Polydactyly of the hands and feet

Polydactyly of the hand, of the foot, and of the hand combined with the foot were studied. As noted in previous reports of polydactyly, inherited cases represent about 10% of polydactyly of the hand or foot, and unilateral involvement is more common than bilateral. In 495 cases of polydactyly 27 patients had polydactyly of the hands and feet. The extra digits in 21 of those 27 were in the same digital rays of the hand and foot. There were 14 inherited cases (51.9%) and most were an autosomal dominant, but some were an autosomal recessive. Both hand and/or feet were involved in 22 cases. Most cases of preaxial polydactyly of the hand combined with central polydactyly of the foot were sporadic and unilateral. All cases of both preaxial and postaxial (polyaxial) polydactyly were inherited and bilateral. Two pairs of sisters had the same clinical features. The parent (mother or father) also had the same deformities in the inherited cases.     

Jugular foramen schwannomas: diagnosis and suggestions for surgical management

Schwannomas arising in the parapharyngeal space are rare lesions; however, those originating in the jugular foramen are even less common. Two cases, each with marked intra- and extracranial extensions, are discussed. Clinical presentation and preoperative evaluation emphasizing computerized tomographic and magnetic resonance imaging will be presented. An aggressive two-stage surgical approach consisting of a retrosigmoid craniectomy combined with infratemporal removal is advocated for those lesions arising within the jugular foramen.     

Aggressive surgical management of craniopharyngiomas in children.

The cases of 50 patients with craniopharyngioma operated on at The Hospital for Sick Children in Toronto between January, 1975, and December, 1989, are reviewed. All patients were under 18 years of age (mean 9.39 years). Headaches, endocrine deficiencies, and visual deficits were the most common symptoms on admission. Forty-five patients underwent what was considered by the surgeon to be total excision of their tumor, and five had subtotal excision. Tumors recurred in 17 patients (mean time of recurrence 32.6 months after surgery). One patient died in the postoperative period and three have been lost to follow-up study. Of the remaining 46 patients, 28 are leading a normal or nearly normal life, although all are receiving endocrine replacement and some have required help to overcome mild deficits in memory or visual acuity. Twelve patients are able to function reasonably well and attend school despite being hampered by intellectual or visual deficits or problems with weight control; four have a significant handicap, and two have died.     

Intraoperative aneurysmal rupture: incidence, outcome, and suggestions for surgical management

Intraoperative rupture of an intracranial arterial aneurysm can dramatically interrupt a deliberate microsurgical procedure and jeopardize the patient's chances for a favorable outcome. Intraoperative rupture occurred in 58 of 307 (19%) consecutive aneurysm procedures done at The University of Texas Health Science Center. Rupture occurred during three specific periods: early or predissection in 7%, dissection in 48%, and clip application in 45%. Outcome after rupture during the predissection interval was poor, with only 1 of 4 patients surviving. Aneurysmal rupture during dissection could be attributed to blunt dissection techniques in 75% of the cases and to sharp subarachnoid dissection in 25%. The outcome was favorable in only 50% of the patients sustaining blunt dissection errors, whereas all patients sustaining intraoperative rupture during sharp dissection recovered well. Rupture during clip application was attributed to incomplete dissection in 65%, poor clip application in 31%, and a mechanical clip failure in 1 case. Eighty-eight per cent of the patients who underwent uneventful operative procedures had favorable outcomes, whereas only 62% of the patients suffering intraoperative rupture recovered well. The use of sharp microsurgical techniques with a systematic contingency plan for dealing with sudden hemorrhage and the judicious use of temporary clips should minimize the adverse effect of intraoperative rupture on overall management morbidity and mortality.     

Gastro-oesophageal reflux in children: surgical management

Gastro-oesophageal reflux disease (GORD) is symptomatic reflux of gastric contents into the oesophagus. Factors predisposing to GORD are loss of the physiological antireflux barrier and anatomic abnormalities of the oesophagus or diaphragm. Conservative measures and medical management results in resolution of symptoms in a majority of children. Surgery is indicated in the event of failure of medical management or severe complications. Surgical procedures include open or laparoscopic fundoplication in children with normal neurology; fundoplication with or without vagotomy and pyloroplasty; surgical feeding jejunostomy and oesophago-gastric dissociation in the severely neurologically impaired children.     

Gastro-oesophageal reflux in children: surgical management

Gastro-oesophageal reflux occurring infrequently and without symptoms or occurring without damage to the oesophagus is not considered pathological, otherwise the reflux and the pathology are referred to as gastro-oesophageal reflux disease (GORD). Factors predisposing to GORD include: impairments to the physiological antireflux barrier and anatomical abnormalities of the oesophagus or diaphragm. Non-operative and medical management result in resolution of symptoms in the majority of children. Surgery is indicated in the event of failure of medical management or severe complications and in neurologically normal children this would usually be a laparoscopic or open fundoplication. In severely neurologically impaired children other options are sometimes considered and these include: fundoplication with or without vagotomy and pyloroplasty; surgical feeding jejunostomy and oesophago-gastric dissociation.     

