis of urokinase in the treatment of catheter sepsis in pediatric hematology-oncology patients.J Pediatr Surg 28(3):350-5, 1993; discussion 355-7
3- Salzman MB, Isenberg HD, Shapiro JF, Lipsitz PJ, Rubin LG: A prospective study of the catheter hub as the portal of entry for microorganisms causing catheter-related sepsis in neonates. J Infect Dis 167(2):487-90, 1993
4- Gauderer MW: Vascular access techniques and devices in the pediatric patient. Surg Clin North Am 72(6):1267-84, 1992
5- Garland JS, Dunne WM Jr, Havens P, Hintermeyer M, Bozzette MA, Wincek J,Bromberger T,Seavers M: Peripheral intravenous catheter complications in critically ill children: a prospective study. Pediatrics 89(6 Pt 2):1145-50, 1992

6- Mulloy RH, Jadavji T, Russell ML: Tunneled central venous catheter sepsis: risk factors in a pediatric hospital.  JPEN J Parenter Enteral Nutr 15(4):460-3, 1991

VOL 02 NO 06 JUNE 1994


Idiopathic Perforation of the Bile Ducts (IPBD) is the second most common cause of surgical jaundice restricted to the first three months of life (biliary atresia is first). The pathogenesis is unknown, most attractive theory is faulty embryogenic malformation of common bile duct associated to distal mechanical obstruction (sludge, stenosis, etc.). Most perforations occur at the junction of the cystic duct with the common bile duct. The infant presents an indolent course of jaundice, acholic stools, choluria, failure to thrive, and progressive abdominal distension most commonly. Less frequently the clinical course is acute with peritonitis and systemic signs of sepsis. Bilious ascites (localized) is the hallmark finding and is pathognomonic of IPBD in this age group. Ultrasound shows loculated ascitic fluid in porta hepatis and can help to guide diagnostic paracentesis (bile fluid). DISIDA scan confirms the diagnosis showing extravasation of isotope to the peritoneum. Management is surgical (medical tx is fatal), consist of intraoperative diagnostic cholangiography, tube cholecystostomy for follow-up, penrose drainage of porta hepatis, systemic antibiotics and TPN. Most perforations seal by 2-3 wk., the drains can be removed when tube cholangiogram shows a patent biliary tree. Prognosis is excellent with absent hepato-biliary long-term sequelae.

1- Rivilla F: Idiopathic perforation of the extrahepatic bile duct in infancy: pathogenesis, diagnosis, and management [letter; comment] J Pediatr Surg 29(7):955-6, 1994
2- Banani SA, Bahador A, Nezakatgoo N: Idiopathic perforation of the extrahepatic bile duct in infancy: pathogenesis, diagnosis, and management [see comments] J Pediatr Surg 28(7):950-2, 1993
3- Hammoudi SM, Alauddin A: Idiopathic perforation of the biliary tract in infancy and childhood. J Pediatr Surg 23(2):185-7, 1988
4- Stringel G, Mercer S: Idiopathic perforation of the biliary tract in infancy. J Pediatr Surg 18(5):546-50, 1983
5- Dinner M: Biliary peritonitis due to idiopathic perforation of the common bile duct. S Afr J Surg 13(4):207-9, 1975


Human beings are homeothermic organisms because of thermoregulation. This equilibrium is maintained by a delicate balance between heat produced and heal lost. Heat production mechanisms are: voluntary muscle activity increasing metabolic demands, involuntary muscle activity (shivering) and non-shivering (metabolizing brown fat). Heat loss occurs from heat flow from center of the body to the surface and from the surface to the environment by evaporation, conduction, convection and radiation. There is an association between hypothermia and mortality in the NICU's. The surgical neonate is prone to hypothermia. Below the 35 degrees centigrade the newborn experiences lassitude, depressed respiration, bradycardia, metabolic acidosis, hypoglycemia, hyperkalemia, elevated BUN and oliguria (neonatal cold injury syndrome). Factors that precipitate further these problems are: prematurity, prolonged surgery, and eviscerated bowel (gastroschisis).

1- Risbourg B, Vural M, Kremp O, de Broca A, Leke L, Freville M: Neonatal thermoregulation. Turk J Pediatr 33(2):121-34, 1991
2- Buczkowski-Bickmann MK: Thermoregulation in the neonate and the consequences of hypothermia. CRNA 3(2):77-82, 1992
3- Goldsmith JR, Arbeli Y, Stone D: Preventability of neonatal cold injury and its contribution to neonatal mortality. Environ Health Perspect 94:55-9, 1991
4- Hedman-Dennis S: Stabilization of the sick infant or child. J Post Anesth Nurs 6(3):165-9, 1991

5- Hazan J, Maag U, Chessex P: Association between hypothermia and mortality rate of premature infants--revisited [see comments] Am J Obstet Gynecol 164(1 Pt 1):111-2, 1991

IA: Female Concepts

The most frequent defect in females patient with imperforate anus (IA) is vestibular fistula, followed by vaginal fistulas. In more than 90% of females cases perineal inspection will confirm the diagnosis. These infants require a colostomy before final corrective surgery. The colostomy can be done electively before discharge from the nursery while the GI tract is decompressed by dilatation of the fistulous tract. A single orifice is diagnostic of a persistent cloacal defect usually accompany with a small- looking genitalia. Cloacas are associated to distended vaginas (hydrocolpos) and urologic malformations. This makes a sonogram of abdomen very important in the initial management of these babies for screening of obstructive uropathy (hydronephrosis and hydroureter). Hydrocolpos can cause compressive obstruction of the bladder trigone and interfere with ureteral drainage. Failure to gain weight and frequents episodes of urinary tract infections shows a poorly drained urologic system. A colostomy in cloacas is indicated. 10% of babies will not pass meconium and will develop progressive abdominal distension. Radiological evaluation will be of help along with a diverting colostomy in this cases. Perineal fistulas can be managed with cutback without colostomy during the neonatal period.

1- Pena A: Anorectal malformations. Semin Pediatr Surg 4(1):35-47, 1995
2- Pena A: Management of anorectal malformations during the newborn period. World J Surg 17(3):385-92, 1993
3- Pena A: Posterior sagittal approach for the correction of anorectal malformations. Adv Surg 19:69-100, 1986
4- Pena A: Surgical treatment of high imperforate anus. World J Surg 9(2):236-43, 1985

5- deVries PA, Pena A: Posterior sagittal anorectoplasty. J Pediatr Surg 17(5):638-43, 1982

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