Juda Z. Jona

Primary Laparoscopic-Assisted Endorectal Colon Pull-Through for Hirschsprung’s Disease: A New Gold Standard

OBJECTIVE To describe the surgical technique and early clinical results after a one-stage laparoscopic-assisted endorectal colon pull-through for Hirschsprungs disease. SUMMARY BACKGROUND DATA Recent trends in surgery for Hirschsprungs disease have been toward earlier repair and fewer surgical stages. A one-stage pull-through for Hirschsprungs disease avoids the additional anesthesia, surgery, and complications of a colostomy. A laparoscopic-assisted approach diminishes surgical trauma to the peritoneal cavity. METHODS The technique uses four small abdominal ports. The transition zone is initially identified by seromuscular biopsies obtained laparoscopically. A colon pedicle preserving the marginal artery is fashioned endoscopically. The rectal mobilization is performed transanally using an endorectal sleeve technique. The anastomosis is performed transanally 1 cm above the dentate line. This report discusses the outcome of primary laparoscopic pull-through in 80 patients performed at six pediatric surgery centers over the past 5 years. RESULTS The age at surgery ranged from 3 days to 96 months. The average length of the surgical procedure was 2.5 hours. Almost all of the patients passed stool and flatus within 24 hours of surgery. The average time for discharge after surgery was 3.7 days. All 80 patients are currently alive and well. Most of the children are too young to evaluate for fecal continence, but 18 of the older children have been reported to be continent. CONCLUSION Laparoscopic-assisted colon pull-through appears to reduce perioperative complications and postoperative recovery time dramatically. The technique is quickly learned and has been performed in multiple centers with consistently good results.

Anatomic observations and etiologic and surgical considerations in choledochal cyst.

Twenty-five operative and postoperative cholangiograms in children with choledochal cysts indicated an intimate relationship between these cysts and anomalous pancreatico-biliary ductal junction. In all of these patients the distal main pancreatic duct (MPD) is excluded from the sphinctor of Oddi mechanism and free reflux of pancreatic juice up the biliary tree occurs. This leads to chronic cholangitis, which is presumed to be responsible for the formation of the choledochal cysts. Abrupt and intense exposure of the common bile duct (CBD) to pancreatic reflux may cause perforation and bile peritonitis. Long-standing exposure may induce malignant transformation in the choledochal cyst. Total cyst excision and separation of the biliary system from exposure to pancreatic reflux is suggested as the procedure of choice. Long-term results and metabolic and digestive consequences of this operation are yet to be determined.

Esophageal bougienage technique for coin ingestion in children

An analysis was performed of 46 consecutive children who received esophageal bougienage for an ingested coin lodged in the esophagus. All patients met the following clinical criteria necessary for performance of this procedure: an acutely ingested single coin, radiographically localized in the esophagus; no previous history of an esophageal disease process, surgical procedure performed or foreign body removed; and no respiratory compromise upon physical examination. All coins were successfully advanced distally into the stomach after one pass of the bougie dilator. No complications were noted during or after performance of any procedure. Esophageal bougienage is a safe and effective method used to dislodge and pass an ingested coin from the esophagus when criteria for its performance are adhered to rigidly.

The megacystis microcolon intestinal hypoperistalsis syndrome: Report of a case

An infant girl with the megacystis microcolon intestinal hypoperistalsis syndrome is reported. The presence of polyhydramnios and the absence of upper urinary tract abnormalities, differentiate this patient from similar cases previously reported. Although the etiology of this disease is unknown, partial success with the use of bethanechol suggests that the deranged intestinal motility may be related to dysfunction of the autonomic nerve endings of the gut.

