Frank A. Vicari

Expanded full-thickness skin grafts in children: case selection, planning, and management.

&NA; Since our initial presentation of our experience with tissue expansion as a means of harvesting large full-thickness skin grafts in children in 1987, we have continued to “expand” both the size of full-thickness skin grafts harvested and the range of reconstructive problems to which we have applied the technique. Sixteen expanded full-thickness grafts have been used for immediate reconstruction following giant nevus excision and in postburn reconstruction. Patients ranged in age from 6 months to 15 years, with follow-up ranging from 6 months to 15 years. Grafts ranging in size from approximately 60 cm2(excluding the dimensions of one submental graft) to greater than 700 cm2 were harvested from expanded donor sites on unilateral or bilateral groin/lower abdomen, clavicular, and a single submental expansion. Five expanded full-thickness grafts were used in facial reconstruction for single aesthetic unit coverage, multiple unit, and one single-sheet full facial graft. One expanded full-thickness graft was used on the breast. Three grafts were used in dorsal hand and finger coverage, and seven were used on the lower extremity, including an entire plantar surface and toes. Graft loss was confined to a 6.25-cm2 area on one cheek in the full facial expanded full-thickness grafts and a 9-cm2 area on the non-weight-bearing area of the full plantar graft. Donor-site complications were negligible. The anatomic confines of the donor sites and size of the patient may require expander replacement (in situ serial expansion) in order to obtain a large enough graft and accomplish primary donor-site closure. Expander and injection port placement in children for ease of injection and planned expander change must be anticipated. Our protocol from preoperative teaching through graft take is reviewed. Experience has demonstrated that expanded full-thickness grafts maintain all the characteristics of non-expanded full-thickness skin grafts and are an excellent reconstructive option in children.

Retrograde fibreoptic intubation in a child with Nager's syndrome.

PurposeThe authors describe a retrograde fibreoptic technique for tracheal intubation in a micrognathic child with a tracheo-cutaneous fistula.Clinical featuresA four-year-old child with Nager’s syndrome presented for surgical closure of a tracheocutaneous fistula. A tracheostomy tube had been placed in the neonatal period for management of upper airway obstruction due to severe micrognathia. At 2 1/2 yr of age, after a successful mandibular advancement procedure, the tracheostomy was removed and the child allowed to breathe through the natural airway. Preoperative physical examination revealed an uncooperative child, unable to open her mouth due to limited temporo-mandibular motion. The child was first anaesthetized with ketamine, 70 mg im, then halothane by mask. The authors were unable to open the child’s mouth sufficiently to allow rigid laryngoscopy. Attempts at oral and nasal fibreoptic intubation were unsuccessful. Ultimately, the authors were able to intubate nasally by passing an ultrathin Olympus LF-P laryngoscope under direct vision through the tracheocutaneous fistula in a cephalad direction, through the larynx and nasopharynx, then out the nares. An endotracheal tube was then advanced over the fibreoptic scope and positioned distal to the tracheocutaneous fistula. The surgical procedure was successfully accomplished and the trachea was extubated postoperatively without difficulty.ConclusionRetrograde fibreoptic intubation may be an option for airway management of a select group of children who cannot be intubated by traditional techniques.RésuméObjectifDécrire la technique d’intubation endotrachéale fibroscopique utilisée par voie rétrograde chez un enfant micrognathe et porteur d’une fistule trachéocutanée.Caractéristiques cliniquesUn enfant de quatre ans porteur du syndrome de Nager était programmé pour la fermeture chirurgicale d’une fistule trachéocutanée. Préalablement, à la période néonatale, une canule de trachéostomie avait été insérée pour traiter l’obstruction des voies aétiennes causée par une importante micrognathie. À deux ans et demi, après unerintervention réussie pour avancer la mandibule, la trachéostomie avait été délaissée pour permettre à l’enfant de respirer par les voies naturelles. L’examen préopératoire montrait un enfant non coopératif et incapable d’ouvrir la bouche suffisamment pour permettre la laryngoscopie rigide. L’enfant a d’abord été anesthésié à la kétamine 70 mg im et ensuite au masque avec halothane: Les tentatives d’intubation par laryngofibroscopie orale et nasale se sont avérées infructueuses. Finalement, les auteurs ont réussi à intuber pr voie nasale en passant un laryngoscope Olympus LF-P ultrafin sous vision directe à travers la fistule trachéocutanée en direction céphalique, à travers la larynx et le nasopharynx et ensuite à travers une narine. Le tube endotrachéal a été ensuite poussé sur le fibroscope et positionné distalement à la fistule trachéocutanée. L’intervention chirurgicale a pu être effectuée et la trachée extubée en postopératoire sans difficultés.ConclusionL’intubation fibroscopîque rétrograde peut représenter une solution applicable à la gestion des voies aériennes chez un groupe choisi d’enfants qui nepeuvent être intubés par les techniques conventionnelles.