Polydactyly of the thumb: a surgical plan based on ninety-five cases

Preaxial polydactyly constitutes the most common group of congenital anomalies of the hand among the Chinese population of Hong Kong. Ninety-five cases from the Orthopaedic/Plastic and Reconstructive Surgery Unit, Princess Margaret Hospital, Hong Kong (March 1976 to March 1981), were reviewed. The cases were classified according to Wassel's classification (1969) into seven types. The most common category was type IV followed by types II, V, III, VII, I, and VI. The treatment of each type was analyzed. Bilhaut's procedure was satisfactory for type I, and a modified technique was used for types II and III. A method of shaving the incongruous articular cartilage was used in type IV. Removal of the extra digit and osteotomy of the metacarpal usually were required for type V. The experience with types VI and VII was too limited for assessment. Short-term results (1 to 6 years) have been satisfactory, but follow-up until skeletal maturity to assess the ultimate functional and cosmetic results is necessary.     

Flat feet in children

In the assessment of the child with flat feet a sound knowledge of arch and subtalar biomechanics, epidemiology, and etiology is important. The occurrence and natural history of flat feet are presented, and treatment modalities that have withstood the test of time are discussed.     

Advances in the surgical management of sarcomas in children

The management of children with soft tissue sarcomas has changed and survival has improved markedly during the past three decades. This is, in large part, due to the multi-modal approach to pediatric malignancies; decreasing the need for radical surgical procedures, while improving survival and decreasing morbidity. The management of patients with rhabdomyosarcomas (RMS) and nonrhabdomyosarcomas soft-tissue sarcomas (NRSTS) as well as neurofibromatosis and related tumors is best accomplished in an organized and systematic fashion with the surgeon involved early in the planning, diagnostic, and treatment phases. Cooperative trials for rhabdomyosarcoma, as part of the Intergroup Rhabdomyosarcoma Study, have led to improved survival with less radical surgery. Ongoing trials of the Soft Tissue Sarcoma Group of the Children's Oncology Group are under way. Since these tumors are rare, cooperative trials are essential to answering the remaining questions regarding extent of surgery and adjuvant therapy.     

The surgical management of insulinomas in children

Purpose

Insulinomas are rare pediatric tumors for which optimal localization studies and management remain undetermined. We present our experience with surgical management of insulinomas during childhood.

Methods

A retrospective review was performed of patients who underwent surgical management for an insulinoma from 1999 to 2012.

Results

The study included eight patients. Preoperative localization was successful with abdominal ultrasound, abdominal CT, endoscopic ultrasound, or MRI in only 20%, 28.6%, 40%, and 50% of patients, respectively. Octreotide scan was non-diagnostic in 4 patients. For diagnostic failure, selective utilization of 18-Fluoro-DOPA PET/CT scanning, arterial stimulation/venous sampling, or transhepatic portal venous sampling were successful in insulinoma localization. Intraoperatively, all lesions were identified by palpation or with the assistance of intraoperative ultrasound. Surgical resection using pancreas sparing techniques (enucleation or distal pancreatectomy) resulted in a cure in all patients. Postoperative complications included a pancreatic fistula in two patients and an additional missed insulinoma in a patient with MEN-1 requiring successful reoperation.

Conclusions

Preoperative tumor localization may require many imaging modalities to avoid unsuccessful blind pancreatectomy. Intraoperative palpation with the assistance of ultrasound offers a reliable method to precisely locate the insulinoma. Complete surgical resection results in a cure. Recurrent symptoms warrant evaluation for additional lesions.     

Long-term outcomes for children with very early-onset colitis: Implications for surgical management

Purpose

The timing of J-pouch surgery following colectomy for children with very early-onset colitis is controversial, with some advocating early reconstruction and others delaying reconstruction because of fear that the colitis may be owing to Crohn's disease (CD). We sought to determine the long-term incidence of CD in this population and whether there may be clinical features that predict the risk of CD.

Fracture of the penis: surgical management.

Two cases of fracture of the penis are reported. Early surgical repair of laceration of the corpus cavernosum brought about excellent results.     

Medial osteochondritis of the talus in children: review and new surgical management

Review of the management of medial osteochondritis of the talus in the young patient has revealed the poor results of casting and the equivocal results of past surgical options. A new surgical technique of bone grafting is described. The results in six patients treated with bone grafting are compared with the results in 10 patients treated by excision. Because of the more favorable results following bone grafting, we recommend it as primary treatment of this condition in children and adolescents.     

儿童隐匿性阴茎的手术治疗

目的:探讨隐匿性阴茎的发病因素和手术治疗方式。方法:总结5年来32例隐匿性阴茎患者的临床资料,对其病理类型及手术治疗方式及效果进行回顾性分析。结果:26例患者都有不同程度的阴茎浅筋膜发育异常,22例患者行阴茎海绵体松解牵拉术,10例行Maizels术。术后随访4个月～5年,3例术后出现龟头及包皮缘水肿,经温盐水坐浴及理疗于手术后1月内消退渐恢复正常。1例术后海绵体部分回缩。31例患者手术后龟头显露良好,阴茎较术前平均延长2.2c(m勃起状态)。结论:隐匿性阴茎的形成与先天性阴茎浅筋膜发育异常有关,彻底解除皮肤浅筋膜纤维索带,将阴茎根部白膜牢靠固定是手术成功的关键。     

Malignant tumor of the trachea in children: diagnostic pitfalls and surgical management

Primary tracheal malignant neoplasms are very rare. Histologically, squamous cell and adenoid cystic carcinomas are the most common types of malignant primary tracheal tumors when all age groups are studied. In the past 5 years, we treated 2 children with tracheal mucoepidermoid carcinoma. Herein we report both cases and review the literature on the subject with particular emphasis on diagnosis and surgical management.