Lymphoid hyperplasia of the bowel and its surgical significance in children

Twelve children with abdominal complaints had lymphoid hyperplasia of the bowel presenting in an acute or chronic form. The etiology is obscure. An infectious process is thought to precipitate the acute form of the disease. Parasites were identified in two patients with the chronic disease. The acute form, with involvement of the appendix or terminal ileum, presents commonly as acute appendicitis. Because of its self-limiting nature, appendectomy with perservation of the terminal ileum is appropriate. When intussusception is present, resection of the ileum is advisable. The chronic form, which is also common in the terminal ileum, produces disabling symptoms, recurrent intussusception, chronic anemia, and weight loss and is, therefore, amenable to surgical resection.

Diagnosis and management of scrofula in children

Abstract Atypical myobacterial infection in humans appears to be of low virulence and is acquired from the environment with no evidence of transmission ever from human contact. The majority of infected patients are never symptomatic and the disease is self-limited. When symptomatic disease does occur, it is usually in children. Treatment should be confined to patients with symptomatic cervical adenitis; surgical excision of the subcutaneous abscess and of the offending caseous lymph node is sufficient. Adjacent hyperplastic nodes should not be excised, and chemotherapy is not indicated.

Necrotizing fasciitis in infancy: An uncommon setting and a prognostic disadvantage

Necrotizing fasciitis is a potentially fatal, progressive soft tissue infection that typically occurs in adults, and only rarely occurs in infants. Although adults in whom necrotizing fasciitis develops are commonly diabetic, malnourished, or otherwise immunocompromised, infants in whom the disease develops are typically healthy and without clear predisposing factors. Herein, however, the authors report the case of an infant with compromised immunity secondary to the manifestations and treatment of panhypopituitarism, in whom postoperative necrotizing fasciitis developed after bilateral inguinal herniorrhaphy. The diagnosis, pathological mechanism, and treatment of necrotizing fasciitis are reviewed and the distinguishing features in infants are highlighted. The combination of a low incidence and very high mortality rate associated with necrotizing fasciitis in this subgroup strengthens the need for hypercritical suspicion. Early diagnosis and the prompt initiation of surgical treatment are the most essential means to improve on the prognosis for necrotizing fasciitis in infants.

Castration-sparing management of an adolescent with huge bilateral cystic teratomas of the ovaries

In order to avoid the castrating effect of bilateral oophorectomy in a teenager with bilateral huge ovarian teratomas, a plane of dissection was developed near each ovarian hilum, and the cysts were excised in toto without spillage. Postoperatively, menstruation resumed promptly as the hormonal levels returned to normal. Benign cystic teratomas of the ovaries can be excised without the need for oophorectomy. This ovarian-sparing technique is particularly useful in cases of bilateral teratomas, where reproductive and hormonal functions should be preserved.

Rickets in Premature Infants Receiving Parenteral Nutrition: A Case Report and Review of the Literature:

There is a multifactorial etiology for rickets in premature infants receiving total parenteral nutrition. As an example of this, we describe a premature infant fed almost exclusively parenterally for the first 8 months of life who developed biochemical and radiologic evidence of rickets despite receiving vitamin D, calcium, and phosphorus at presently recommended levels. From our experience and a review of previous literature, there appears to be considerable uncertainty regarding the intravenous requirements of calcium, phosphorus, and vitamin D, particularly in low-birth weight infants. Therefore, measurements of serum 25-hydroxyvitamin D levels, vitamin D, and mineral intakes, and calcium and phosphorus excretion might be clinically useful for monitoring premature infants receiving total parenteral nutrition. Further research delineating more precisely the vitamin D, calcium, and phosphorus requirements of such infants is also suggested.

Steroids and bag and mask ventilation in the treatment of acute epiglottitis

A non-instrumentation treatment protocol for acute epiglottitis that features the use of dexamethasone to reduce edema and positive pressure ventilation by bag and mark to support ventilation, if needed, has been used in 40 cases, 1974--1977, with no mortality. The presence of the airway specialist (pediatric surgeon) in the Intensive Care Unit with the patient further assured the airway. The importance of having a protocol in place that is workable for the specific local circumstances of each institution is emphasized.