Reply: Effectiveness of Conservative Therapy and Helmet Therapy for Positional Cranial Deformation

Background: The authors investigated the effectiveness of conservative (repositioning therapy with or without physical therapy) and helmet therapy, and identified factors associated with treatment failure. Methods: A total of 4378 patients evaluated for deformational plagiocephaly and/or deformational brachycephaly were assigned to conservative (repositioning therapy, n = 383; repositioning therapy plus physical therapy, n = 2998) or helmet therapy (n = 997). Patients were followed until complete correction (diagonal difference <5 mm and/or cranial ratio <0.85) or 18 months. Rates of correction were calculated, and independent risk factors for failure were identified by multivariate analysis. Results: Complete correction was achieved in 77.1 percent of conservative treatment patients; 15.8 percent required transition to helmet therapy (n = 534), and 7.1 percent ultimately had incomplete correction. Risk factors for failure included poor compliance (relative risk, 2.40; p = 0.009), advanced age (relative risk, 1.20 to 2.08; p = 0.008), prolonged torticollis (relative risk, 1.12 to 1.74; p = 0.002), developmental delay (relative risk, 1.44; p = 0.042), and severity of the initial cranial ratio (relative risk, 1.41 to 1.64; p = 0.044) and diagonal difference (relative risk, 1.31 to 1.48; p = 0.027). Complete correction was achieved in 94.4 percent of patients treated with helmet therapy as first-line therapy and in 96.1 percent of infants who received helmets after failed conservative therapy (p = 0.375). Risk factors for helmet failure included poor compliance (relative risk, 2.42; p = 0.025) and advanced age (relative risk, 1.13 to 3.08; p = 0.011). Conclusions: Conservative therapy and helmet therapy are effective for positional cranial deformation. Treatment may be guided by patient-specific risk factors. In most infants, delaying helmet therapy for a trial of conservative treatment does not preclude complete correction. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Anaesthetic anagement of Miller’s syndrome

Miller’s syndrome is a rare congenital disorder with facial features similar to that of Treacher-Collins syndrome. This report details the anaesthetic management of an infant during multiple surgical procedures, beginning with pylormyotomy at one month of age. Airway management was difficult because of severe micrognathia and was accomplished using an awake intubation with a conventional straight blade modified for continuous administration of oxygen (“oxyscope”). Due to recurrent upper airway obstuction and the anticipated need for multiple surgical procedures in the first years of life, a tracheostomy was placed. Because of the multiple airway, orthopaedic, and nutritional difficulties, it is important that a prospective, multidisciplinary approach be used in these patients’ care. Consideration should be given to early tracheostomy for airway maintenance.RésuméLe syndrome de Miller est une maladie congénitale rare avec des caractéristiques faciales similaires au syndrome de Treacher-Collins. Ce rapport étudie la conduite anesthésique chez un enfant lors de procédures chirurgicales multiples, commençant par une pyloromyotomie à l’àge d’un mois. Le contrôle des voies aériennes fut difficile à cause d’une micrognatie et fut accompli par une intubation réveillée avec une lame droite conventionnelle modifiée pour l’administration continue d’oxygène (« oxyscope »). A cause de problèmes d’obstruction des voies aériennes hautes récurrentes et l’anticipation d’une nécessité de procédures chirurgicales multiples dans les premières années de la vie, une trachéotomie fut faite. A cause de problèmes multiples des voies aériennes, orthopédique et nutritionnelles, it est important d’instaurer une approche multidisciplinaire pour le soin de ces patients. Des considérations doivent être faites pour une trachéotomie précoce pour le maintien des voies aériennes.

Parasitic rachipagus conjoined twin: case report

A parasitic twin represents a rare developmental anomaly in which an asymmetrical, nonviable conjoined twin is attached to the host body at the time of birth. Rachipagus is among the rarest of conjoined twin subtypes and typically features a parasitic twin mass attached at the spine. Herein, the authors review the literature and describe the case of a 9-month-old girl presenting with a rachipagus parasitic twin consisting of a fully developed set of lower extremities originating from the midline upper back. After a complete workup to delineate parasite and autosite anatomy, the parasitic twin mass was successfully excised by a multidisciplinary team of surgeons, and the resulting defect was closed in a single stage.

Distal Airway Lesions in Mandibular Distraction Patients

Objective: 1) Determine prevalence and type of synchronous airway lesions (SALs) in patients undergoing mandibular distraction osteogenesis (MDO) at Children’s Memorial Hospital. 2) To determine whether these SALs affect surgical success of MDO in relieving airway obstruction. Method: Restrospective review of patients with micrognathia undergoing MDO and direct laryngoscopy and bronchoscopy (DLB) over a period of 11 years (2001-2011) at a tertiary care pediatric hospital. Charts were reviewed for demographics, prevalence/type of SALs, success rate for airway improvement for MDO, and coexisting medical and craniofacial conditions. Results: Eighteen patients were found who had undergone both MDO and DLB. The incidence of at least 1 SAL was 77.8%. Types of SALs encountered include laryngeal edema (61.1%), tracheobronchitis (50%), laryngomalacia (38.9%), tracheomalacia (16.7%), bronchomalacia (16.7%), subglottic stenosis (11.1%), pulsatile airway compression (11.1%), posterior laryngeal cleft (0%), and subglottic cysts (0%). 94.4% of MDO patients received tracheostomy, and 88.9% had airway improvement following MDO. Craniofacial anomalies include micrognathia (100%), cleft palate (61.1%), midface hypoplasia (33.3%), craniosynostosis (5.6%), choanal stenosis/atresia (5.6%), pyriform aperture stenosis (5.6%), and macroglossia (0%). Baseline medical characteristics/syndromes include Pierre Robin sequence (61.1%), Nager syndrome (11.1%), Stickler syndrome (11.1%), asthma (11.1%), Goldenhar syndrome (11.1%), arthrogryposis (5.6%), Mobius syndrome (5.6%), and Cornelia de Lange syndrome (5.6%). Conclusion: There is a high rate of SALs in patients undergoing MDO. The most common SALs are laryngeal edema, tracheobronchitis, and laryngomalacia. MDO is highly successful in relieving upper airway obstruction in micrognathic patients despite the high incidence of SALs. DLB should be considered in all patients who require MDO.